Articles: pulmonary-fibrosis-etiology.
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Clin. Exp. Dermatol. · Mar 1992
Case ReportsSystemic sclerosis sine scleroderma presenting with vitiligo-like depigmentation and interstitial pulmonary fibrosis.
We report a case of systemic sclerosis sine scleroderma in which vitiligo-like depigmentation and early interstitial pulmonary fibrosis predominated. The pigmentary changes occurring in systemic sclerosis are reviewed and the distinctive features of the case discussed.
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Int. J. Radiat. Biol. · Jan 1991
Long-term changes in mouse lung following inhalation of a fibrosis-inducing dose of 239PuO2: changes in collagen synthesis and degradation rates.
Mice were exposed by nose-only inhalation to 239PuO2, which resulted in an IAD of 1110 +/- 29 Bq. At various times after exposure, rates of collagen metabolism were measured using validated in vivo methods based on the administration of radiolabelled proline, together with a large flooding dose of unlabelled proline and measurement of its incorporation into lung collagen as hydroxyproline. Dramatic increases in both synthesis and degradation rates of collagen were observed. ⋯ A similar pattern of change was observed for collagen degradation. The combination of changes in synthesis and degradation rates led to a 60% increase in lung collagen content by 300 days (control: 3.05 +/- 0.24 mg/lung, 239PuO2-exposed: 4.88 +/- 0.42 mg/lung). The data suggest that extensive remodelling of the lung connective tissue matrix occurs during development of fibrosis and that, over long periods of time, small imbalances between synthesis and degradation may result in quite large increases in protein content.
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Mayo Clinic proceedings · Jun 1990
Case ReportsPulmonary fibrosis as an unusual clinical manifestation of a pulmonary-renal vasculitis in elderly patients.
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. ⋯ 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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Ryūmachi. [Rheumatism] · Jun 1990
Case Reports[Two cases of amyopathic dermatomyositis with fatal rapidly progressive interstitial pneumonitis].
Two middle-aged women showed typical erythematous heliotrope eruption and Gottron's sign without any symptom of myositis. The patients were diagnosed as 'amyopathic dermatomyositis' because of normal serum CPK levels, normal EMG and no histological abnormality by muscle biopsy. Clinical manifestations improved by the treatment with corticosteroids. ⋯ In spite of these extensive immunosuppressive therapies, both patients died of pulmonary insufficiency a few months after admission. In the literature there has been only several cases of amyopathic dermatomyositis and only one case with fatal rapidly progressive interstitial pneumonitis. A new approach to the treatment of this disease should be made.
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In general aluminium dust induced fibrosis of the lungs occurs very rarely. Based on an own observation of a clinical case the problem of clinical and pathological diagnosis is discussed. Aluminium was detected in the lung by histological, chemical and atomic absorption spectroscopy procedures. Anamnesis, epicrisis, clinical symptomatology and the result of autopsy permit to diagnose aluminium dust induced fibrosis of the lung, taking into consideration the detection of aluminium dust by chemical and atomic absorption spectroscopy methods.