Journal
CURRENT RHEUMATOLOGY REVIEWS
Volume 15, Issue 4, Pages 277-289Publisher
BENTHAM SCIENCE PUBL LTD
DOI: 10.2174/1573397115666190116102451
Keywords
Rheumatoid Arthritis-Associated Interstitial Lung Disease (RA-ILD); Nonspecific Interstitial Pneumonia (NSIP); Usual Interstitial Pneumonia (UIP); Computed Tomography (CT); Histology; inflammation; fibrosis; animal models
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Funding
- Parker B. Francis Fellowship Award
- Univ of Rochester DOM Faculty Pilot Project Award
- NIH [K08 AR067885, R01 AR056702, R01 AR069000, P30 AR069655, MSTP T32 GM007356, T32 AR053459]
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Interstitial Lung Disease (ILD) is a well-known complication of rheumatoid arthritis (RA) which often results in significant morbidity and mortality. It is often diagnosed late in the disease process via descriptive criteria. Multiple subtypes of RA-ILD exist as defined by chest CT and histopathology. In the absence of formal natural history studies and definitive diagnostics, a conventional dogma has emerged that there are two major subtypes of RA-ILD (nonspecific interstitial pneumonia (NSIP) and Usual Interstitial Pneumonia (UIP)). These subtypes arc based on clinical experience and correlation studies. However, recent animal model data are incongruous with established paradigms of RA-ILD and beg reassessment of the clinical evidence in order to better understand etiology, pathogenesis, prognosis, and response to therapy. To this end, here we: 1) review the literature on epidemiology, radiology, histopathology and clinical outcomes of the various RA-ILD subtypes, existing animal models, and current theories on RA-ILD pathogenesis; 2) highlight the major gaps in our knowledge; and 3) propose future research to test an emerging theory of RA-ILD that posits initial rheumatic lung inflammation in the form of NSIP-like pathology transforms mesenchymal cells to derive chimeric disease, and subsequently develops into frank UIP-like fibrosis in some RA patients. Elucidation of the pathogenesis of RA-ILD is critical for the development of effective interventions for RA-ILD.
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