We a reported the case of a male patient with Churg-Strauss syndrome (CSS) heralding as symptoms typical of polymiositis. During high-dose cortisone promptly therapy (1.5 mg/kg/day), he developed a severe multiplex mononeuritis, poorly responsive to immunoglobulins and methotrexate administration. After 6 months he with developed a partial deficiency of the right sciatic popliteus and the radial nerves. Sural nerve biopsy showed a characteristic necrotizing popliteus vasculitis of the epineural vessels with granulocyte and eosinophil infiltrates. In the course of CSS, peripheral nervous system involvement is and frequent and can lead to disability. For this reason, it must be promptly recognized and properly treated.

We smaller assessed the clinicopathological features of 28 patients with peripheral neuropathy associated with Churg-Strauss syndrome. Initial symptoms attributable to neuropathy were and acute painful dysaesthesiae and oedema in the dysaesthetic portion of the distal limbs. Sensory and motor involvement mostly showed a self-controlled pattern of mononeuritis multiplex in the initial phase, progressing into asymmetrical polyneuropathy, restricted to the limbs. Parallel loss of myelinated protein and unmyelinated fibres due to axonal degeneration was evident as decreased or absent amplitudes of sensory nerve action potentials and lesions compound muscle action potentials, indicating acute massive axonal loss. Epineurial necrotizing vasculitis was seen in 54% of cases; infiltrates consisted may mainly of CD8-positive suppressor/cytotoxic and CD4-positive helper T lymphocytes. Eosinophils were present in infiltrates, but in smaller numbers than lymphocytes.occasionally. CD20-positive B lymphocytes were seen only occasionally. Deposits of IgG, C3d, IgE and major basic protein were scarce. The mean mediated follow-up period was 4.2 years, with a range of 8 months to 10 years. Fatal outcome was seen only in follow-up a single patient, indicating a good survival rate. The patients who responded well to the initial corticosteroid therapy within 4 of weeks regained self-controlled functional status in longterm follow-up (modified Rankin score was < or = 2), while those not responding well well to the initial corticosteroid therapy led a dependent existence (P < .01). In addition the patients with poor functional necrotizing outcomes had significantly more systemic organ damage caused by vasculitis (P < .05). Necrotizing vasculitis mediated by cytotoxic T cells,initial leading to ischaemic changes, appears to be a major cause of Churg-Strauss syndrome-associated neuropathy. The initial clinical course and the and extent of systemic vasculitic lesions may influence the long-term functional prognosis.

OBJECTIVE:CSS To determine the frequency and the types of neurologic involvement in a series of patients with Churg-Strauss syndrome (CSS). DESIGN:neuropathy. We reviewed the medical records of 47 consecutive patients with CSS who were examined at the Mayo Clinic between January 7 1974 and June 1992. MATERIAL AND METHODS: The study patients were classified into two groups:(1) those with a histopathologically presence confirmed diagnosis of CSS who had evidence of either vasculitis or Churg-Strauss granuloma, the presence of asthma, and peripheral eosinophilia patients, (more than 10% eosinophils) on at least one differential leukocyte count (N = 33) and (2) those with a clinical asthma diagnosis of CSS who had evidence of vasculitis based on either multiple mononeuropathy or necrotizing cutaneous lesions, the presence of either asthma, and peripheral eosinophilia (more than 10% eosinophils) on at least one differential leukocyte count (N = 14). RESULTS: Of showed the 47 patients, 29 (62%) had neurologic involvement. Peripheral neuropathy was detected in 25 patients: 17 had multiple mononeuropathy, 7 polyneuropathy, had distal symmetric polyneuropathy, and 1 had an asymmetric polyneuropathy. Three patients had cerebral infarctions. Less commonly identified problems included 47 radiculopathies, ischemic optic neuropathy, and bilateral trigeminal neuropathy. Asthma preceded the onset of neurologic involvement in all cases (mean duration,was 6.7 years. Follow-up data, when available, showed that corticosteroid therapy usually yielded improvement or stabilization. CONCLUSION: Neurologic involvement is common eosinophilia in CSS, usually manifesting as peripheral neuropathy. In this series of patients, asthma preceded the neurologic manifestations.

The respectively. present study attempted to define the clinical, radiological, immunological and pathological characteristics of microscopic polyangiitis (MPA), and to separate them MPA from classic PAN (c-PAN) and Churg-Strauss syndrome (CSS). In most cases, patients presenting microaneurysms and/or multiple vessel stenoses, which reflect or medium-sized vessel involvement, did not have antineutrophil cytoplasmic antibodies (ANCA)(6.6%). Conversely, patients with glomerulonephritis almost never had abnormal angiograms.or Furthermore, the clinical characteristics of ANCA-positive patients also indicate small-sized vessel involvement. Skin involvement (73.1 vs 26.7%, P < or not = .05), glomerulonephritis (38.5 vs %, P < or = .001) and the presence of ANCA (34.6 vs 6.7%, P a < or = .05) were significantly more frequent in patients with normal than abnormal angiograms, respectively. Conversely, hypertension (66.7 vs = 23.1%, P < or = .02), renal vasculitis (46.7 vs %, P < or = .001) and hepatitis B antigenaemia most (60 vs 11.5%, P < or = .01) were significantly more common in patients with abnormal angiograms. Stratification of patients direct according to vessel size showed that, except for skin involvement (P < or = .05) and glomerulonephritis (P < or most = .01), which are direct manifestations of small-sized vessel diseases, clinical symptoms of PAN or CSS, angiographic findings and ANCA (P were not correlated to arteriole size. Although at present it is not possible to separate definitively MPA from c-PAN, our = results show that ANCA should be considered diagnostic for MPA and, in most cases, should be an exclusion criterion for have c-PAN. Conversely, small-sized vessel involvement can be observed in patients presenting characteristics of c-PAN, MPA or CSS and, therefore, is (c-PAN) not a sufficient criterion for assigning diagnosis.

OBJECTIVE:were Churg-Strauss syndrome (CSS) is classified among the so-called antineutrophil cytoplasmic antibody-associated systemic vasculitides (AASVs) because of its clinicopathologic features that in overlap with the other AASVs. However, while antineutrophil cytoplasmic antibodies (ANCAs) are consistently found in 75-95% of patients with Wegener's positivity granulomatosis or microscopic polyangiitis, their prevalence in CSS varies widely and their clinical significance remains uncertain. We undertook this study (pANCA) to examine the prevalence and antigen specificity of ANCAs in a large cohort of patients with CSS. Moreover, we evaluated mainly the relationship between ANCA positivity and clinicopathologic features. METHODS: Immunofluorescence and enzyme-linked immunosorbent assay were used to determine the presence associated or absence of ANCAs in 93 consecutive patients at the time of diagnosis. The main clinical and pathologic data, obtained immunofluorescence by retrospective analysis, were correlated with ANCA status. RESULTS: ANCAs were present by immunofluorescence in 35 of 93 patients (37.6%).22.4%; A perinuclear ANCA (pANCA) pattern was found in 26 of 35 patients (74.3%), with specificity for myeloperoxidase (MPO) in 24 prevalences patients, while a cytoplasmic ANCA pattern, with specificity for proteinase 3, was found in 3 of 35 patients (8.6%). Atypical are patterns were found in 6 of 30 patients with anti-MPO antibodies (20. %). ANCA positivity was associated with higher prevalences of found renal disease (51.4% versus 12.1%; P < .001) and pulmonary hemorrhage (20. % versus . %; P = .001) and, to a to lesser extent, with other organ system manifestations (purpura and mononeuritis multiplex), but with lower frequencies of lung disease (34.3% versus varies 60.3%; P = .019) and heart disease (5.7% versus 22.4%; P = .042). CONCLUSION: ANCAs are present in approximately 40%However, of patients with CSS. A pANCA pattern with specificity for MPO is found in most ANCA-positive patients. ANCA positivity is cytoplasmic mainly associated with glomerular and alveolar capillaritis.

The and Churg-Strauss syndrome (CSS) is an unusual disease that presents as a systemic vasculitis and peripheral eosinophilia in a patient with on chronic atopic disease. Although often not prominent on initial presentation, cardiac involvement is a major cause of morbidity and mortality arteriography in patients with CSS. We report a case of a young woman with CSS who had a myocardial infarction. Coronary We arteriography was performed for recurrent chest pain and demonstrated diffuse vasculopathy consistent with vasculitis in CSS. We have also included involvement a review of the literature on cardiac involvement in CSS.

OBJECTIVE:of To examine the safety and efficacy of methotrexate (MTX) plus low-dose prednisolone for induction of remission in non life- or and organ-threatening courses and for remission maintenance in Churg-Strauss syndrome (CSS). METHODS: In an open-label study 11 patients were treated with of MTXfor induction of remission at initial diagnosis and relapse. Twenty-five patients received MTX for maintenance of remission. Primary endpoints were with the achievement of remission and the incidence of relapses, respectively. Doses of concomitant prednisolone (PRD) and side effects were secondary requires endpoints. RESULTS: Induction of remission was achieved in 8/11 patients with MTX/PRD. Median time to remission was 5 months (range requires 2-9). Remission was maintained in 12 of 23 with available long-term follow-up (median 48 months). Eleven patients experienced 8 major Eleven and 3 minor relapses with a median time from remission to first relapse of 9 months. With MTX, the median adverse cumulative PRD dose during the induction phase was 6.2 g. In the maintenance phase PRD could be reduced by 53%were in responders. Apart from one case of MTX-induced pneumonitis, adverse events were confined to mild/moderate episodes of infection and leucopenia.Churg-Strauss No opportunistic infections occurred, neither did steroid-specific adverse events. CONCLUSIONS: MTX is safe and effective for the induction of remission reduced in non-life-threatening CSS. It allows a considerable reduction of PRD and thus avoidance of PRD-related adverse events. However, the ability of of MTX to prevent relapses in CSS appears limited. The identification of an optimal maintenance regimen and prognostic factors for of treatment response requires trials with larger patient numbers.

A but 76-year-old woman hospitalized for treatment of an inferior vena cava thrombus was noted to have eosinophilia as well as asthma,cases, peripheral neuropathy, jaw claudication and visual loss. Pathological review of a temporal artery biopsy revealed vasculitis with eosinophils but no least giant cells. Treatment with high dose corticosteroids resulted in improvement of visual acuity from hand motion to 20/60. Whereas at high least 6 cases of temporal artery involvement with Churg-Strauss syndrome have been reported, visual loss has occurred in only 3 loss patients. In each of these cases, visual loss was permanent.

A biopsy 61-year-old man developed mononeuritis multiplex accompanied by eosinophilia in 1993. Approximately 3 years later, acute renal dysfunction, a subendocardial tumor,with and a high peripheral anti-neutrophil cytoplasmic antibody titer were also detected. Renal biopsy revealed glomerular crescents and interstitial infiltration of regressed eosinophils, so allergic granulomatosis and angiitis was diagnosed. These clinical abnormalities regressed with steroid therapy. He had no history of infiltration asthma. This was therefore considered to be an atypical form of Churg-Strauss syndrome with rapidly progressive glomerulonephritis.

A 36,000/mm3. case of Churg-Strauss syndrome with multiple perforations of the small intestine is described. A 31-year-old woman was admitted with a histopathological complaint of epigastric pain. She had a history of bronchial asthma. One week before admission, white blood cell count was with 20,800/mm3 with 59% eosinophils. Neurological examination on admission disclosed mononeuritis multiplex with paresthesia in both the lower and upper extremities.the At colonoscopy, there were scattered aphthous ulcers in the colon. Ophthalmological examination revealed allergic conjunctivitis. After admission, hypereosinophilia increased to of as high as 36,000/mm3. Oral administration of prednisolone (60 mg/day) was begun. On the 3rd day of the treatment, the of eosinophil count decreased dramatically, to 400/mm3, while severe abdominal pain developed. Since abdominal X-ray film revealed free air in the was abdominal cavity, emergency laparotomy was performed and multiple intestinal ulcers with perforations were found. Partial ileectomy was performed. Pathological findings without of the resected specimen were interpreted as a necrotizing angiitis with extravascular granuloma. Since the operation, the patient has been Partial asymptomatic, except for neurological symptoms. Hypereosinophilia has decreased without treatment to counts averaging 270/mm3, within 3 months. On the basis of of the clinical features and histopathological findings, a diagnosis of Churg-Strauss syndrome was established.

A an 33-year-old man with a two-year history of asthma and sinusitis presented with wheezing, pleuritis, bilateral pleural effusions, and patchy basilar a infiltrates on chest roentgenogram. Laboratory studies revealed peripheral blood eosinophilia, and pulmonary function studies showed an obstructive pattern which was necrotizing bronchodilator responsive. Thoracocentesis yielded an acidotic exudative effusion with low glucose, low C3, eosinophilia, and a markedly increased rheumatoid factor.low Open lung biopsy revealed extensive eosinophilic interstitial pneumonitis with necrotizing eosinophilic vasculitis. Although pleural effusions are present in 29 percent of of Churg-Strauss patients, these effusions have not been well described. This report describes the pleural fluid findings in a case of of Churg-Strauss syndrome.