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Wegener's granulomatosis (WG) is an uncommon disease of unknown aetiology which is characterised histologically by a necrotising granulomatous angiitis. The airway, lungs and the kidneys are predominantly involved, but the disease has been documented to affect virtually every organ system. The clinical course is variable and ranges from a short, rapidly fatal illness at one end of the spectrum to indolent involvement compatible with several years of survival at the other. A majority of patients have pulmonary disease evidenced clinically by cough, sputum production and haemoptysis and radiologically by infiltrates, nodules and cavitation. Pleural effusions, however, are rare. No detailed information regarding the nature and clinical behaviour of these effusions is available and only a recent French study has listed the nature of the fluid in passing. We have observed pleural effusions in five patients with WG who are the basis of this report.
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PMID:Pleural effusions in Wegener's granulomatosis: report of five patients and a brief review of the literature. 177 3

We treated two children with scleritis (one unilateral, one bilateral), in whom Wegener's granulomatosis was diagnosed on the basis of pathologic changes in respiratory tract mucosa. Both patients were girls, 13 and 14 years of age, respectively. One patient had otitis media and a nodular scleritis. Laboratory test results demonstrated an increased erythrocyte sedimentation rate and microscopic hematuria. A biopsy of the sinus confirmed the diagnosis of Wegener's granulomatosis. The second patient had fever, arthralgias, a nonproductive cough, and bilateral scleritis. Laboratory test results demonstrated an increased erythrocyte sedimentation rate, positive test results for rheumatoid factor, and bilateral pulmonary nodules on chest x-ray. Open-lung biopsy confirmed the diagnosis of Wegener's granulomatosis. Both patients responded well to treatment with a combination of prednisone and cyclophosphamide.
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PMID:Scleritis and Wegener's granulomatosis in children. 188 59

Although fiberoptic bronchoscopy (FOB) has been traditionally used to evaluate nonresolving pneumonia, its efficacy is unknown. We, therefore, reviewed FOB in 35 consecutive patients who had (1) a roentgenographic infiltrate, (2) cough, (3) either temperature greater than 38.1 degrees C, leukocytosis, sputum production, (4) symptoms present for at least ten days, and antibiotic therapy for at least one week. Known lung cancer and AIDS were excluded. Fiberoptic bronchoscopy was diagnostic in 86 percent (12/14) in whom a specific cause was found. No patient had endobronchial cancer. Two patients with nondiagnostic FOB and persistent systemic symptoms had open lung biopsy specimens showing Wegener's granulomatosis and bronchiolitis obliterans with organizing pneumonia (BOOP). Twenty-one patients with nondiagnostic FOB had no final diagnoses other than community-acquired pneumonia. We conclude that FOB is extremely useful in finding a specific diagnosis for a nonresolving pneumonia when a specific diagnosis can be made. Fiberoptic bronchoscopy was most likely to yield a specific diagnosis in nonsmoking patients with multilobar infiltrates of long duration and could have been avoided in older, smoking, or otherwise compromised patients with lobar or segmental infiltrates with no decrease in diagnostic yield in our series.
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PMID:Utility of fiberoptic bronchoscopy in nonresolving pneumonia. 224 65

Most patients with Wegener's granulomatosis initially present with upper airway symptoms, persistent rhinosinusitis or otitis media. We report the case of a young female who presented with progressive dyspnea, inspiratory stridor and barking cough due to a subglottic stenosis. We would like to stress this often unrecognized type of presentation. The differential diagnosis, the limitations of histopathological examinations, and the interest of looking for anticytoplasmic antibodies in the diagnosis of Wegener's granulomatosis, are briefly discussed.
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PMID:[Subglottic stenosis: unrecognized type of presentation of Wegener's granulomatosis]. 234 57

A 60-year-old woman was admitted to our hospital in June 1985, complaining of fever, cough and right lower chest pain, with a five-year history of asymptomatic primary biliary cirrhosis. Chest X-ray on admission showed an infiltrative shadow in the right lower lung field. She was first treated with various antibiotics unsuccessfully. Hemoptysis continued. Dyspnea and anemia appeared. Chest X-ray 17 days after admission showed multiple infiltrative shadows in the both lung fields. She was treated with steroid pulse therapy successfully. During prednisolone treatment decreasing nodular shadows with cavities appeared on chest X-ray. An open lung biopsy was performed in March 1986. The histologic findings showed a necrotizing vasculitis with granuloma and perivascular fibrosis. She was treated with prednisolone and prednisolone-azathioprine therapy unsuccessfully, but successfully with prednisolone-cyclophosphamide therapy. This case was a rare case of Wegener's granulomatosis with transition from fulminant type to granulomatous type. No similar case of Wegener's granulomatosis with asymptomatic primary biliary cirrhosis has been reported in the literature.
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PMID:[Wegener's granulomatosis in a woman with asymptomatic primary biliary cirrhosis]. 263 Jul 76

Eleven patients with Wegener's granulomatosis were seen at this Institute over a period of 20 years. There were six men and five women. The average age of presentation was 38.3 years, and the mean duration of symptoms was 10.5 months. Constitutional symptoms (82%), cough (82%), ocular symptoms (64%), arthralgias (55%), rhinorrhoea (55%), haemoptysis (45%), nasal granuloma (45%), otorrhoea (36%), sinusitis (36%), skin lesions (27%), and renal failure (27%) were the clinical manifestations encountered. All patients had an elevated ESR, and 55% had leucocytosis. Proteinuria and haematuria were observed in 64% and 55% respectively. Chest radiographs were abnormal in 82%. In four patients the disease had a fulminant course and the patients died before adequate treatment was given. Two patients received corticosteroids alone and have since been lost to follow up. Five (45%) received adequate cytotoxic therapy and have done well for 8-46 months (mean, 24.8 months) after diagnosis. Wegener's granulomatosis in India is apparently similar to that seen elsewhere, but the high incidence of tuberculosis interferes with early diagnosis and treatment.
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PMID:Wegener's granulomatosis in north India. An analysis of eleven patients. 343 65

We have analyzed an unusual group of 19 patients (15 previously reported) with Wegener's granulomatosis, who presented with severe glomerulonephritis and developed diagnostic respiratory lesions only after 4 to 78 months. Necrotizing glomerulonephritis, often with crescents, and rarely with vasculitis, was the predominant renal lesion. Wegener's granulomatosis was unsuspected initially, since systemic manifestations, such as fever, arthralgias, malaise, and even pulmonary hemorrhage, were nonspecific or transient, and because renal biopsy findings resembled those seen in microscopic polyarteritis or idiopathic crescentic nephritis. Despite therapy, usually with corticosteroids, only 4 patients maintained adequate renal function. Most patients were receiving chronic dialysis when respiratory involvement developed. Cavitary nodular pulmonary infiltrates were seen in 12 of the 17 patients with lung involvement, and otorhinological disease occurred in 10 patients. Arthralgias, fever, and cough, with or without hemoptysis, were common. Wegener's granulomatosis was diagnosed by lung biopsy in 15 cases and by nasal biopsy in 4. Specific treatment was required for the respiratory disease and was delayed in many patients, because of lack of awareness that Wegener's granulomatosis may present with primary glomerulonephritis and become active during chronic renal failure or dialysis. Nevertheless, all but 1 patient eventually responded to treatment, although 3 additional patients died of late complications.
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PMID:Severe glomerulonephritis with late emergence of classic Wegener's granulomatosis. Report of 4 cases and review of the literature. 357 16

A 26 year-old woman first complained at a sparse sputum stained with blood. 18 days later haemorrhagic petechias on the lower limbs skin appeared and rapidly disappeared. The gradually enhancing cough was followed by haemoptysis and bleeding. The death occurred at the 41st day of the disease from persisting lung bleeding. The cause of lung bleeding was Wegener's granulomatosis which was characterized by destructive angiitis, haemic and tissue eosinophilia, lymph node granulomatosis and diffuse glomerulonephritis.
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PMID:[Case of Wegener's granulomatosis]. 672 7

The major manifestations of giant cell arteritis have been well described. Pulmonary manifestations, however, are rare. We report the case of a 75 year old woman with temporal arteritis, presenting with atypical manifestations, i.e. nodular pulmonary lesions, dry cough, rhinitis, conjunctivitis, and otitis with hearing loss. We conclude that overlapping features of giant cell arteritis and Wegener's granulomatosis occur in some patients.
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PMID:Unusual manifestations of giant cell arteritis: pulmonary nodules, cough, conjunctivitis and otitis with deafness. 771 13

A 36-year-old man with hemophilia A was admitted to hospital because of otalgia, hearing loss, nasal obstruction, nonproductive cough, and high fever. His laboratory data showed high-grade acute inflammatory reactions. His chest X-ray and CT films showed multiple cavitary masses in the right lower lung field. Bronchoscopy performed at our institution revealed bronchial nodules in the intermediate truncus, and BAL revealed increases in the neutrophils and an IgG index (BAL IgG/albumin divided by serum IgG/albumin). Biopsy specimens obtained from nasal mucosa showed epithelioid granulomas with Langerhans' giant cells and necrotizing vasculitis. Antineutrophil cytoplasmic antibodies were also positive, but no evidence of glomerulonephritis was observed. The diagnosis of limited Wegener's granulomatosis was thus made. He was treated with standard therapy (daily cyclophosphamide and glucocorticoids), but within 1 month he had complications of empyema with herpes zoster, and bronchopleural fistula. The complications resolved with appropriate treatment.
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PMID:[A case of limited Wegener's granulomatosis with hemophilia A, complicated by empyema, bronchopleural fistula and herpes zoster during therapy]. 781 60


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