Articles: pulmonary-fibrosis-etiology.
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Acute respiratory distress syndrome is a severe disease, the treatment and pathophysiology of which are not completely established. The pathology of acute respiratory distress syndrome involves diffuse alveolar damage, which comprises severe alveolar epithelial cell damage, hyaline membrane formation, and festinate myofibroblast proliferation and fibrosis in the intra-alveolar spaces. We performed a clinicopathologic investigation of 26 autopsy cases of diffuse alveolar damage. ⋯ When diffuse alveolar damage was diagnosed pathologically as being due to severe infection, all 7 patients showed multiple organ dysfunction syndrome, whereas only 2 of 7 patients showed interstitial myofibroblast proliferation. When diffuse alveolar damage was attributed to tumor treatment with chemotherapy or to drug toxicity, 3 of 16 patients showed multiple organ dysfunction syndrome; 15 of 16 showed interstitial myofibroblast proliferation, 3 of 3 acute interstitial pneumonia patients did not show multiple organ dysfunction syndrome; and 3 of 3 acute interstitial pneumonia showed marked interstitial myofibroblast proliferation. These results suggest that the pathophysiologic mechanism of diffuse alveolar damage caused by severe infection is one of systemic circulation disturbance, although the mechanism underlying diffuse alveolar damage due to tumor with chemotherapy or drug toxicity appears to involve interstitial pneumonia-like lesions that are similar to acute interstitial pneumonia.
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Arch. Bronconeumol. · Aug 2009
Case Reports[Familial pulmonary fibrosis in 2 Mexican sisters with Hermansky-Pudlak syndrome].
Hermansky-Pudlak syndrome is an autosomal recessive disorder commonly found in individuals of Puerto Rican ancestry. We present 2 cases of familial pulmonary fibrosis in 2 Mexican sisters with Hermansky-Pudlak syndrome. Pulmonary fibrosis was biopsy-proven in 1 of the patients. This report shows that Hermansky-Pudlak syndrome may occur in individuals of Mexican ancestry.
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To describe the clinical presentation of the association between pulmonary fibrosis (PF) and systemic vasculitis related to antineutrophil cytoplasmic antibodies (ANCA-V). ⋯ The association of PF and ANCA-V does not seem to be fortuitous, even though their clinical evolutions are clearly not related. PF was the major cause of death.
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Pneumonol Alergol Pol · Jan 2009
Review[Combined pulmonary fibrosis and emphysema - case report and literature review].
We describe the case of a 61-year-old male patient, in which the search for the cause of chronic respiratory failure, severe pulmonary hypertension and secondary erythrocytosis resulted in a diagnosis of combined pulmonary fibrosis and emphysema (CPFE). This is a unique, recently characterised syndrome with upper-lobe emphysema and pulmonary fibrosis of the lower lungs. The cause is unknown, but one of the main risk factor remains smoking. ⋯ Diagnosis was based on chest computer tomography, which revealed upper lobe emphysema and lower lobe ground glass changes and honeycombing. Severe pulmonary hypertension (SPAP 80 mm Hg) was confirmed by echocardiography and right cardiac catherisation. The patient received long-term oxygen therapy, inhaled corticosteroid and Ca-blocker.
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Of the idiopathic lung diseases, idiopathic pulmonary fibrosis (IPF) and sarcoidosis have been the focus of a growing number of epidemiological investigations on the risk of environmental and occupational exposures. To date, the consistency of epidemiological evidence is suggestive of a causal relationship between several environmental exposures and IPF, with the strongest evidence for cigarette smoking and metal dust. Current knowledge about pathogenesis provides further support for a causal link. ⋯ Two studies provide consistent evidence for exposures to agricultural dust and musty odor/mold/mildew, and studies among military personnel and firefighters suggest mixed dust and fume exposures as risk factors for sarcoidosis. Although studies of the pathogenesis of sarcoidosis also provide evidence supporting environmental causation, more epidemiological studies are needed to establish consistency of associations, dose-response, and temporality. Future investigations, of gene-environment interaction offer the potential for strengthening the evidence of causation between several environmental and occupational exposures and idiopathic lung diseases.