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Churg-Strauss syndrome is defined as an eosinophil-rich, granulomatous inflammation involving the respiratory tract, along with necrotizing vasculitis affecting small- to medium-sized vessels, and is associated with asthma and eosinophilia. We encountered an unusual case of year-old man with asthma and primary focal segmental glomerulosclerosis diagnosed as CSS showing myeloperoxidase anti-neutrophil cytoplasmic antibody-associated acute kidney injury with crescentic glomerulonephritis. Patient responded to steroid and cyclophosphamide.
Eosinophilic leukocytoclastic vasculitis - a spectrum ranging from Wells' syndrome to Churg-Strauss syndrome? Wells' syndrome is defined as an inflammatory disorder with the histopathological presence of eosinophilic infiltrates and flame figures in the absence of vasculitis.
Eosinophilic leukocytoclastic vasculitis shows eosinophilic infiltrates in combination with vasculitic changes. To determine whether these three diseases are distinct entities or different manifestations of a similar clinicopathologic process. Histopathological samples and clinical courses of 17 patients with eosinophilic infiltrates, flame figures and clinical features of Wells' syndrome were re-evaluated. Histopathologically, we focused on the presence or absence of vasculitic features.
Clinically, we included only patients who were diagnosed with Wells' syndrome at least once in the course of their disease. Further, we had one case of an overlap between Wells' syndrome and eosinophilic vasculitis and one case of Wegener granulomatosis. Vasculitic features were found in the samples of all patients. Histologically, we find vasculitic features in typical presentations of Wells' syndrome. Clinically, we find typical features of Wells' syndrome in patients finally diagnosed with eosinophilic leukocytoclastic vasculitis or Churg Strauss syndrome. Furthermore, we have observed and formerly reported 3 patients with progression from Wells' syndrome to Churg Strauss syndrome.
Thus, we assume that eosinophilic leukocytoclastic vasculitis might form a bridge between Wells' syndrome and Churg Strauss syndrome. Few case reports have described successful treatment of this rare disease.
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However, no one has reported on the application of mechanical life support with extracorporeal membrane oxygenation ECMO to treat this life-threatening disease. Laboratory tests revealed that eosinophil was significantly increased WBC: On the next day, cardiogenic shock had been developed as demonstrated by deteriorating the perfusion index. Churg-Strauss Syndrome with cardiogenic shock. A series of conservative therapy with drugs such as corticosteroids, anticoagulant, antiplatelet, nitrates, calcium antagonists, inotrope, and vasopressors were initiated on the day of admission.
The treatment was ineffective and a cardiogenic shock developed on the next day. Thus, ECMO was initiated immediately to stabilize circulation and perfusion. At the same time, high-dose corticosteroids combined with immunosuppressive therapy were continuously used.
Symptoms of cardiogenic shock were gradually improved after ECMO treatment. Elevated values of cardiac enzymes were decreased and the dose of vasoactive drugs was reduced. Extracorporeal membrane oxygenation was discontinued after 8 days, and the patient was eventually weaned off the ventilator. The patient was discharged after 40 days treatment. Once a CSS develops into a. Churg-Strauss syndrome , recently renamed eosinophilic granulomatosis with polyangiitis EGPA , is a rare form of systemic vasculitis, characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring among patients with asthma and tissue eosinophilia.
EGPA is classified as a small and medium-sized vessel vasculitis associated with antineutrophil cytoplasmic antibodies ANCA and the hypereosinophilic syndrome. Typical clinical features include asthma, sinusitis, transient pulmonary infiltrates and neuropathy. Blood eosinophils should always be tested in unexplained cardiac disorders, and may normalize even after low doses of corticosteroids. Any cardiac structure can be involved, and patients present with myocarditis, heart failure, pericarditis, arrhythmia, coronary arteritis, valvulopathy, intracavitary cardiac thrombosis.
Although cardiovascular involvement is usually an early manifestation, it can also occur later in the course of the disease. A significant proportion of patients with cardiac involvement is asymptomatic. All patients with EGPA should be studied not only with a detailed history of cardiac symptoms and ECG, but also with echocardiography; if abnormalities are detected, a cardiac magnetic resonance study should be performed. Coronary angiography and endomyocardial biopsy should be reserved to selected cases.
It is often insidious and underestimated. Optimal therapy is therefore important and based on high-dose corticosteroids plus immunosuppressive. Ocular involvement in Churg-Strauss syndrome is infrequent. We describe the case of a year-old woman with eosinophilia and involvement of the respiratory tract, skin, and peripheral nervous system, fulfilling the American College of Rheumatology criteria for Churg-Strauss syndrome. The patient presented with acute, painless vision loss in her right eye. Central retinal artery occlusion CRAO without accompanying retinal vasculitis was diagnosed by angiographic findings and funduscopic findings of retinal whitening with a cherry-red spot.
Although her antineutrophil cytoplasmic antibody ANCA status was negative, CRAO was thought to be an ocular manifestation of Churg-Strauss syndrome , and appropriate treatment was planned. She was treated with high-dose corticosteroids and anticoagulant therapy. Her macular edema improved, but visual recovery was poor. Churg-Strauss syndrome CSS is an allergic granulomatous angiitis, a rare disease of small and medium arteries and veins, associated with the presence of perinuclear antineutrophil cytoplasmic antibodies p-ANCA. We reported a female patient, aged 80 years, with asthma for many decades and repeatedly verified eosinophilia in peripheral blood, in which CSS was suspected only after the occurrence of skin changes in the form of vesicles, vesiculopustule, purpuric macula, papule and petechiae.
Further tests verified pulmonary infiltrates, paranasal sinusitis, extravascular eosinophils on histopathologic sample of skin tissue, and polyneuropathy. Despite long-standing pulmonary symptoms and laboratory findings of eosinophilia, the appearance of skin changes raised suspicion of possible CSS. Skin changes resolved and the patient was reffered to rheumatologist. Coronary involvement in Churg-Strauss syndrome. Systemic autoimmune diseases are themselves a relevant and independent risk factor for atherosclerosis and coronary ectasia.
We describe a case of a year-old Caucasian man who was admitted to our department for unstable angina. History of asthma, paranasal sinus abnormality, and peripheral eosinophilia given a high suspicion of Churg-Strauss syndrome CSS. Diagnosis was performed with 5 of the 6 American College of Rheumatology criteria.
The knowledge that CSS is often associated with significant coronary artery involvement and the persistence of chest pain led us to performing immediately a coronary angiography. Coronary angiography showed diffuse ectasic lesions, chronic occlusion of left anterior descending artery with homocoronary collateral circulation from left circumflex artery and subocclusive stenosis in the proximal tract of posterior descending artery.
The early recognition of CSS, an aggressive invasive diagnostic approach, and an early appropriate therapy are important to prevent the progressive and permanent cardiac damage in these patients. In the setting of a multidisciplinary approach, careful cardiac assessment is an essential step in CSS, even in mildly symptomatic patients. Published by Elsevier B. A case of Churg-Strauss syndrome: A case is presented of Churg-Strauss syndrome in a young man in whom the definitive diagnostic procedure was a full thickness sigmoidoscopic rectal biopsy, with submucosal sampling.
Gastrointestinal changes in Churg-Strauss syndrome , a rare systemic illness characterised by asthma, blood and tissue eosinophilia, vasculitis, and granulomatous inflammation are common but poorly reported. The endoscopic and histopathological features of a case are described and emphasise the potential value of a limited sigmoidoscopy in establishing the diagnosis, when lower gastrointestinal symptoms are present.
Chronic calcific constrictive pericarditis complicating Churg-Strauss syndrome: Churg-Strauss syndrome is a necrotizing systemic vasculitis characterized by extravascular granulomas and eosinophilic infiltrates of small vessels. Although cardiac complications are considered to be relatively common, no case of constrictive calcified pericarditis has ever been previously described in this setting.
In this report, we present the case of a year-old man with Churg-Strauss syndrome , in whom we were able to document the development of symptomatic calcific constrictive pericarditis during a year period despite long-term corticosteroid therapy. Crescentic glomerulonephritis in non-asthmatic Churg-Strauss syndrome.
A year-old male presented with sensory motor polyneuropathy and rapidly progressive renal failure. Investigations revealed marked peripheral eosinophilia and elevated perinuclear antineutrophil cytoplasmic antibody titers. Renal biopsy showed pauci-immune cre-scentic glomerulonephritis with interstitial eosinophil infiltrates. He had no history of asthma. Computed tomography of the chest and X-ray of the paranasal sinuses were normal.
On Day 1, the patient developed ileal perforation. Resected ileal segments showed small vessel vasculitis with extravascular eosinophils. A diagnosis of non-asthmatic variant of Churg-Strauss syndrome was made. Renal recovery was achieved in 12 weeks with a combination therapy of corticosteroid and cyclophosphamide. The patient has been relapse-free for 12 months on oral prednisolone therapy. Churg-Strauss syndrome concomitant with chronic symmetrical dacryoadenitis suggesting Mikulicz's disease. A case of Churg-Strauss syndrome complicated by chronic symmetrical dacryoadenitis suggestive of Mikulicz's disease is herein presented.
A year-old Japanese man, who had been previously diagnosed with asthma, presented with weakness of the left leg and purpura on the lower extremities. A neurological examination showed multiple mononeuropathies and a laboratory examination revealed elevated eosinophil counts, IgE levels and the presence of Myeloperoxidase-antineutrophil cytoplasmic antibody MPO-ANCAs. Churg-Strauss syndrome was diagnosed, although the patient also exhibited bilateral swelling of the lachrymal glands.
Furthermore, elevated serum IgG4 levels, an infiltration of a relatively large number of IgG4-positive plasmacytes in the nasal mucosa and hypocomplementemia were also observed. These findings were consistent with a diagnosis of Mikulicz's disease MD. Oral prednisolone 30 mg was administered and the swelling of the lachrymal glands resolved.
Churg-Strauss syndrome may be accompanied by Mikulicz's disease an IgG4-related disease , and common pathogeneses between Churg-Strauss syndrome and IgG4-related disease may exist. Eosinophilic granulomatosis with polyangiitis Churg-Strauss syndrome presenting as diffuse myositis. Eosinophilic granulomatosis with polyangiitis is a complex multisystemic syndrome with heterogeneous presentation. Most often, there is a clinical history of asthma or other atopic conditions, and current presentation generally includes signs of cutaneous or pulmonary involvement. Very few reports described myalgia or weakness as the chief complaint.
Of these, only a few included muscle biopsy evaluation and none showed convincing evidence of primary myositis. We believe this report is the first to demonstrate true myositis in the setting of early eosinophilic granulomatosis with polyangiitis. This report describes a 74 year old Caucasian man, with no known allergies, presenting severe myalgia, muscle weakness, jaw claudication, and fever.
Blood work showed marked eosinophilia and high creatine kinase levels. Biceps brachialis muscle biopsy revealed eosinophilic necrotizing vasculitis and true myositis with myophagocytosis of non-necrotic fibers and strong sarcolemmal MHC-1 overexpression by immunohistochemistry. This patient was successfully treated with prednisone and azathioprine. Our finding of true myositis in a case of eosinophilic granulomatosis with polyangiitis suggests that primary auto-immunity against muscle fibers, distinct from the secondary effects of vasculitis, can occur in this entity and may represent an overlap syndrome.
Early recognition of eosinophilic granulomatosis with polyangiitis in patients presenting with myositis may provide an opportunity to treat the vasculitis before onset of severe multisystemic disease. We recommend the use of muscle biopsy with immunohistochemistry for MHC-1 to confirm the diagnosis of myositis in the setting of eosinophilic granulomatosis with polyangiitis.
Churg Strauss syndrome associated with montelukast--case report. Churg-Strauss Syndrome allergic granulomatous angiitis is a rare systemic and pulmonary vasculitis. The patient was admitted in our hospital with fever, malaise, sensory deficits in the lower extremities, diffuse musculoskeletal and thoracic pain. Pulmonary infiltrates with eosinophilia can be used to define eosinophilic lung diseases. We made the differential diagnosis of eosinophilic lung disease: Bronchoalveolar lavage showed Few days after hospital admission patient experienced nausea, vomiting and diarrhea.
She underwent a digestive endoscopy, which showed eosinophilic enteritis according to colon biopsy. Nasal mucosa biopsy found granulomas. Anti-neutrophil cytoplasmatic antibody ANCA was positive at 1: This case report illustrates the importance of early diagnosis of Churg-Strauss syndrome and the possible pathogenic link between leukotriene receptor antagonist use and CSS development. Cutaneous manifestations of Churg-Strauss syndrome: Churg-Strauss syndrome is a rare systemic vasculitis characterized by asthma and other allergy symptoms as well as eosinophilia and necrotizing vasculitis involving small and medium-sized vessels.
Its prevalence in the general population ranges from cases per million a year, varying according to the population studied. The authors describe a case of a female patient affected by the disease with important systemic manifestations and not very florid skin lesions. Usefulness of cardiac resonance imaging in Churg-Strauss syndrome.
Churg-Strauss syndrome CSS is a rare entity that is characterized by widespread vasculitis, which affects both small and medium-sized blood vessels of nearly all organs. We describe a case of a patient with cardiac biopsy proven CSS, and we discuss the usefulness of cardiovascular MRI for its diagnosis. Eosinophilic granulomatosis with polyangiitis is a rare primary vasculitic disease characterized by hypereosinophilia, late onset asthma and extravascular eosinophil granulomas.
We report a case presented initially with acute myocardial infarction which later only proceed with asthma, skin manifestations and peripheral neuropathy. Laboratory parameters showed hypereosinohpilia with negative perinuclear pattern of antineutrophil cytoplasmic autoantibodies p-ANCA. Skin biopsy showed leucocytoclastic vasculitis with eosinophilic infiltration while coronary angiography was normal.
The patient's symptoms improved with IV methylprednisolone, pulse cyclophosphamide and azathioprine. Churg-Strauss syndrome CSS is a vasculitis syndromes and is only rarely complicated by subarachnoid hemorrhage. In the current report, we describe a case of CSS with subarachnoid hemorrhage, which showed a favorable outcome following conservative treatment. After 2 months, he presented sudden pain in the occipitocervical area, and CT revealed subarachnoid hemorrhage. Although both findings suggested cerebral artery dissection, the source of hemorrhage could not be identified.
The Japanese Guidelines for the Management of Stroke recommends early diagnosis and treatment of hemorrhagic cerebral artery dissection because of the high risk of re-bleeding. However, considering the risks of vasculitis aggravation, development of systemic complications, and recurrence, conservative treatment was selected.
In addition, owing to the risk of complications associated with the frequent use of iodinated contrast agents and angiography procedures, patient was followed up using MRI. His course was favorable, and he was discharged despite mild right abducens paralysis. When patients with hemorrhagic cerebral artery dissection have a history of allergic diseases, CCS should be considered; conservative treatment consisting of rest, strict blood pressure control, and steroid therapy may be the most appropriate option for certain patients.
Churg-Strauss syndrome CSS is a granulomatous small vessel vasculitis. It is characterized by asthma, allergic granulomatosis and vasculitis. This syndrome is rare in children. A 5 years old boy was admitted with cough, fever and dyspnea for 2 weeks. On the basis of laboratory data peripheral eosinophilia , associated with skin biopsy, and history of CSS in his sister, this disease was eventually diagnosed.
The patient had good response to corticosteroid. In every asthmatic patient with prolonged fever, eosinophilia and multisystemic involvment, CSS should be considered. A woman in her 70s with Churg-Strauss syndrome presented with epigastric pain. She was being treated with steroids at the time of admission. Computed tomography showed swelling of the gallbladder, and percutaneous transhepatic cholangiography revealed bloody secretion. On duodenoscopy, bleeding was observed from the orifice of the major duodenal papilla.
Emergency cholecystectomy was performed under a diagnosis of hemorrhagic cholecystitis;intraoperatively, extensive hematoma was detected in the thickened wall of the gallbladder. Subsequent histopathological examination revealed mucosal ulceration with infiltration of inflammatory cells, torn small vessels, and extensive transmural bleeding and abscess formation in the thickened wall of the gallbladder.
We considered that the hemorrhagic cholecystitis was induced by either vasculitis or corticosteroid therapy. To the best of our knowledge, this is the first report of hemorrhagic cholecystitis associated with Churg-Strauss syndrome. Anterior ischemic optic neuropathy in a patient with Churg-Strauss syndrome. We describe a patient with Churg-Strauss syndrome who developed unilateral anterior ischemic optic neuropathy. A year-old man with a history of bronchial asthma, allergic rhinitis, and sinusitis presented with sudden decreased visual acuity in his right eye that had begun 2 weeks previously.
Ophthalmoscopic examination revealed a diffusely swollen right optic disc and splinter hemorrhages at its margin. Goldmann perimetry showed central scotomas in the right eye and fluorescein angiography showed remarkable hyperfluorescence of the right optic nerve head. Marked peripheral eosinphilia, extravascular eosinophils in a bronchial biopsy specimen, and an increased sedimentation rate supported the diagnosis of Churg-Strauss syndrome.
Therapy with methylprednisolone corrected the laboratory abnormalities, improved clinical features, and preserved vision, except for the right central visual field defect. Early recognition of this systemic disease by ophthalmologists may help in preventing severe ocular complications. A twenty year old male presented with purpuric lesions with chronic painful ulcers over the lower extremities and a recurrent pruritic rash on the trunk for 10 years.
He was diagnosed as idiopathic leukocytoclastic vasculitis LCV after investigations failed to reveal a systemic association. He was treated with immunosuppressants at each visit with partial remission. In , he was diagnosed with bronchial asthma and allergic rhinitis. In his recent admission, he showed necrotic ulcers on legs and extensive shiny, truncal micropapules. Examination revealed maxillary sinus tenderness and loss of sensation on the medial aspect of the left lower limb. Radiography confirmed maxillary sinusitis, nerve conduction studies revealed mononeuritis of the anterior tibial nerve and pulmonary function tests PFT were normal.
This report highlights the development of full-blown CSS over a period of 12 years in a patient initially diagnosed as idiopathic LCV, emphasizing the need for regular follow-up of resistant and recurrent cases of LCV. To describe a central retinal artery occlusion in a patient with antineutrophil cytoplasmic antibody ANCA negative Churg-Strauss syndrome.
Review of clinical and laboratory findings of a year-old woman with ANCA negative Churg-Strauss syndrome that developed sudden vision loss in left eye. Left central retinal artery occlusion was diagnosed by retinal whitening, a cherry-red spot, and delayed arterial filling on fluorescein angiography. Patients with this diagnosis should be considered for prophylactic high dose corticosteroid, regardless of their ANCA status. Microvascular vaso-occlusion leading to digital gangrene is not a feature of CSS. While the full clinical spectrum of Churg Strauss Syndrome is still being defined, identification of additional features associated with this syndrome might help to better understand the pathogenesis of the disease and to have an impact on management and prognosis.
Cutting edge issues in the Churg-Strauss syndrome. Churg-Strauss syndrome CSS is a rare systemic small-vessel vasculitis that develops in the background of bronchial asthma, which is characterized by eosinophilia and eosinophilic infiltration of various tissues. It belongs to the group of antineutrophil cytoplasmic autoantibody ANCA -associated vasculitides. The triggering factors and pathogenesis of CSS are still unknown.
The possible role of eotaxin-3 and CCR4-related chemokines in selective recruitment of eosinophils to the target tissues in CSS has been recently suggested, but the role of eosinophilic inflammation in the development of vasculitic lesions is not completely understood. From the clinical view, two distinct phenotypes of the disease are slowly emerging depending on the ANCA-positivity status. Glucocorticoids are still the mainstay of treatment; however, data are accumulating regarding the beneficial role of novel immunosuppressants and biologic compounds, especially in patients with poorer prognosis.
EGPA occurs in patients with asthma. Asthma is sever, associated with eosinophilia and extrapulmonary symptoms. Among them, mononeuritis multiplex is the most frequent symptom. When cardiac involvement is present , prognosis is poor. Despite a good overall prognosis, deaths are caused by vasculitis activity, gastrointestinal and cardiac involvement. Treatment is well codified based on steroids, which are quickly effective. Immunosuppressants combined with corticosteroids are compulsory to treat the most sever forms, mainly when cardiac and gastrointestinal or renal symptoms are present.
Churg-Strauss syndrome associated with antiphospholipid antibodies in a patient with retinal vasculitis.
We present the case of a year-old woman with unilateral retinal vasculitis. Investigations showed asthma, rhinosinusitis, nasal polyposis, peripheral blood eosinophilia, increased sedimentation rate, proteinuria, and antiphospholipid antibodies. Anti-neutrophil cytoplasmic antibodies ANCA were negative.
Although her anti-neutrophil cytoplasmatic antibody ANCA status was negative, taking into account the other clinical and laboratory features, retinal vasculitis was thought to be an ocular manifestation of Churg-Strauss syndrome. Treatment was started with high-dose corticosteroids and anticoagulant therapy.
Clinical features and prognostic factors of Churg-Strauss syndrome. The purposes of our study were to characterize the clinical features of CSS and to identify factors associated with CSS prognosis in Koreans. Results Data from 52 CSS patients were analyzed. The respiratory tract was the most commonly involved organ Clinical remission occurred in Characteristics of good responders were older age, diagnosis at earlier stages, less cutaneous involvement, more respiratory involvement, high CRP values, and more generalized symptoms.
Churg-Strauss syndrome CSS is a rare systemic necrotizing small-vessel vasculitis, with accompanying bronchial asthma, eosinophilia, and eosinophilic infiltration of various tissues. Data from 52 CSS patients were analyzed. Loeffler's endocarditis is generally caused by hypereosinophilic syndrome.
It is a restrictive cardiomyopathy characterized with eosinophilia and eosionophilic penetration leading to the fibrous thickening of endocardium of both ventricles, apical obliteration and heart failure. We report a case of a year-old male with Loeffler's endocarditis caused by Churg-Strauss syndrome.
The echocardiogram showed that biventricular failure with large thrombus in left ventricle. His symptoms and typical echocardiographic findings markedly improved within 2 months after treatment for Churg-Strauss syndrome. Churg-Strauss syndrome associated with AA amyloidosis: Churg Strauss syndrome is a rare systemic and pulmonary vasculitis exceptionally associated with AA amyloidosis. We report the case of a year old woman with past medical history of asthma. She developed polyarthralgia, headache and purpura.
Renal biopsy showed an important type AA amyloid deposit. The patient was treated with steroids with a good response of the vasculitis and amyloidosis with disappearance of the proteinuria. Sequential rituximab and omalizumab for the treatment of eosinophilic granulomatosis with polyangiitis Churg-Strauss syndrome. Eosinophilic granulomatosis with polyangiitis EGPA , formerly known as Churg-Strauss syndrome CSS , is a small vessel vasculitis associated with eosinophilia and asthma.
Clinical manifestations commonly seen in patients presenting with EGPA range from upper airway and lung involvement to neurological, cardiac, cutaneous, and renal manifestations. Treatment for severe presentations includes steroids, cyclophosphamide, plasmapheresis, and recently, rituximab.
Rituximab is associated with a good response in the treatment of vasculitis, but a variable response for the control of allergic symptoms. Here, we report a year-old female patient with severe EGPA gastrointestinal and cutaneous vasculitis, rhinitis and asthma refractory to conventional treatment. She was treated with rituximab, which enabled rapid control of the vasculitis component of the disease, but there was no response to rhinitis and asthma. Additionally, she developed severe bronchospasm during rituximab infusion. Sequential rituximab and omalizumab were initiated, leading to remission of all manifestations of vasculitis, rhinitis, and asthma, in addition to bronchospasm related to rituximab infusion.
Combined Churg-Strauss syndrome and allergic bronchopulmonary aspergillosis - case report and review of the literature. The diagnosis of ABPA was established on the pathological findings of allegic mucin impaction and fungal hyphae on lung biopsy. It was further proved by positive serum IgE and IgG antibodies specific to afumigatus. The role of lung biopsy in the diagnosis of the condition was emphasized. Acute coronary syndrome caused by coronary vasospasms associated with Churg-Strauss syndrome: A year-old woman with a history of aspirin-induced asthma was admitted with severe chest pain.
Emergency coronary angiography revealed coronary artery spasms. The administration of vasodilators did not suppress the anginal symptoms, and the differential white blood cell count continued to show eosinophilia. The patient's symptoms of aspirin-induced asthma, eosinophilia and other allergic states led to the diagnosis of Churg-Strauss syndrome CSS. After starting betamethasone therapy, the eosinophilia and cardiac symptoms rapidly disappeared. Although coronary vasospasms related to CSS are rare, the present case suggests that a differential white blood cell count should be obtained in patients with refractory coronary vasospasms.
Systemic vasculitis is a rare disease, and the diagnosis is very difficult when patient shows atypical symptoms. We experienced an unusual case of dysphagia caused by Churg-Strauss syndrome with lower cranial nerve involvement. A year-old man, with a past history of sinusitis, asthma, and hearing deficiency, was admitted to our department for evaluation of dysphagia. He also complained of recurrent bleeding of nasal cavities and esophagus. Brain magnetic resonance imaging did not show definite abnormality, and electrophysiologic findings were suggestive of mononeuritis multiplex.
Dysphagia had not improved after conventional therapy. Biopsy of the nasal cavity showed extravascular eosinophilic infiltration. All these findings suggested a rare form of Churg-Strauss syndrome involving multiple lower cranial nerves. Dysphagia improved after steroid therapy. A year-old woman was admitted to our chest pain unit because of an acute coronary syndrome non ST-elevation myocardial infarction. She complained of asthma, chronic sinusitis and involuntary weight loss, occasional fever and night sweats over the past six months.
Coronary angiography did not show any signs of macroscopic coronary artery disease, while echocardiography demonstrated a hemodynamically not significant pericardial effusion. Magnetic resonance imaging of the heart revealed a subendocardial scar, extension and localization pointing to a vascular genesis. Thoracic computed tomography revealed pulmonary opacities and blood tests showed an eosinophilia, leading to the clinical diagnosis of Churg-Strauss syndome.
The patient responded quickly to oral steroids, and blood parameters returned to normal. Acute coronary syndrome in youngish patients without classical cardiovascular risk factors is suggestive for myocarditis but also for vasculitis. Churg-Strauss syndrome usually responds quickly to immunosuppressive therapy, associated with a rather good prognosis without high mortality.
Polyangiitis overlap syndrome of granulomatosis with polyangiitis Wegener's granulomatosis and eosinophilic granulomatosis with polyangiitis Churg-Strauss syndrome. Polyangiitis overlap syndrome is defined as systemic vasculitis that cannot be classified into one of the well-defined vasculitic syndromes. In this report, a female patient who presented with vasculitis-like and asthmatic symptoms was diagnosed as having polyangiitis overlap syndrome of granulomatosis with polyangiitis GPA; formerly known as Wegener's granulomatosis and eosinophilic granulomatosis with polyangiitis EGPA; formerly known as Churg-Strauss syndrome.
She was successfully treated with immunosuppressants and steroids and has been in remission for 20 months. It is important to establish a proper diagnosis and introduce an appropriate treatment modality in patients with this rare and serious pathology to prevent irreversible organ damage. Churg-Strauss syndrome CSS is a systemic necrotizing vasculitis of the small and medium vessels, associated with extravascular eosinophilic granulomas, peripheral eosinophilia, and asthma.
The exact etiology of CSS is unknown. This syndrome commonly affects the lungs, peripheral nerves, skin, heart, and gastrointestinal tract, but rarely the central nervous system. Subarachnoid and intracerebral hemorrhage in CSS patients is extremely rare; however, clinicians should consider that CSS may be a cause of intracranial hemorrhage and its high rate of mortality and morbidity. The authors report on two cases of subarachnoid and intracerebral hemorrhage with CSS and discuss a brief review of CSS. Multiple cerebral and cerebellar infarcts as the first clinical manifestation in a patient with Churg-Strauss syndrome: Churg-Strauss syndrome CSS is a rare autoimmune disease with small-vessel vasculitis.
Neurological manifestation of CSS is common. Central nervous system is less frequently involved than that of peripheral nervous system. We report a case of year-old man who presented with acute onset of right hemiparesis and impaired cognition. The presence of hypereosinophilia, asthma, sinusitis and extravascular eosinophil accumulation led to the diagnosis of Churg-Strauss syndrome. Brain magnetic resonance imaging MRI revealed multiple infarcts in bilateral cerebral and cerebellar hemispheres. The neurophysiology study did not reveal peripheral neuropathy. The patient was effectively treated with methylprednisolone, cyclophosphamide and warfarin.
Symptoms and signs of central nervous system can be the initial neurological manifestation of CSS patients. CSS should be considered while patients have stroke and hypereosinophilia. In our patient, there is a good response to timely steroid, immunosuppressant and anticoagulant therapies. Churg-Strauss syndrome and persistent heart failure: Churg-Strauss syndrome CSS is a rare small-vessel vasculitis typically associated with adult-onset asthma, peripheral and tissue hypereosinophilia, migratory pulmonary infiltrates, upper respiratory tract symptoms, and clinical evidence of systemic vasculitis.
When the news of the decree spread, many of the vagabonds and beggars quickly departed the city. The police rounded up the rest, confined the men who were able to work in a large house in the faubourg Saint-Victor, and the women and children in another large house in faubourg Saint-Marcel. Those with incurable illnesses or unable to work were taken to a third house in the faubourg Saint-Germain. They were supposed to awake at five in the morning and to work from 5: The work consisted of grinding wheat, brewing beer, cutting wood, and other menial tasks; the women and girls over the age of eight were employed by sewing.
The city judged the program a success, and acquired three large new buildings for the beggars. But within four years the program was abandoned; the work was poorly organized, and many of the beggars simply escaped. Renaudot, a Protestant and a physician, founded the first weekly newspaper in France, La Gazette in Based on the newspaper, he founded the first employment bureau in Paris, matching employers and job-seekers. He organized public conferences on topics of public interest. He also organized the first free medical consultations for the poor. This put him into opposition with the medical school of the University of Paris, which denounced him.
After the death of his protectors, Richelieu and Louis XIII, the University had his medical license taken away, but eventually the University itself began to offer free medical consultations to the poor. Another pioneer in helping the Paris poor during the period was Vincent de Paul. As a young man, he had been captured by pirates and held as a slave for two years. When he finally returned to France, he entered the clergy and became chaplain to the French prisoners in Paris sentenced to the galleys.
He persuaded wealthy women from noble families to prepare and distribute meat broth to the patients, and to help the patients with their needs. He instructed the women visiting the hospital to dress simply, and to speak to the poor with "humility, gentleness and cordiality. The young women, dressed in gray skirts and white cornettes , carried pots of soup to the poor of the neighborhoods. The first house of the order was at La Chapelle. Between and , eight more houses of the order were opened to serve food to the poor. Four hundred unwanted babies were abandoned each year at the maison de la Couche, or maternity hospital, where most died in a very short time.
There was only one nurse for four or five children, they were given laudanum to keep them from crying, and they were often sold to professional beggars, who used them to inspire pity and donations. In , he persuaded wealthy Parisians to donate money to establish a home for found children on rue des Boulangers, near the porte Saint-Victor. De Paul and the sisters of the order made visits to churches to bring babies abandoned there at night to the new home.
His work came to the attention of the King and Queen, who provided funding in to build a large new home for abandoned children near Saint-Lazare. De Paul died in In , he was canonized as a saint by the Catholic Church. Beside the beggars and the poor there was another underclass in Paris, composed of thieves. They often were expert in cutting the cords of the purses that wealthy Parisians carried around their necks and running off with them. They also sometimes pretended to be blind or lame, so they could attract charity from the Parisians.
In the 17th century the most famous residence of such thieves was the Cour des Miracles , or Courtyard of Miracles, located between rue Montorgueil, the convent of the Filles-Dieu, and rue Neuve-Saint-Saveur, in the center of the city. It was named because its residents often appeared to blind or lame when on the streets but were perfectly whole when they returned home.
It was described by the 18th-century Paris historian Sauval,  based on his father's description of a visit to the Courtyard. I saw a house half-buried in mud, crumbling with age and rot, which had only a fraction of its roof tiles, where however were lodged more than fifty families, with all their legitimate, illegitimate and undetermined children. I was assured that in this house and its neighbors there were more than five hundred large families living one on top of the other". The courtyard was a school of crime; the children were taught the best techniques for stealing purses and escaping, and were given a final examination of stealing a purse in a public place, such as the Cemetery of Saints-Innocents, under the scrutiny of their teachers.
The Courtyard had its own King, laws, officers and ceremonies. The young inhabitants became expert in not only stealing, but also in simulating blindness, gangrene, rabies, and a wide variety of horrifying wounds and illnesses. In , the city wanted to build a street through the Courtyard to connect rue Saint-Sauveur and rue Neuve-Saint-Sauveur, but the workmen were showered with rocks and assaulted and beaten by the residents of the Courtyard, and the project was abandoned.
In , shortly after being named Lieutenant-General of Police, de la Reynie decided to finally put an end to the Courtyard. He gathered one hundred fifty soldiers, gendarmes and sappers to break down the walls, and stormed the Courtyard, under a barrage or rocks from the residents. The inhabitants finally fled, and de la Reynie tore down their houses. The empty site was divided into lots and houses constructed.
Sauval's description of the Courtyard was the source for Victor Hugo's courtyard of miracles in his novel Notre-Dame de Paris , though Hugo moved the period from the 17th century to the Middle Ages. King Henry IV, who frequently was short of money, made one decision which was to have fateful consequences for Paris for two centuries to follow. At the suggestion of his royal secretary, Charles Paulet, he required the hereditary nobility of France to pay an annual tax for their titles.
This tax, called "la Paullete" for the secretary, was so successful that it was expanded, so that wealthy Parisians who were not noble could purchase positions which gave them noble rank. When kings needed more money, they simply created more positions. By , during the reign of Louis XIV, there were 45, positions of state. It cost 60, livres to become President of the Parlement of Paris, and , livres to be President of the Grand Council.
The status of bourgeois was granted to those Parisians who owned a house, paid taxes, had long been resident in Paris, and had an "honorable profession", which included magistrates; lawyers and those engaged in commerce, but excluded those whose business was providing food. Almost all the Provosts for generations came from among about fifty wealthy families. Elections were held for Provost every two years, and for the four positions of echevins , or deputies.
After the election, held on August 16 of even-numbered years, the new Provost and new echevins were taken by carriage to the Louvre where they took an oath in person to the King and Queen. The position of Provost of the Merchants had no salary, but it had many benefits. The Provost received , livres a year for expenses, he was exempt from certain taxes, and he could import goods without duty into the city. He wore an impressive ceremonial costume of a velour robe, silk habit and a crimson cloak, and was entitled to cover his horse and his dress his household servants in a special red livery.
He had his own honor guard, made up of twelve men chosen from the bourgeoisie, and he was always accompanied by four of these guards when about the city on official business. The Provost was assisted by twenty-four conseillers de ville , a city council, who were chosen by the Provost and echevins, when there was an opening. Beneath the Provost and echevins there were numerous municipal officials, all selected from the bourgeoisie; two procureurs, three receveurs, a greffier, ten huissiers, a Master of Bridges, a Commissaire of the Quais, fourteen guardians of the city gates, and the Governor of the clock tower.
Most of the positions of the Bureau of the City had to be purchased with a large sum of money, but once acquired, many of them could be held for life. Each of the sixteen quarters or neighborhoods of the city also had its own administrator, called a quarternier , who had eight deputies, called dizainiers. These positions had small salaries but were prestigious and came with generous tax exemptions. The Bureau of the City had its own courts and prison. The city officials were responsible for maintaining order in the city, fire safety, and security in the Paris streets.
They assured that the gates were closed and locked at night, and that the chains were put up on the streets. They were responsible for the city's small armed force, the Milice Municipale and the Chevaliers de la guet , or night watchmen. The local officials of each quarter were responsible for keeping lists of the residents of every house in their neighborhoods, and also keeping track of strangers. They recorded the name of a traveler coming into the quarter, whether he stayed in a hotel, a cabaret with rooms, or a private home.
During the second half of the 17th century, most of the independent institutions of the Parisian bourgeois had their powers taken away and transferred to the King. In March the King created the position of Lieutenant General of the police, with his office at the Chatelet, and gave the position to La Reynie, who held it for thirty years, from to He was responsible not only for the police, but also for supervising weights and measures, the cleaning and lighting of the streets, the supply of food to the markets, and the regulation of the corporations, all matters which previously had been overseen by the merchants of Paris.
The last of the ancient corporations of Paris, the corporation of the water merchants, had its authority over river commerce taken away and given to the Crown in In , Louis XIV took away almost all of the real powers of the municipal government. The Provost of the Merchants and the Echevins were still elected by the Bourgeois, but they had no more real power. Selling positions in the city government became an effective way to raise funds for the royal treasury. All other municipal titles below the Provost and Echevins had to be purchased directly from the King. At the beginning of the 17th century, the most important industry of the city was textiles; weaving and dyeing cloth, and making bonnets, belts, ribbons, and an assortment of other items of clothing.
The dyeing industry was located in the Faubourg Saint-Marcel, along the River Bievre, which was quickly polluted by the workshops and dye vats along its banks. The largest workshops there, which made the fortunes of the families Gobelin, Canaye and Le Peultre, were dyeing six hundred thousand pieces of cloth a year in the midth century, but, because of growing foreign competition, their output dropped to one hundred thousand pieces at the start of the 17th century, and the whole textile industry was struggling.
They encouraged French businessmen to make the same luxury products in Paris. With this royal encouragement, the financier Moisset launched an enterprise to make cloth woven with threads of gold, silver and silk. It failed, but was replaced by other successful ventures. The first tapestry workshop was opened, with royal assistance, in the Louvre, then at the Savonnerie and at Chaillot. The Gobelins' enterprise of dyers brought in two Flemish tapestry makers in and began to make its own tapestries in the Flemish style.
Master craftsmen from Spain and Italy opened small enterprises to make high-quality leather goods. Workshops making fine furniture were opened by German craftsmen in the faubourg Saint-Antoine. A large factory was opened at Reuilly to produce and polish mirrors made by Saint-Gobain.
The most skilled artisans in Europe were recruited and brought to Paris. The Gobelins' workshops began to produce furniture for the royal residences as well as tapestries, while the Savonnerie Manufactory produced magnificent carpets for the royal palaces. The quality of the carpets, tapestries, furniture, glass and other products was unmatched; the problem was that it was nearly all destined for a single client, the King, and his new residence at Versailles.
The royal manufactories were kept going by enormous subsidies from the royal treasury. The greatest resource of the Paris economy was its large number of skilled workers and craftsmen.
Since the Middle Ages, each profession had had its own corporation, which set strict work rules and requirements to enter the profession. There were separate corporations for drapers, tailors, candle-makers, grocer-pharmacists, hat-makers, bonnet-makers, ribbon-makers, saddle-makers, stone carvers, bakers of spice breads, and many more. Doctors and barbers were members of the same corporation.
Access in many professions was strictly limited to keep down competition, and the sons of craftsmen had priority. The palace was no longer occupied by the King, and had become the administrative headquarters of the kingdom, occupied by the courts, the treasury, and other government offices. The small shops in the gallery sold a wide variety of expensive gowns, cloaks, perfumes, hats, bonnets, children's wear, gloves, and other items of clothing. Books were another luxury items sold there; they were hand-printed, expensively bound, and rare. Clocks and watches were another important luxury good made in Paris shops.
Access to the profession was strictly controlled; at the beginning of the 17th century, the guild of horlogers had twenty-five members. Each horloger was allowed to have no more than one apprentice, and apprenticeship lasted six years. By , under new rules of the guild, the number of masters was limited to seventy-two, and the apprenticeship was lengthened to eight years. Two families, the Martinet and the Bidauld, dominated the profession; they had their workshops in the galleries of the Louvre, along with many highly skilled artists and craftsmen.
Nearly all the clock and watchmakers were Protestants; when Louis XIV revoked the Edict of Nantes in , most of the horlogers refused to renounce their faith and emigrated to Geneva, England and Holland, and France no longer dominated the industry. For most of the 17th century Paris was governed by two Cardinals, Richelieu and Mazarin, and Paris was a fortress of the Roman Catholic faith, but it was subject to considerable religious turmoil within. His elder brother had been Bishop of Paris before him, and he was succeeded as Archbishop by his nephew; members of the Gondi family were the bishops and archbishops of Paris for nearly a century from to The hierarchy of the Church in Paris were all members of the higher levels of the nobility, with close connections to the royal family.
As one modern historian noted, their dominant characteristics were "nepotism While the leaders of the church in Paris were more concerned with high political matters, the lower levels of the clergy were agitating for reform and more engagement with the poor. The Vatican had decided to create seminaries in Paris to give priests more training; the Seminary of Saint-Nicolas-de-Chardonnet was opened in , the Seminary of Sant-Magliore in , the Seminary of Vaugirard in , before moving to Saint-Sulpice in ; and the Seminary of Bons-Enfants also opened in The seminaries became centers for reform and change.
Thanks in large part to the efforts of Vincent de Paul , the parishes became much more actively involved in giving assistance to the poor and the sick, and giving schooling to young children. Confreries or brotherhoods of wealthy nobles, such as the Compagnie du Saint-Sacrement, were formed, to assist the poor in Paris, to convert Protestants, and to send missions abroad to convert the inhabitants of new French colonies.
More than eighty religious orders also established themselves in Paris; sixty orders, forty for women and twenty for men, were established between and These included the Franciscans at Picpus in , the Congregation of the Feuillants next to the gates of the Tuillieries palace in ; the Dominican Order at the same location in , and the Carmelites from Spain in at Notre-Dame des Champs.
They became particularly useful, because, before the formation of a formal fire department by Napoleon, they were the principal fire-fighters of the city. The arrival of all these new orders, directed from Rome and entirely out of the control of the Archbishop of Paris, caused the alarm and eventually the hostility of the Paris church establishment. Followers of the church in Paris were divided by a new theological movement called Jansenism , founded by a Dutch theologian named Cornelius Jansen , who died in It was based at the Port-Royal-des-Champs Abbey , and was based on variations of the doctrines of original sin and predestination which were strongly opposed by the Jesuits.
Cardinal Richelieu had the leader of the Jansenists in Paris put in prison in , and the Jesuits persuaded Pope Innocent X to condemn Jansenism as a heresy in , but the doctrines spread and Jansenism was broadly tolerated by most Parisians, and contributed to undermining the unquestioned authority of the church which followed in the 18th century. On August 1, , at the request of his chancellor, Sully, Henry IV granted the Protestants of Paris permission to build a church, as long as it was far from the center of the city.
The new church was constructed at Charenton , six kilometers from the Bastille. In , there were an estimated eight thousand five hundred Protestants in the city, or about two percent of the population. Louis XIV revoked the Edict of Nantes in , leading to an important exodus of Protestants from the city, and forcing those who remained to practice their faith in secret. The Jewish population of Paris in the 17th century was extremely small, following centuries of persecution and expulsion; there were only about a dozen Jewish families in the city, coming originally from Italy, Central Europe, Spain or Portugal.
At the beginning of the 17th century, the nobles and wealthy Parisians traveled by carriage, horse, or in a chair inside an elegant box carried by servants. In , there were three hundred carriages in the city. Less fortunate travelers had to go on foot. Paris could be crossed on foot in less than thirty minutes. However, it could be a very unpleasant walk; the narrow streets were crowded with carts, carriages, wagons, horses, cattle and people; there were no sidewalks, and the paving stones were covered with a foul-smelling soup of mud, garbage and horse and other animal droppings.
Shoes and fine clothing were quickly ruined. In about a new form of public transport appeared, called the fiacre , a coach and driver which could be hired for short journeys. The business was started by an entrepreneur from Amiens named Sauvage on the rue Saint-Martin. It took its name from the enseigne or hanging sign on the building, with an image of Saint Fiacre.
By , there were several different companies offering the service. In , a decree of the Parlement of Paris gave the exclusive rights to operate coaches for hire to an ecuyer of the King, Pierre Hugon, the sieur of Givry. In , the Parlement fixed the fare at twenty sous for the first hour and fifteen sous for each additional hour; three livres and ten sols for a half day, and four livres and ten sols if the passenger desired to go into the countryside outside Paris, which required a second horse.
In , fiacres were required to have large numbers painted in yellow on the sides and rear of the coach. In January the mathematician and philosopher Blaise Pascal , the inventor of one of the first calculating machines, proposed an even more original and rational means of transport; buying seats in carriages which traveled on a schedule on regular routes from one part of the city to another. He prepared a plan and the enterprise was funded by three of his friends, and began service in March Each carriage carried eight passengers, and a seat in one cost five sols.
The doors of the carriages had the emblem of the city of Paris, and the coachmen wore the city colors, red and blue. The coaches followed five different itineraries, including from rue Saint-Antoine to the Luxembourg by the Pont Neuf, from the Luxembourg to rue Montmartre, and a circular line, called "The Tour de Paris. The fiacre remained the main means of public transport until well into the 19th century, when it was gradually replaced by the omnibus, the horse-drawn tramway, and the eventually by the motorized fiacre, or taxicab. At the beginning of the century, the streets of Paris were dark at night, lit only here and there with candles or oil lanterns.
Lantern-bearers were located at posts eight hundred steps apart on the main streets, and customers paid five sols for each portion of a torch used, or for fifteen minutes of lantern-light. The company of torch and lantern bearers was in business until In , the royal government decided to go further, and to require the placement of lanterns in each quarter, and on every street and place, at the expense of the owners of the buildings on that street.
In the first year, three thousand oil lanterns were put in place. The system was described by English traveler, Martin Lister, in The lanterns are suspended from in the middle of the street at a height of twenty feet and at a distance of twenty steps between each lantern. The cords were attached to iron bars fixed to the walls, so they could be lowered and refilled with oil.