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lüll Rapidly fatal pulmonary fibrosis: the accelerated variant of interstitial pneumonitis Pratt DS; Schwartz MI; May JJ; Dreisin RBThorax 1979[Oct]; 34 (5): 587-93We sought to explore immunological factors in patients who died with rapidly fatal fibrosing lung diseases (Hamman-Rich syndrome). A retrospective review of cases of interstitial lung disease showed 12 recent deaths from Hamman-Rich syndrome. The mean age was 62, men outnumbering women 3 : 1. Five patients had proved collagen vascular disease (rheumatoid arthritis three, lupus two). Four others had a history of allergic disorders, syphilis, chronic eosinophilia, or hypersensitivity reactions. One patient showed disappearance of immunofluorescence as fibrosis advanced, which has not previously been reported. The study suggests a possible aetiological link between disorders of immunity and Hamman-Rich syndrome. The evidence also supports the notion that Hamman-Rich syndrome is an accelerated variant of the more indolent interstitial pneumonias.|Adult[MESH]|Aged[MESH]|Antibodies, Antinuclear/analysis[MESH]|Arthritis, Rheumatoid/complications[MESH]|Collagen Diseases/complications[MESH]|Female[MESH]|Humans[MESH]|Immune Complex Diseases/complications[MESH]|Immunoglobulins/analysis[MESH]|Lung/immunology[MESH]|Male[MESH]|Middle Aged[MESH]|Pulmonary Fibrosis/etiology/*immunology/mortality[MESH]|Rheumatoid Factor/analysis[MESH] |