Churg-Strauss syndrome associated with pregnancy

Churg-Strauss syndrome, an uncommon condition, occurs even more rarely in association with pregnancy. One month postpartum, a 25-year-old Japanese woman was hospitalized for dyspnea, fever, diarrhea, and complaints of dysesthesia in the right lower limb and right lumbar and abdominal region. Marked eosinophilia was present. Symptoms of bronchial asthma had developed early in the pregnancy, which concluded with delivery of a healthy baby at 39 weeks of gestation. Churg-Strauss syndrome was diagnosed and prednisolone was administered with a good response leading to remission. Three years after the first pregnancy, low-dose steroid therapy was continued through another pregnancy. The patient delivered another healthy baby at 39 weeks of gestation, this time with no exacerbation of symptoms.     

Churg-Strauss syndrome associated with omalizumab

Reports have suggested that the leukotriene receptor antagonists may cause Churg-Strauss syndrome following the tapering of oral corticosteroids or even in patients not treated with corticosteroids. We report a patient not treated with long-term corticosteroids and who developed Churg-Strauss syndrome with myocarditis and central nervous system involvement following the initiation of omalizumab. Given the possible role of omalizumab in the development of this vasculitis in our patient, we recommend careful monitoring of the appearance of Churg-Strauss symptoms and rising eosinophil count in patients treated with omalizumab.     

Churg-Strauss syndrome associated with hypersensitivity to acetaminophen

Acetaminophen is a widely used antipyretic drug. We describe a 64-year-old Japanese woman who developed typical Churg-Strauss syndrome after frequent use of acetaminophen. Following the ingestion of acetaminophen, she exhibited various allergic reactions such as asthmatic attacks, pyrexia and petechiae on legs. In the lymphocyte transformation test, a positive reaction to acetaminophen was detected. A muscle biopsy revealed massive extravascular eosinophil infiltration and a necrotizing vasculitis. Hypersensitivity to acetaminophen may be implicated in the development of Churg-Strauss syndrome in this case.     

Churg-Strauss syndrome complicated by colon erosion, acalculous cholecystitis and liver abscesses

We report on a case of Churg-Strauss syndrome (CSS) with colon erosion, cholecystitis and liver abscesses. A 21-year-old woman with a history of bronchial asthma for 3 years was admitted with a complaint of abdominal pain. Laboratory findings included remarkable leukocytosis and eosinophilia, and a colonoscopy revealed erosion from the rectum to the ileocecal region. In addition, a colonic biopsy specimen showed necrotizing vasculitis and marked eosinophilic infiltration. On the basis of the clinical features and histopathological findings, she was diagnosed with CSS and subsequently treated with oral prednisolone, after which the eosinophilia and abdominal pain disappeared. However, on the 15th d in hospital she developed cholecystitis and liver abscesses. She was therefore treated with antibiotics and as a result went into clinical remission.     

Acute acalculous cholecystitis associated with primary antiphospholipid antibody syndrome. Cure with low-molecular-weight heparin and antivitamin K

We report the first case of acute acalculous cholecystitis associated with primary antiphospholipid-antibody syndrome. The diagnosis was serological and was based on positive tests for lupus anticoagulant or anticardiolipin antibodies. The treatment was exclusively medical. Cholecystitis was cured with low-molecular weight heparin and oral anticoagulants. A rapid diagnosis can prevent lack of therapeutic errors such as surgery, antibiotherapy or corticotherapy, and long-term anticoagulant treatment can be proposed to prevent recurrent thrombosis.     

Allergic granulomatosis and angiitis (Churg-Strauss syndrome) presenting as acute acalculous cholecystitis

A patient with bronchial asthma and hypereosinophilia developed acute acalculous cholecystitis. Histopathologic study of the surgically removed gallbladder revealed allergic granulomatosis and angiitis (Churg-Strauss syndrome). Allergic granulomatosis and angiitis having acute acalculous cholecystitis as an initial manifestation is rare. It should be recognized that acute acalculous cholecystitis may be the initial sign of a systemic vasculitis, specifically allergic granulomatosis and angiitis.     

Cytomegalovirus cholecystitis and colitis associated with the acquired immunodeficiency syndrome

Cytomegalovirus (CMV) is an important cause of acalculous gangrenous cholecystitis in immunocompromised persons. We report a case of acalculous acute cholecystitis and active colitis associated with CMV in a patient suffering from the acquired immune deficiency syndrome. The condition was treated successfully with surgery and 9-(1,3,-dihydroxy-2-propoxymethyl)guanine intravenously.     

Acute acalculous cholecystitis associated with acute hepatitis A virus infection

Although acute cholecystitis in the context of acute hepatitis A virus (HAV) infection is extremely rare, previous cases have been reported. However, this manifestation of HAV infection is little known. In the present article, we briefly review previously documented cases and present a new case. We report the case of a 39-year-old woman with fever, abdominal pain and moderately elevated transaminase levels who developed jaundice and peritoneal irritation. Diagnosis of acute cholecystitis was given by abdominal ultrasound and magnetic resonance imaging. The patient underwent surgery. In the postoperative period, positive IgM antibody titers for HAV were obtained, confirming the diagnosis of HAV infection.     

Churg-Strauss syndrome

Churg-Strauss syndrome is a systemic ANCA-associated vasculitis arising almost exclusively in patients with a pre-existent asthma. Common clinical manifestations are marked blood eosinophilia, asthma, chronic sinusitis, cardiomyopathy, pulmonary infiltrates, gastrointestinal complaints and a multiplex neuropathy. The morphological substrate is an eosinophilic necrotizing vasculitis.Other eosinophilic disorders such as parasitic diseases, allergies and idiopathic hyper-eosinophilic syndrome have to be excluded. The mainstay of therapy is high-dose corticosteroids with the addition of cytotoxic drugs in patients with poor prognosis.     

Churg-Strauss syndrome

PURPOSE OF REVIEW: Churg-Strauss syndrome is a small-vessel necrotizing vasculitis typically characterized by asthma, lung infiltrates, extravascular necrotizing granulomas and hypereosinophilia. The most recent clinical studies on its pathogenesis and therapeutic management are reviewed here. RECENT FINDINGS: French and Italian clinical studies found that the clinical characteristics of patients with Churg-Strauss syndrome differed according to their antineutrophil cytoplasmic autoantibody status: cardiomyopathy predominated in antineutrophil cytoplasmic autoantibody-negative patients while necrotizing glomerulonephritis was more often observed in antineutrophil cytoplasmic autoantibody-positive patients. These histologically documented findings suggest the existence of different Churg-Strauss syndrome subtypes, characterized by the predominance of distinct pathogenetic mechanisms. To date, following the therapeutic recommendations for Churg-Strauss syndrome (i.e. corticosteroids and, when required, immunosuppressants), patient outcomes are good, with 5-year survival exceeding 90%, but often with the need to continue low-dose corticosteroids to control residual asthma. SUMMARY: The precise pathogenetic mechanisms of Churg-Strauss syndrome are only partly elucidated. Recent results suggest that antineutrophil cytoplasmic autoantibodies are probably more involved in the vasculitic manifestations of Churg-Strauss syndrome (e.g. glomerulonephritis) whereas eosinophil tissue infiltration and associated cytotoxicity would be responsible for cardiomyopathy. If confirmed, these results could support individual therapeutic stratification according to the clinical pattern. Furthermore, some patients may benefit from new biologic therapies under development, for example antiinterleukin-5 or antiimmunoglobulin E monoclonal antibodies.     

Churg-Strauss综合征

Churg-Strauss综合征(Churg-Strauss syndrome,CSS)是一种血管炎性疾病,表现为全身小至中等血管坏死性血管炎、血管内外肉芽肿形成、外周血嗜酸粒细胞增多以及组织及血管周围嗜酸粒细胞浸润。该病又称为变应性肉芽肿血管炎,但国内外文献多以CSS描述本病。CSS发病率为0.5/10万~6     

变应性肉芽肿性血管炎

变应性肉芽肿性血管炎 ,是系统性血管炎变 ,以哮喘、坏死性血管炎、血管外肉芽肿、外周血嗜酸粒细胞增多和多器官组织嗜酸粒细胞浸润为特征。临床少见 ,根据组织病理确诊的病例统计 ,发生率约 2 4 / 10 0万人口。因首先由Ｃｈｕｒｇ和Ｓｔｒａｕｓｓ两位病理学家描述 ,通常又称Ｃｈｕｒｇ Ｓｔｒａｕｓｓ综合征 (Ｃｈｕｒｇ Ｓｔｒａｕｓｓｓｙｎｄｒｏｍｅ ,ＣＳＳ)1　病因和发病机制　　多数ＣＳＳ病因不明。有个案报道吸入变应原 ,如放线菌 ,以及使用别嘌呤醇、抗惊厥制剂等药物可诱发ＣＳＳ ,但极少见。自 1996年扎鲁斯特上市应用以来 …     

Churg-Strauss syndrome

Churg-Strauss syndrome is a rare diffuse vasculitis that is almost invariably accompanied by severe asthma. Although overall prognosis is good, and treatment with prednisone alone or in combination with immunosuppressive drugs is usually successful, severe asthma typically persists. Diffuse organ involvement of Churg-Strauss syndrome, especially cardiovascular and rare involvement of the CNS and renal system, suggests a poorer prognosis than usual, and can be fatal. The cause of Churg-Strauss syndrome is unknown, but its characteristic histological findings and association with asthma distinguish it from other vasculitides. Controversy surrounds the use of asthma drugs-especially antileukotrienes--and development of the disorder. We review the epidemiological evidence for an association of drug treatment with Churg-Strauss syndrome, the diverse diagnostic and pathological criteria for this syndrome, and treatment options.