Multiple tracheobronchial mucosal lesions in two cases of Churg-Strauss syndrome

Churg-Strauss syndrome (CSS) is characterized by hypereosinophilia and a systemic necrotizing vasculitis seen almost exclusively in patients with asthma. The most common pathological findings in the chest in CSS are eosinophilic pneumonia, necrotizing vasculitis and granulomatous inflammation (extravascular granuloma). However, tracheobronchial mucosal lesions have rarely been reported in CSS. The authors report two patients with CSS who had multiple tracheobronchial mucosal lesions that were found by fibreoptic bronchoscopy. They were tiny nodular lesions and necrotizing bronchial inflammation with many eosinophils was observed upon pathological examination. The authors concluded that tracheobronchial mucosal lesions may be one of the manifestations of vasculitis seen in CSS.     

The lung in systemic vasculitis

Pulmonary vasculitis is usually caused by one of three disorders: (1) Wegener's granulomatosis (WG); (2) Churg-Strauss syndrome (CSS), or allergic angiitis and granulomatosis; or (3) a nonspecific small vessel systemic necrotizing vasculitis (SNV), or microscopic polyarteritis. WG, the most common cause of lung vasculitis, has features of a granulomatous vasculitis of the upper airway and lung and widespread small vessel vasculitis involving the kidneys and other organs. The features of pulmonary WG overlap with those of malignancy and infectious granulomatous lung disease; accurate diagnosis generally requires open lung biopsy. CSS is defined by the triad of asthma, eosinophilia, and systemic vasculitis. Easily accessible tissues should be biopsied, but the clinical features are so distinctive that tissue biopsy is not invariably required for diagnosis. CSS must be differentiated from other diseases that cause pulmonary infiltrates with eosinophilia, including infections. Nonspecific SNV causes diffuse alveolar hemorrhage due to pulmonary capillaritis. Concomitant segmental necrotizing glomerulonephritis is almost always present. Diagnosis is made by renal biopsy, compatible extrarenal features, exclusion of nonimmune causes of lung hemorrhage, and exclusion of WG to the extent possible.     

Churg-Strauss syndrome revealed by granulomatous acute pericarditis: two case reports and a review of the literature

BACKGROUND: Churg-Strauss syndrome (CSS) is a necrotizing systemic vasculitis with extravascular granulomas and eosinophilic infiltrates of small vessels. CSS is usually revealed by nonspecific signs of necrotizing vasculitis in a context of late-onset asthma and blood eosinophilia. It is considered a systemic vasculitis with the highest prevalence of cardiac involvement and can lead to rapid-onset heart failure due to specific cardiomyopathy. Pericardial effusion may also occur during CSS and is usually well tolerated. OBJECTIVE: The objective of these case reports was to indicate that CSS may present as tamponade, with or without other visceral involvement. METHODS: Among CSS patients treated during the past 10 years at 2 French university hospitals, we have identified and described 2 cases revealed by tamponade with pericardial biopsy-proven granulomatous vasculitis. We have also reviewed the international medical literature in PubMed on cardiac involvement in CSS. RESULTS: The first case report describes a 66-year-old man who had an isolated cardiac tamponade with both inflammatory syndrome and eosinophilia. Long-term remission was obtained with corticosteroids. The second case report describes a 46-year-old woman whose CSS presented with tamponade and associated central nervous system and myocardial involvement. Remission was obtained with corticosteroids and cyclophosphamide. In both cases, CSS was assessed by histological analysis of a pericardial sample. CONCLUSIONS: CSS may present as isolated cardiac tamponade. Whereas pericarditis with myocardial injury warrants immunosuppressive therapy, isolated pericarditis without other visceral involvement of poor prognosis only requires corticosteroid therapy.     

Postpartum Churg–Strauss syndrome with severe cardiac involvement: Description of a case and review of the literature

Churg–Strauss syndrome (CSS) is a rare small- or intermediate-vessel necrotizing vasculitis typically characterized by asthma, lung infiltrates, necrotizing granulomas, and hypereosinophilia. In this report, we describe the case of a 35-year-old woman who, during her third trimester of pregnancy, developed dyspnea and, after delivery, severe cardiac failure which required heart transplantation. Diagnosis of CSS was made after performing a myocardial biopsy. We have also undertaken a review of the English-language literature regarding previously reported cases of pregnancies in women suffering from Churg–Strauss syndrome with particular attention to those patients with cardiovascular involvement.     

Nephropathy,polyneuropathy, and gastroenteritis in a child with Churg–Strauss syndrome

Churg–Strauss syndrome (CSS) is a serious but rare pauci-immune vasculitis of small- and medium-sized blood vessels. It is commonly seen in association with bronchial asthma and/or allergic disorders. The syndrome is characterized by the presence of asthma, hypereosinophilia, and vasculitis in any part of the body. Vasculitis is often associated with significant distortion of normal functions. A rather severe case of CSS in an 8-year-old Nigerian girl with asthma and allergic rhinoconjunctivitis is reported. She presented with multiple morbidities, namely, vasculitic polyneuropathy and also nephritic–nephrotic syndrome that eventuated in acute renal failure after an onset of vasculitic gastroenteritis. Routine screening of all asthmatic patients for CSS is advocated.     

Syndrome de Churg-Strauss

Churg–Strauss syndrome (CSS) is a granulomatous, necrotizing vasculitis affecting small-sized blood vessels. The disease is characterized by asthma, eosinophilia and extra-respiratory manifestations. ANCA are found in one-third to half of the patients with CSS. The prognosis depends on whether there is cardiac and/or gastrointestinal involvement. Steroids are indicated in every case; in severely affected cases, an immunosuppressive agent, e.g. cyclophosphamide, should be prescribed as well. Nevertheless, the prognosis of CSS remains very good; the remission rate approaches 90%.     

Churg–Strauss syndrome presenting with massive pericardial effusion

We describe a case of Churg-Strauss syndrome (CSS) presenting with a massive pericardial effusion without overt myocardial dysfunction. A 60-year-old man was referred to our hospital because of exertional dyspnea and fever. Initial chest multidetector-row computed tomography showed a massive pericardial effusion. The presence of eosinophilia, infiltrates of both lungs, pathological evidence of necrotizing vasculitis associated with eosinophilic infiltration, and history of asthma fulfilled the criteria of CSS. Massive pericardial effusion can be the first manifestation of cardiac involvement in CSS.     

Small intestinal perforation in Wegener's granulomatosis

Wegener's granulomatosis is a disease characterized by necrotizing vasculitis and granulomatous inflammation. Gastrointestinal (GI) involvement is uncommon in this disorder. Only a few cases of perforation of GI tract have been reported, but vasculitis has not been demonstrated to be a cause of perforation in these cases. We report a case of Wegener's granulomatosis in which a single perforation in the terminal ileum was disclosed on laparotomy and active necrotizing vasculitis was found in the submucosal layer of the resected specimen.     

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.     

Churg-Strauss syndrome: 2005–2008 update

Churg-Strauss syndrome (CSS) is a rare necrotizing small-vessel vasculitis associated with eosinophil-rich granulomatous inflammation of tissues and vessels and is also associated with asthma and eosinophilia. Epidemiologic studies continue to show that CSS is the rarest of the necrotizing small-vessel vasculitides. However, it is not possible to know with any certainty if there has been an increase in incidence. There has been an attempt to divide the patients with CSS into an antineutrophil cytoplasmic antibody-positive and cytoplasmic antibody-negative group. The former group has an increased frequency of renal involvement, parenchymal pulmonary disease, constitutional symptoms, and peripheral and central nervous system involvement, whereas the latter group has more frequent cardiac disease. The role of eosinophils and antineutrophil cytoplasmic antibodies remains poorly defined but provocative. Leukotriene receptor antagonists do not appear to induce CSS but facilitate the tapering of glucocorticoids, which unmasks the condition. Glucocorticoids and cyclophosphamide remain the foundation of treatment for vasculitis, but there are other promising and less toxic alternatives on the horizon.     

Allergic granulomatosis and angiitis in the absence of asthma and blood eosinophilia: a rare presentation of limited Churg-Strauss syndrome

Allergic granulomatosis and angiitis, also known as Churg-Strauss syndrome (CSS), is an uncommon vasculitis of unknown etiology. We report a 21-year-old male patient with fatigue, dry cough, and progressive dyspnea. He had no history of asthma or eosinophilia. Thorax computed tomography showed bullous/cystic areas with thin walls in varying sizes (5–15 mm). Histopathological examination of the open lung biopsy revealed granulomatous infiltration with histiocytes and eosinophilic leukocytes. This extremely rare variant of CSS is discussed.     

Churg–Strauss syndrome associated with leukotriene receptor antagonists (LTRA)

Churg–Strauss syndrome (CSS) is a rare vasculitic disorder that generally occurs in patients with bronchial asthma. CSS is being increasingly recognized in asthmatic patients treated with leukotriene receptor antagonists. However, the nature of this relationship remains to be elucidated. The present report describes three asthmatic patients who developed clinical manifestations highly suggestive of CSS, although one patient lacked the presence of eosinophilia. The patient, however, exhibited biopsy-proven cutaneous necrotizing vasculitis, which improved after withdrawal of montelukast. The second patient presented with systemic constitutional signs including fever, malaise, arthralgias, clinical jaundice, peripheral blood eosinophilia, and biopsy-proven eosinophilic hepatitis. The third patient also had circulating eosinophilia, scleritis, and arthritis. All patients improved after discontinuation of the leukotriene receptor antagonist (montelukast).     

Acute cholecystitis at initial presentation of polyarteritis nodosa

Polyarteritis nodosa (PAN) is a systemic vasculitis of unknown etiology. Although gastrointestinal involvement may be seen in half of the cases of PAN, vasculitis of the gallbladder at the disease onset is a rare presentation. We report a case of a 48-year-old man who was admitted due to acute cholecystitis. He had complained of myalgia, fever and weight loss for about one month prior to admission. At physical examination, mild hypertension and calf pain were noted. He underwent a cholecystectomy; histopathological evaluation disclosed necrotizing vasculitis suggestive of PAN. We emphasize that cholecystitis may be part of the initial presentation of systemic vasculitis.     

Two cases of rheumatoid arthritis complicated with vasculitis-induced ischemic enterocolitis

Episodes of catastrophic entero-colitis associated with mesenteric vascular insufficiency in patients with rheumatoid arthritis(RA) have rarely been recorded thus far. We herein report two cases of RA complicated with severe attacks of entero-colitis presumably due to mesenteric vasculitis. Surgical intervention was necessary in the first case, while the second patient recovered well only through conservative therapy. Case 1: A 74-year-old man with history of RA since 1985 started to complain of abdominal discomfort and nausea early in February, 1989. On February 12, Episodes of tarry stool developed. Rapid down-hill clinical course prompted laparotomy under the clinical diagnosis of peritonitis. Ischemic changes were observed at the ileum end, the entire length of which was 120 cm orally from the cecum. The site was resected. Multiple linear and aphthoid ulcer lesions were discovered throughout the entire lumen. Histopathologically, evidence of necrotizing vasculitis such as fibrinoid necrosis and mural thrombi was demonstrated in small arteries of the submucosal layer underlying the ulcer lesion. Case 2: A 63-year-old woman who had been suffering from RA since 1980 noticed the onset of nausea, abdominal pain and bloody diarrhea in July, 1989. Colonoscopy examination revealed multiple linear and aphthoid ulcers in the sigmoid colon which was presumed to be due to ischemia. Laboratory evaluation at that time demonstrated hypocomplementemia, positive circulating immune complex and high titer of anti-DNA antibody. Corticosteroid therapy with moderate dose was successful in alleviation of all the abnormal findings and the patient fully recovered three months after her initial GI episode.     

Hypereosinophilic syndrome associated with antineutrophil cytoplasmic antibody]

A 68-year-old man was admitted to our hospital with complaints of fever, cough, and shortness of breath. He had several erythematous maculae on the trunk and experienced hypesthesia in his lower extremities. Laboratory data showed marked eosinophilia (20,235/mm3) and enhanced hepatobiliary enzymes. Chest X-ray films and computed tomographic scans revealed diffuse patchy infiltrative changes in the lungs. Histologic findings confirmed eosinophilic infiltration of the skin, liver, and lungs. A diagnosis of hypereosinophilic syndrome (HES) was made in accordance with clinical criteria proposed by Chusid et al. The patient was positive for antineutrophil cytoplasmic antibodies (a marker for vasculitis). This suggested a clinical picture resembling Churg-Strauss syndrome (CSS) despite the lack of bronchial asthma. The findings in this report could contribute to a better understanding of the diversity of HES cases, several of which are considered to represent a continuum of pathologies sharing an etiology similar to that of CSS.     

An aggressive and lethal course of Churg–Strauss syndrome with alveolar hemorrhage,intestinal perforation,cardiac failure and peripheral neuropathy

Churg–Strauss syndrome (CSS) is a rare type of necrotizing vasculitis affecting small to medium-sized vessels typically characterized by asthma, lung infiltrates, necrotizing granulomas and hypereosinophilia. Herein, we describe a case of CSS presenting severe and aggressive course. A 35-year-old male patient with weight loss, dyspepsia, dyspnea and hemoptysis was admitted. The laboratory analyses indicated a remarkable eosinophilia, elevated levels of serum total IgE and positive cANCA. Thorax CT findings were suggestive of alveolar hemorrhage. Bronchoalveolar lavage revealed alveolar hemorrhage with eosinophilia and transbronchial lung biopsy showed eosinophilic vasculitis. Cardiac enzymes were increased and murmurs were audible revealing cardiomyopathy proven by echocardiography. Pulse cyclophosphamide and methyl prednisolone was immediately started. On the 21st day, intestinal perforation developed and urgent surgery was performed. During a follow-up, although a radiological improvement was observed in the chest X-ray, cardiac failure, peripheral neuropathy and skin lesions developed and high-dose intravenous immunoglobulin and anti-TNF therapy (adalimumab) were applied. Despite the therapy, he died from heart failure and septicemia at 68th day of therapy.     

Churg Strauss syndrome after introducing oral steroid to inhaler: a report of three cases

The tetrad of bronchial asthma, severe sinusitis, nasal polyp, eosinophilia, and systemic vasculitis is the main feature of allergic granulomatosis and angitis (Churg- Strauss Syndrome). This vasculitis is usually seen idiopathic in patients with a long history of asthma; oral steroids using steroid inhalers, vaccination and desensitization might be triggering factors. Drugs such as leukotriene receptor antagonists (LTRAS), penicillin, sulphonamides, anticonvulsants and thiazides have also been implicated. By presenting the cases in this article, the authors suggest that some cases of CSS may be partially or totally suppressed by corticosteroid therapy of asthma for long periods and replacing oral steroid by inhaler will reveal a pathologic condition of CSS, called frustes CSS forms. We report three subjects with asthma who had been receiving previously multiple corticosteroid courses for control, but when systemic corticosteroids were discontinued or switched over to steroid inhaler, the patients developed a similar syndrome.     

Churg-Strauss syndrome with pulmonary eosinophilia and intrapulmonary lymph nodes 2 years before the onset of vasculitis]

We report a case of a 55-year-old man who had been treated for bronchial asthma diagnosed at the age of 51. One year following diagnosis, chest X-ray films disclosed nodular shadows. Biopsy specimens obtained by video-assisted thoracoscopic surgery were histopathologically identified as intrapulmonary lymph nodes. Three years after the initial diagnosis, the patient experienced sensory disturbance of the lower extremities, low-grade fever, and weight loss. At this point he was admitted to our hospital. On admission, physical examination and clinical investigations showed peripheral eosinophilia and signs of vasculitis. Specimens obtained by transbronchial lung biopsy and bronchoalveolar lavage showed strong evidence of tissue damage with infiltration of eosinophils but no evidence of necrotizing vasculitis or extra-vascular granuloma. Churg-Strauss syndrome (CSS) was diagnosed, and treatment was initiated with prednisolone at a dose of 60 mg/day. Except for the sensory disturbances in the lower extremities, after a few days of treatment the patient's symptoms subsided and his clinical data improved. This case was clinically important because pulmonary eosinophilic infiltration into vessel walls was confirmed a year after the diagnosis of bronchial asthma, and 2 years before the patient demonstrated signs of vasculitis. Further, it was a very rare case of CSS in which intrapulmonary lymph nodes had developed beneath the visceral pleura despite the absence of a history of heavy smoking, thus suggesting continuous stimulation by some as yet unknown antigen.     

Repressed ileal artery aneurysms in Churg-Strauss syndrome following combination treatment with glucocorticoid and cyclophosphamide

Churg-Strauss syndrome (CSS) is a systemic small-vessel vasculitis characterized by asthma and eosinophilia. We report a case of CSS complicated by multiple aneurysms in the small intestine. A 57-year-old man was admitted to our hospital with fever and blood eosinophilila. During admission, aneurysms in his small intestine ruptured and partial resection of the small intestine was required. After combination therapy of glucocorticoid (GC) and cyclophosphamide (CYC) for 6 months, aneurysms in the small intestine disappeared. The gastrointestinal involvements usually result in poor prognosis in CSS. However, combination therapy of GC and CYC might be effective for remission of aneurysms caused by small vessel vasculitis.     

Rare manifestations of Churg–Strauss syndrome: coronary artery vasospasm, temporal artery vasculitis, and reversible monocular blindness—a case report

Churg–Strauss syndrome (CSS) is a rare illness with clinical findings characterized by asthma, eosinophilia, and vasculitis affecting medium and small-sized arteries and veins in a variety of organs. Involvement of the temporal arteries by non-giant cell eosinophilic vasculitis in CSS is quite rare and has only been published as isolated case reports or small patient series. Myocardial infarction due to coronary artery vasospasm is an unusual manifestation of CSS. We describe a case of a 39-year-old woman who had two myocardial infarctions due to severe coronary artery vasospasm and was diagnosed with CSS based on a temporal artery biopsy. During the course of her treatment, she also had another rare manifestation of CSS, monocular blindness reversible with immunosuppressive therapy.