Lung Involvements in Rheumatic Diseases: Update on the Epidemiology, Pathogenesis, Clinical Features, and Treatment

Abstract

Lung illness encountered in patients with rheumatic diseases bears clinical significance in terms of increased morbidity and mortality as well as potential challenges placed on patient care. Although our understanding of natural history of this important illness is still limited, epidemiologic knowledge has been accumulated during the past decade to provide useful information on the risk factors and prognosis of lung involvements in rheumatic diseases. Moreover, the pathogenesis particularly in the context of genetics has been greatly updated for both the underlying rheumatic disease and associated lung involvement. This review will focus on the current update on the epidemiologic and genetics features and treatment options of the lung involvements associated with four major rheumatic diseases (rheumatoid arthritis, systemic sclerosis, myositis, and systemic lupus erythematosus), with more attention to a specific form of involvement or interstitial lung disease.

Figure 1

Schematic presentation of shared pathogenesis of RA and RA-ILD.

Figure 2

DETECT algorithm. ACA: anti-centromere antibody; DLCO: diffusing capacity of carbon monoxide; FVC: forced vital capacity; NT-proBNP: N-terminal probrain natriuretic peptide; TR: tricuspid regurgitation (cited and modified from “Evidence-Based Detection of Pulmonary Arterial Hypertension in Systemic Sclerosis: The DETECT Study” by Coghlan JG, et al. Ann Rheum Dis 2014; 73: 1340-9).

Figure 3

A schematic picture on screening and monitoring rheumatic disease associated lung involvements. DLCO: diffusing capacity of carbon monoxide; FVC: forced vital capacity; HRTC: high resolution computed tomography; ILD: interstitial lung disease; NT-proBNP: N-terminal probrain natriuretic peptide; PAH: pulmonary arterial hypertension; TR: tricuspid regurgitation.