Rapidly fatal pulmonary fibrosis: the accelerated variant of interstitial pneumonitis.

Abstract

We 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.

A monoclonal antibody to the carboxyterminal domain of procollagen type I visualizes collagen-synthesizing fibroblasts. Detection of an altered fibroblast phenotype in lungs of patients with pulmonary fibrosis.

“…Moreover, in active pulmonary fibrosis, the number of fibroblasts staining for procollagen and their average intracellular collagen content (and almost certainly their synthetic activity) is increased. Thus, it appears clear from this and related studies that unknown factors in pulmonary fibrosis stimulate both increased numbers offibroblasts and a more active fibroblast synthetic phenotype (1,3,21,22). Lung fibroblasts staining with Anti-pC could contain more procollagen than fibroblasts from quiescent lung simply because procollagen secretion is somehow impeded, as in the case of ascorbate deprivation (23).…”

“…6, A and B is a biopsy obtained from patient C.S. who had rheumatoid disease and accelerated fibrosis with a fatal outcome (21). Anti-pC stained myriad fibroblastic cells in the greatly thickened interstitium.…”

Acute interstitial pneumonia: report of a series

“…However, cases of rapidly progressive idiopathic organising pneumonia [19] can show overlapping clinical, radiological and histological findings with those occurring in AIP patients. The boundaries between these two entities are, in a few cases, arbitrary and the absence of an evident lymphocytosis in BAL fluid might be the more important element to distinguish AIP with a prominent intra-alveolar organisation and cryptogenic organising pneumonia [2].…”

Rheumatoid Arthritis-Associated Interstitial Lung Disease

“…Acute worsening of disease has been reported in RA-ILD patients, 41 and appears to occur primarily in those patients with UIP pattern. 29,39 In a study of a large cohort of 93 subjects with CTD-ILD, Park et al 42 reported a 1-year incidence of acute worsening of 3.3% (four subjects).…”