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Query: UMLS:C0010200 (cough)
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Between 1978 and 1988, three patients at our institution had an initial diagnosis of idiopathic pulmonary fibrosis but later were correctly diagnosed as having pulmonary-renal syndrome and microscopic polyarteritis. These cases involved elderly patients with progressive dyspnea and nonproductive cough, bilateral dry crackling rales, bilateral interstitial infiltrates evident on a chest roentgenogram, and restrictive findings on pulmonary function testing. In two patients, lung biopsy specimens were obtained, and an initial diagnosis of nonspecific pulmonary fibrosis was made. All three patients eventually had microhematuria and renal insufficiency. A revised diagnosis of small-vessel pulmonary-renal vasculitis was based on the demonstration of segmental necrotizing glomerulonephritis in renal biopsy specimens in two patients, thrombotic microangiopathy consistent with healed vasculitis on postmortem examination of the kidney in one patient, and subsequent detection of small-vessel vasculitis on review of the two lung biopsy specimens. Anti-neutrophil cytoplasmic antibodies with perinuclear staining on indirect immunofluorescence microscopy were positive in the two patients in whom determinations were performed. The clinical manifestations of vasculitis were notably scarce--no involvement of the skin, nervous system, or gastrointestinal tract; no episodes of fever; and minimal or absent musculoskeletal symptoms. These cases illustrate the importance of a high index of suspicion for the diagnosis of systemic vasculitis in elderly patients and the need to consider a vasculitis in the differential diagnosis of idiopathic pulmonary fibrosis, especially if an active urinary sediment is present.
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PMID:Pulmonary fibrosis as an unusual clinical manifestation of a pulmonary-renal vasculitis in elderly patients. 219 45

Sixteen of 32 Friesian calves, 8 to 10 weeks old, died over 4 weeks. The calves were housed in pens previously used by dogs. Clinical signs included anorexia, pale mucous membranes, rapid weight loss, coughing and palpably enlarged superficial lymph nodes. At necropsy, calves were emaciated and had generalised enlargement of lymph nodes, pale mottling of skeletal muscles, excess peritoneal, thoracic and pericardial fluid and subpleural and subepicardial haemorrhages. Histologically there was a lymphadenitis, myositis, myocarditis, glomerulonephritis, interstitial pneumonitis and encephalitis. Schizonts of a sporozoan parasite, presumably Sarcocystis cruzi were found in the endothelial cells of blood vessels in many organs.
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PMID:An outbreak of sarcocystosis in dairy cattle. 308 19

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

The clinical findings during a major epidemic of Q-fever which affected 415 people in the Val de Bagnes (Valais, Switzerland) in the autumn of 1983 are reported. Q-fever symptoms were evident in 191 cases but inconspicuous or absent in 224 cases. The symptoms most frequently reported were prolonged high fever, headaches, severe exhaustion, loss of appetite, cough and myalgia. Amongst disorders which accompany acute Q-fever, pneumonia and granulomatous hepatitis are very frequent, while myopericarditis and glomerulonephritis are less frequently observed. Endocarditis, a later complication of Q-fever, is a severe illness which more frequently affects patients with underlying valvular lesions. New serological techniques now permit more rapid and more accurate diagnosis of both acute and chronic Q-fever.
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PMID:[Clinical aspects observed during an epidemic of 415 cases of Q fever]. 389 64

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

A 53 year old man first presented with a mild anti-neutrophil cytoplasmic antibody (ANCA), type (MPO-ANCA) associated microscopic polyangiitis. Approximately one year later, he presented with fatigue, fever, cough, sore muscles and erythrocyturia. At admission he was uraemic. Kidney biopsy showed in LM extracapillary glomerulonephritis and in immunofluorescence microscopy linear deposition of IgG along the glomerular basement membrane (GBM). MPO-ANCA was still positive, but antibodies against GBM were now also present. Retrospective analysis revealed the presence of anti-GBM antibodies nine months before clinical illness. Pr3-ANCA (C-ANCA) was negative during the whole course. In spite of decreasing anti-GBM antibody levels during treatment, his condition aggravated, and he died from complications. Thus, coexisting anti-GBM antibodies may explain suddenly deteriorating renal function in patients with ANCA associated vasculitis.
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PMID:[Rapidly progressing glomerulonephritis. Microangiitis associated with antibodies against the glomerula membrane]. 757 Nov 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

A 49-year-old man was admitted because of general fatigue, cough and hematuria. During the hospital course, acute renal failure, hemoptysis and dyspnea developed. A percutaneous renal biopsy revealed a diffuse crescentic glomerulonephritis, and direct immunofluorescence showed a linear pattern of IgG along the glomerular basement membrane. Although serum anti-glomerular basement membrane (anti-GBM) antibody was not detected. Goodpasture's-like syndrome was suspected, and methylprednisolone pulse therapy and plasmapheresis were administered. Concomitantly, extracorporeal membrane oxygenation (ECMO) was instituted because of deterioration in respiratory status due to a severe pulmonary hemorrhage despite maximal ventilatory support. Temporarily, the patient improved and ECMO was discontinued. ECMO may be a useful therapeutic support for hypoxia resulting from pulmonary hemorrhage in Goodpasture's syndrome (GPS) and Goodpasture's-like syndrome.
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PMID:Goodpasture's-like syndrome and effect of extracorporeal membrane oxygenator support. 800 Jan 12

Here we report an autopsy case with anti-neutrophil antibodies (ANCA) associated vasculitis accompanied by autoimmune hepatitis and hepatocellular carcinoma. A 69-year-old woman was admitted to Tokyo Metropolitan Ohtsuka Hospital in October 1995 because of leg edema. She had presented cough in 1990 and diagnosed as interstitial pneumonia, esophageal varices and liver chirosis. On admission, laboratory data showed mild anemia, hypoproteinemia, and marked gammagloblinemia. IgM-HA antibody, HBs antigen, HBs antibody, HCV antibody and HDV antibody were negative. Anti-nuclear antibody, anticentromere antibody, anti-neutrophil cytoplasmic antibody against myeloperoxidase and cathepsin G (MPO-ANCA and cathepsin G), rheumatoid factor and direct coombs test were positive. Serum level of AFP and CEA were elevated. Ultrasonography and computed tomography of abdomen scowed liver chirosis and tumor in left lobe of liver. The diagnosis of liver chirosis based on autoimmune hepatitis and Interstitial pneumonia was made with clinical course, laboratory findings and radiographic findings although liver biopsy was not performed. She complained of bloody stool due to ulcer of the large intestine, and died of liver failure which progressed rapidly. The autopsy findings detected that pulmonary fibrosis, liver fibrosis with multiple hepatocellular carcinoma, necrotizing crescentic glomerulonephritis, and vasculitis of small artery inn colon. This was the first report of MPO-ANCA associated vasuculitis complicated with autoimmune hepatitis and hepatocellular carcinoma. Clinical significance of ANCA and immunogenetic background of these diseases were discussed.
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PMID:[An autopsy case of anti-neutrophil cytoplasmic antibodies associated vasculitis accompanied by autoimmune hepatitis and hepatocellular carcinoma]. 917 69

A 69-year-old woman presented with persistent dyspnea and continuous coughing and mediastinal mass. The mass was found to be a malignant thymoma and was resected incompletely. A full-blown nephrotic syndrome appeared 1 year after removal of the thymoma. Renal biopsy revealed minimal-change glomerulonephritis. There was no evidence of other autoimmune diseases or causes of the minimal-change glomerulonephritis.
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PMID:Minimal-change nephropathy and malignant thymoma. 948 41


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