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

The clinical and immunological features of fifteen cases of cryptogenic pulmonary eosinophilia are reported. There were ten women (mean age 35.4 years) and five men (mean age 42 years). Eight gave a previous history of asthma and seven had none. Thirteen of the fifteen patients had negative skin test to common allergens. Many features of a systemic illness were present in the asthmatic and non-asthmatic groups including anaemia, weight loss, fever and a grossly raised ESR. An absolute polymorphonuclear leucocytosis was frequent as well as the obligatory increase in blood eosinophils used as one of our criteria for inclusion. Hepatomegaly (three cases), splenomegaly (four cases) and hilar node enlargement (one case) were seen in the group without asthma. Evidence of renal involvement or necrotizing vasculitis was notably absent and the response to small doses of corticosteroids was dramatic. Immunologically the striking feature was a disproportionate increase in blood eosinophils compared with only minor elevations in the total serum IgE levels. This stands in contrast to patients with bronchopulmonary aspergillosis and helminth infestation. Studies of cytophilic antibodies using histamine liberation after challenge with antibodies to immunoglobulin sub-classes in six patients showed a marked increase in IgG2 and lesser increases of IgE and IgG3. No evidence of antibodies specific to A. fumigatus was found. The amount of cytophilic antibody was also in contrast to that found in bronchopulmonary aspergillosis.
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PMID:Cryptogenic pulmonary eosinophilia. 5 41

Fourteen randomly chosen patients with "garden variety" urticaria were studied for the presence of vasculitis and immunoglobulins and complement. Results of direct immunofluorescence (DIF) of the involved skin were negative, although two patients had immunoglobulins and complement demonstrable in the cytoplasm of the epidermal cells. Results of DIF of uninvolved skin were also predominantly negative. Findings from serum samples tested by indirect immunofluorescence (IIF) were negative, except for one positive in low titer (1:10, the basement membrane zone). Serum C3 and C4 levels were normal in five patients, both levels were low in two, and the C4 level was low in one patient. No skin-reactive immunoglobulins were found in these three patients by DIF or IIF. The ESR was measured and found to be elevated in four patients. Results of immunofluorescence proved negative in these cases. Of the 12 patients studied by hematoxylineosin staining to determine histology, none exhibited vasculitis. We believe that vasculitis with antigen-antibody reactions is not the rule in "garden variety" urticaria.
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PMID:Urticaria. An immunofluorescence and histopathology study. 38 64

Seven cases with the syndrome of thalassemia minor and pauciarticular, non-erosive, seronegative arthritis are described. Other known causes of seronegative arthritis had been excluded. There was a predilection for middlesized joints such as ankles, wrists and elbows. Usually the synovitis was asymmetrical from onset, with one to eight joints affected. Finger or toe joint were usually spared. Extraarticular synovitis such as tenosynovitis or bursitis were not observed, nor were nodules, signs of vasculitis or visceral involvement. The course of this arthritis showed chronicity and mild, persistent, non-erosive synovitis without joint effusions. X-ray revealed juxtaarticular osteoporosis of the affected joints, characterized by a diminution of the number of trabeculae (hypertrophic atrophy) combined with broadening of the singular trabeculae; this picture is typical of hemoglobinopathies. - Computed tomography showed a probable slight deficit of bone mineralization. Laboratory investigations including ESR, routine immunological tests, blood chemistry, and HLA-tissue typing were all normal. - The combination of this peculiar arthropathy with thalassemia minor would appear to be worthy of note and requires a further search among the forms of arthritis of unknown origin.
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PMID:[Arthritis in thalassemia minor]. 89 52

A 47-year-old woman was admitted to our hospital because of dry cough and throat discomfort. Chest X-ray film showed reticular shadows with Kerley B line and scattered nodular shadows. Blood examination revealed normal WBC count (5100/mm3) with eosinophilia (21%), negative CRP, elevated ESR (49 mm/l hr), normal IgE level and positive antinuclear antibody with speckled pattern. Skin tests and precipitating antibodies for common allergens were negative. Results of arterial blood gas analysis and respiratory function test were almost normal. Bronchoalveolar lavage fluid yields 85.7% eosinophils, which suggested eosinophilic lung disease. To establish the diagnosis, thoracotomy was performed and lung specimens were obtained from S3a and S8a. In the area of the nodule, the alveolar spaces were filled with eosinophils and mononuclear cells, with no evidence of vasculitis, granuloma or parasites. Alveolar spaces were almost preserved in residual areas. The walls of air ways, pleura and lobular septa were heavily infiltrated with eosinophils and mononuclear cells. Thus, open lung biopsy confirmed the diagnosis of idiopathic eosinophilic pneumonia. The areas of intraalveolar filling with eosinophils and mononuclear cells were found to correspond to the nodular shadows on chest X-ray film. The relationship between the findings of chest X-ray films and lung histology are discussed.
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PMID:[A case of eosinophilic pneumonia with diffuse reticular shadows and scattered nodular shadows on chest X-ray film--comparison of findings of chest X-ray and lung histology]. 128 40

The prevalence of various forms of peripheral neuropathy has not been previously assessed in large series of patients with essential mixed cryoglobulinaemia (EMC). Clinical and electrophysiological signs of peripheral neuropathy were observed in 21 of 37 EMC patients, consisting of polyneuropathy in 19, mononeuropathy or multiple mononeuropathy in eight, and both in six. The various forms of peripheral neuropathy occurred differently in the subgroups of EMC. Isolated polyneuropathy was more common with type II (eight of 10) than type III EMC (two of eight). Multifocal neuropathy, in association with polyneuropathy, was the most common form in type III EMC (five of eight). Patients with peripheral neuropathy and type II EMC were significantly older than type II EMC patients without neuropathy, regarding present age and age of onset of EMC. Patients with peripheral neuropathy and type III EMC tended to have higher values of ESR and IgM than type III EMC patients without neuropathy. Electrophysiological findings and sural nerve biopsy specimens (nine cases) showed prominent axonal changes. Vascular changes included vasculitis and alterations of the endoneurial microvessels in type II and type III EMC. Our findings suggest that distinct pathogenic factors are implicated in the subgroups of cryoglobulinaemic neuropathy, possibly inducing different types of vascular changes underlying polyneuropathy or, respectively, mononeuropathy and multiple mononeuropathy.
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PMID:Peripheral neuropathy in essential mixed cryoglobulinaemia. 131 72

To determine the efficacy and safety of intermittent intravenous pulse cyclophosphamide in patients of severe systemic lupus erythematosus (SLE), 50 patients having severe/refractory lupus nephritis, vasculitis or neuropsychiatric manifestations were treated with 3 weekly pulses of cyclophosphamide for 6 such pulses. This treatment was found to be associated with significant and sustained improvement during a 2 yr follow up with respect to the mean renal activity score, individual renal parameters (proteinuria, erythrocyturia, and serum creatinine levels), focal neurological manifestations, vasculitic lesions, antinuclear antibody titers, complement component C3, anti-dsDNA antibodies levels and ESR. There was a sustained decrease in the overall mean disease activity score, and the mean daily dose of prednisolone (pretreatment 32.62 mg daily to 3.75 mg daily after 24 months). There was a significant decline in the percentage and absolute B cell count after 7, 14 and 21 days of this treatment. Effect on other lymphocyte subsets (CD3+, CD4+ and CD8+) was not marked. Pulse cyclophosphamide could therefore be an effective and less toxic form of treatment in patients with SLE having severe lupus nephritis, focal neurological lesions or vasculitis.
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PMID:Intermittent intravenous pulse cyclophosphamide treatment in systemic lupus erythematosus. 142 48

A 40-yr-old Caucasian woman who had been suffering from systemic lupus erythematosus (SLE) since five years developed vague abdominal complaints whilst under treatment with a low dose of steroids. She had been admitted because of vomiting and abdominal tenderness. The ESR and CRP levels were rising and the C4 level had been persistently low in the preceding months. Normal non-invasive procedures did not allow a diagnosis to be made. Therefore exploratory laparotomy was performed and revealed a non-bacterial peritonitis and an oedematous jejunum. She responded well to a high dose of prednisone. Serositis of the peritoneum as well as bowel vasculitis may be a rare manifestation of SLE despite apparent control of other lupus manifestations. In this patient serositis flares were associated with a rise in CRP level.
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PMID:Lupus peritonitis presented as vague abdominal complaints in a SLE patient. 143 59

In order to describe the British experience of Wegener's granulomatosis Hospital Activity Analysis was used to collect cases diagnosed in England, Wales and Scotland between 1975 and 1985. Where possible clinical details, histological material and chest radiographs were obtained. Two hundred and sixty five patients were considered to have Wegener's granulomatosis. In 109 a single pathologist confirmed the diagnosis by finding both granulomas and vasculitis in biopsy material. The diagnosis was made on clinical grounds or clinical grounds together with histological diagnosis in the local hospital in 156 patients. Wegener's granulomatosis was confined to the lung or upper respiratory tract in 22 per cent of patients and renal disease occurred in 58 per cent. Laboratory tests showed a pattern of mild anaemia, polymorph leucocytosis, eosinophilia and an elevated ESR and hypergammaglobulinaemia, with no specific pattern of changes. Histological confirmation was most frequently obtained by examination of nasal biopsy specimens, but multiple biopsies were often required. Renal biopsies showed focal proliferative glomerulonephritis but granulomatous glomerulonephritis was uncommon. Of available chest radiographs 61 per cent were abnormal, large opacities being most common. Small irregular opacities were found less often and other abnormalities were uncommon. Treatment varied widely and 10 per cent of patients received no drug therapy. This large series illustrates that even without specific treatment, patients with Wegener's granulomatosis can survive for several years and with modern treatment survival for more than a decade is possible. Conclusions about the effectiveness of the various therapies cannot be drawn from this retrospective study. Renal failure and disseminated vasculitis were the commonest causes of death; death was considered to result from complications of treatment with cytotoxic drugs or prednisolone in 6 per cent of patients.
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PMID:Wegener's granuloma. A series of 265 British cases seen between 1975 and 1985. A report by a sub-committee of the British Thoracic Society Research Committee. 144 44

Aortic valve lesions in progressive systemic sclerosis (PSS) are very uncommon. To our knowledge, aortic regurgitation (AR) associated with PSS has not been reported previously. We would like to report the case of a 58-year-old woman who had PSS with AR due to Raynaud's symptom, fever, positive ANA, accelerated ESR, and diastolic blowing murmur along the left sternal border. After treatment with adreno-cortico steroid and an immunosuppressive agent, the patient improved serologically and symptomatically. However, she was later admitted to our hospital again due to heart failure with progressive AR. She died of refractory heart failure with severe AR and tricuspid regurgitation (TR). The former was caused by aortic cusp lesions and the latter by pulmonary hypertension. An autopsy confirmed the diagnosis of PSS, which was found to have involved the heart, lungs and pancreas. Vasculitis with infiltration and fibrotic changes were noted in these organs. Moreover, there were fibrotic thickenings and shortenings in the aortic cusps with cell infiltration. There were no indications of rheumatic disease. These results suggest that the cause of our patient's aortic valve disease may have been PSS vasculitis.
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PMID:[Report of a case with aortic regurgitation in progressive systemic sclerosis]. 151 78

Kawasaki syndrome was first described in 1967 in Japan. It is due to a generalized vasculitis of probably infectious etiology. Diagnosis is based exclusively on clinical criteria. Both morbidity and mortality are mainly determined by coronary artery involvement. The principal purpose of the present analysis of the 32 patients treated for Kawasaki syndrome at the University Children's Hospital in Berne (22 males = 69%, 11 females = 31%; mean age +/- SD = 2.7 +/- 2 years) was to describe the clinical and laboratory presentation and to evaluate high-risk factors and therapeutic efficacy with regard to course of the disease and cardiac involvement. Treatment has included aspirin and, since 1986, intravenous immunoglobulins (IVIG) as well. Routine electrocardiographic and echocardiographic checks detected 10 patients (31%) with cardiovascular complications, all of whom remained clinically asymptomatic: 9 with carditis (28%) and 3 with coronary artery aneurysms (CAA) (9%). The 3 patients with CAA were boys aged 18-25 months who presented an extremely long duration of fever (12-30 days) and markedly elevated ESR (100-128 mm/h). Since the therapeutic use of high-dose IVIG (9 patients), no CAA has been observed, and carditis in only 2 patients (22%). All patients are clinically cured. The carditis findings and 2 of the 3 CAA completely resolved; one patient has persistent CAA and myopathic cardiomegaly after more than 3 1/2 years. This study, together with the review of the literature, should enable the physician to diagnose and correctly manage the childhood disease Kawasaki syndrome.
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PMID:[Kawasaki syndrome]. 169 23


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