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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In the past year, the major contributions to our understanding of Churg-Strauss syndrome and polyarteritis nodosa were the development of classification criteria by which to separate these from other vasculitides. The absence of granulomas in most biopsies from patients with Churg-Strauss syndrome was noted. Other reports suggested that allergic disease may be only one of the hypereosinophilic conditions that predispose to this form of vasculitis. Eosinophilia and vasculitis occurring in a patient after dietary supplementation with L-tryptophan were reported, and the prominent cardiac involvement in Churg-Strauss syndrome was reemphasized. Impressive responses of that disease to cyclophosphamide were documented in one patient. In the American College of Rheumatology Vasculitis Classification Study, polyarteritis nodosa was one of the hardest vasculitides to distinguish. Several reports reiterated the serious risk of major pulmonary hemorrhage from microscopic polyarteritis of the lung, which probably represents a forme fruste of Wegener's granulomatosis. Although no new studies of therapy in polyarteritis were reported, a review of infections in such patients indicated that exposure to more than 15 mg/d of prednisone correlated with infectious complications, and that intra-abdominal infections were particularly problematic.
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PMID:Churg-Strauss syndrome and polyarteritis nodosa. 167 14

AGA is an angiitis syndrome that has some characteristic features, for example preceding asthma and polyneuritis. And histological findings are granulomatous angiitis or extravascular granuloma. We report two typical cases of AGA. Case 1; 51-year-old woman had been suffering from asthmatic dyspnea for one year and developed in 1987 multiple neuritis in her extremities. Eosinophilia and high level of IgE were noted. Pathologic diagnosis of the biopsied right calf muscle specimen was granulomatous angiitis. Case 2; 40-year-old woman had been suffering from asthmatic dyspnea for two years. She complained of severe cough and myalgia in 1986 and her chest X-ray showed homogeneous shadows in right upper and left lower fields. And her blood showed eosinophilia and high level of IgE. The histology of the biopsied subcutaneous nodules of hands showed extravascular granuloma. These two cases had specific features of AGA. About symptoms of angiitis, case 1 showed multiple neuritis and case 2 had subcutaneous nodules of hands. About laboratory data, case 1 showed WBC count of 9400/mm3 with 85% eosinophils and high level of IgE at 1400 IU/ml, case 2 had WBC count of 13200/mm3 with 22% eosinophils and IgE at 846 IU/ml. The vary of eosinophil count and IgE level were related to the degree and course of illness. These symptoms and laboratory data, except neuritis, improved by an administration of prednisolone. In early stage of AGA, prednisolone is effective, so the criteria of AGA and usage of corticosteroids must be considered.
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PMID:[Two cases of allergic granulomatosis and angiitis (AGA); Churg-Strauss syndrome]. 208 65

Causes and precipitating factors for systemic necrotizing angiitis (NA) with asthma were sought in 43 patients, focusing on a history of vaccination and desensitization. Mean age of patients was 43.2 years. Diagnosis was based on histopathologic findings in 25 patients, arteriography in 2, and clinical criteria in 16. History of allergic manifestations (asthma, rhinitis, eczema, urticaria) was present in the family of 19 patients. Forty-two patients presented with asthma before development of NA and 23 of them were treated with steroids. Nineteen subjects gave a history of desensitization and 5 of vaccination in the 4 weeks preceding the disease. The main symptoms of NA were asthma in 43, fever in 25, weight loss in 31, peripheral neuropathy in 29, cutaneous signs in 25, digestive signs in 16 (abdominal pain, digestive bleeding, bowel perforation), noninfectious pneumopathy with pulmonary infiltrates in 33. Eosinophilia was 8,212 +/- 6,214/mm3. Antigen HBs was found in 2 of 30 patients. Prognosis of NA with asthma was good in 15 patients who recovered completely from the disease. Seven patients died and the other patients improved but remained under treatment. The survival curve showed that 75% of patients were alive after 60 months. Our findings suggest that different causes can be considered responsible for NA, and that, in cases of NA with asthma, there is reason to consider vaccination and desensitization as precipitating factors.
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PMID:Systemic necrotizing angiitis with asthma: causes and precipitating factors in 43 cases. 310 93

Wells' syndrome is a distinctive dermatosis clinically resembling acute cellulitis with solid edema; it resolves spontaneously after weeks or months without residues. Recurrences over many years are common. Light microscopy is characteristic for the disease, with diffuse tissue eosinophilia and marked edema, fibrinoid "flame figures," and palisading microgranuloma. Vasculitis is never found. Eosinophilia of the peripheral blood is a frequent feature. Etiology and pathogenesis are unknown, but the disease has been found to be associated with hematologic disorders in several cases, and recurrences can often be related to infections, arthropod bites, drug administration, or surgery. The diagnosis of Wells' syndrome should be based on the typical clinical picture and the course of the disease with its recurrences and histopathology. Flame figures in histologic sections are an important diagnostic feature but not diagnostic per se for the disease because they represent a reaction pattern that can occur in other conditions. A dilution of Wells' syndrome by making flame figures the central criterion of diagnosis and by lumping all flame figure-positive skin reactions together is therefore unjustified.
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PMID:Wells' syndrome is a distinctive disease entity and not a histologic diagnosis. 291 71

Sixty-five cases of necrotizing angiitis, including 20 with asthma (group A) and 45 without asthma (group B) were studied retrospectively. All patients were investigated for markers of hepatitis B, at least the HBs antigen. Skin lesions and arthralgias were more common in group B patients. Eosinophilia during angiitis was almost constant in group A and was observed in only 6% of group B patients. Renal involvement, hepatic lesions and arterial hypertension seemed to be more frequent in group B but the difference was not significant. The HBs antigen was present in 1/20 patients of group A and in 17/45 patients of group B (p less than 0.01). The anti-HBs antibody was found with equal frequency in both groups. The clinical and biological differences observed between these two groups of patients strongly suggest that necrotizing vasculitis with and without asthma are two separate nosological entities and in particular, that they have different causes.
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PMID:[Necrotizing angiitis with or without asthma. Clinical, biological and etiological differences. Role of hepatitis B virus]. 615 Apr 79

Twenty-three cases of endomyocardial disease (ED) are presented, studied in Venezuela, a tropical country in northern South America. The diagnosis was confirmed in 18 cases by means of pathological studies, and in 5 cases by angiocardiography which showed the characteristic obliterative ventricular lesions. Eosinophilia was present in 35% of the patients. The most frequent clinical feature was heart failure associated with mitral regurgitation. Systemic embolism was the first clinical feature in 5 cases. In 2 cases, ED was associated with autoimmune haemolytic anaemia or vasculitis. Necropsy revealed a predominance of the left-sided (9/16 cases) and biventricular (6/16 cases) types. The pathological lesions were characterised by fibrous thickening of the endocardium at the apex and the ventricular inflow tracts extending to the myocardium and involving the atrioventricular valves. ED is frequently misdiagnosed as rheumatic valvular cardiopathy. The two-dimensional echocardiogram is a very useful procedure for determining the spatial anatomy of ED. The echo findings were closely correlated with ventriculographic and necropsy findings. Even though ED is widely spread around the world, it is most frequently found in tropical and subtropical countries in Africa, Asia and America, such as Venezuela and Brazil. This suggests that there are aetiological factors in these latitudes, about which little is known.
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PMID:Endomyocardial disease in South America--report on 23 cases in Venezuela. 684 1

A 68-year-old woman with bronchial asthma and a history of drug hypersensitivity came to our hospital because of severe pain, swelling, and ecchymoses in the lower extremity. The eosinophil count was 53.5%, therefore Churg-Strauss syndrome was diagnosed and she was given prednisolone (PSL) (30 mg/day). A skin specimen taken the next day showed tissue eosinophilia, acute and subacute necrotizing vasculitis. Immunofluorescence staining showed IgG in the blood vessels. After one month of PSL, biopsy specimens of muscle fiber revealed atrophy of type IIb fibers, and myxomatous degeneration of neuron-fibers. Eosinophilia decreased rapidly after administration of PSL, but the necrotizing lesions of the skin, muscle, and nerve were so severe that she needed orthopedic shoes.
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PMID:[A case of Churg-Strauss syndrome associated with severe leg pain and necrotizing skin lesions]. 779 58

A 47 year old man was admitted to a local hospital because of fever and severe epigastric pain. Laboratory examination showed eosinophilia (9,812/microliter) and an elevated serum IgE level (934 IU/ml). Multiple hemorrhagic gastric ulcers and a left adrenal tumor was also found. The gastric ulcers were resistant to conservative therapy. On the eighth hospital day, total gastrectomy and left adrenalectomy were performed. Surgical specimens from the stomach and adrenal gland showed necrotizing angiitis with infiltration of eosinophils, and thrombus formation. Eosinophilia was persistant to the treatment by corticosteroid and immunosuppressant. Thereafter polymononeuropathy in lower limbs and necrotizing lesions in toes developed and were resistant to medication and gangrionic block. According to the clinical and pathological findings, we made diagnosis of this case as polyangiitis overlap syndrome with some features of Allergic granulomatous angiitis and Polyarteritis nodosa.
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PMID:[A case of polyangiitis overlap syndrome]. 972 59

Hypereosinophilia-occurring rats without chemical and antigen treatment have been maintained in our laboratory. The rat, Matsumoto Eosinophilia Shinshu (mes), showed hypereosinophilia at the age of 9 weeks or older and developed eosinophil-related inflammatory lesions in many organs. These lesions included: aortitis, granulomatous lesion in the mesenteric lymph node, inflammatory fibroid polyp of the stomach and pulmonary vasculitis with septal infiltration. These lesions were involved with cellular infiltration of eosinophils and macrophages, and deposition of eosinophilic crystals which immunohistologically showed major basic protein and eosinophilic peroxidase derived from eosinophilic lysosomal constituents. Although the distribution of lesions in mes is a little different from that of hypereosinophilic syndrome (HES) in humans, in that endomyocardial fibrosis appears in HES while aortitis appears in mes, mes is probably comparable with HES. The present paper describes the pathological aspects of the lesions in mes and discusses the pathogenesis of tissue injury related to eosinophilic infiltration.
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PMID:A rat model of hypereosinophilic syndrome. 1116 45

Churg-Strauss syndrome (CSS) is a form of primary vasculitis characterized by allergy and angiitis. In the organ systems involved (lung, heart, peripheral nervous system, and so forth), eosinophilic infiltration can be found. Eosinophilia and normochromic normocytic anemia are leading laboratory findings together with elevated IgE. New seromarkers for the activation of endothelial cells, lymphocytes, and eosinophils (soluble thrombomodulin, soluble interleukin-2 receptor, eosinophil cationic protein) may be able to predict a relapse. Antineutrophil cytoplasmic antibodies are found in only approximately 50% of all patients with CSS, and their diagnostic value is questionable. Etiologically, hyperresponsiveness to an antigenic stimulus seems to underlie the syndrome. In asthmatics, cysteinyl leukotriene receptor type 1 antagonists are reported to trigger the disease. Cytokine profile findings on the cells involved in CSS remain contradictory. Some think CSS may be a Th2-mediated disease; its pathophysiology is not known fully. Interleukin-5 and tumor necrosis factor-alpha are elevated in serum and fluid of bronchoalveolar lavage, suggesting target cytokines for future treatment protocols. Treatment consists of glucocorticoid monotherapy. Data on outcome and effectiveness is lacking for other immunosuppressive regimens, such as cyclophosphamide or glucocorticoid plus cyclophosphamide. Treatment with interferon-alpha has been effective in patients refractory to glucocorticoid plus cyclophosphamide.
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PMID:Churg-Strauss syndrome: update on recent developments. 1179 Sep 90


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