Clinical Course of Interstitial Lung Disease in Patients With Rheumatoid Arthritis

Abstract

Interstitial lung disease (ILD) is a frequent manifestation of rheumatoid arthritis (RA) that is associated with high mortality. RA-ILD may initially be asymptomatic, and lung function may be markedly impaired by the time it is diagnosed. The course of RA-ILD is highly variable, with some patients experiencing no discernable progression or a slow decline, whereas others experience more rapid deterioration. Some patients develop progressive pulmonary fibrosis, which is associated with high mortality. Although risk factors for the progression of RA-ILD have been identified, including older age, worse lung function, and a usual interstitial pneumonia pattern on high-resolution computed tomography, it is not possible to predict the course of RA-ILD in an individual patient. The association between RA disease activity and progression of RA-ILD remains unclear. Regular monitoring is important to enable the prompt identification of progression and early intervention to preserve lung function. The management of RA-ILD requires a multidisciplinary and individualized approach, taking account of the severity and progression of articular and lung disease, risk factors for the progression of RA-ILD, and the patient's preferences, and may include immunosuppression, antifibrotic therapy, and supportive care.

Figure 1

HRCT with axial images showing UIP‐type fibrosis with reticulation, traction bronchiectasis, and honeycomb fibrosis (top row) and progression on scans taken 16 months later (bottom row). HRCT, high‐resolution computed tomography; UIP, usual interstitial pneumonia. Source: Reproduced with permission from Mary E. Strek.

Figure 2

Course of RA‐ILD based on lung function, HRCT, and death. HRCT, high‐resolution computed tomography; RA‐ILD, rheumatoid arthritis–interstitial lung disease.

Figure 3

Survival in patients with RA‐ILD and in a matched cohort of patients with RA without ILD. RA, rheumatoid arthritis; ILD, interstitial lung disease. Source: Adapted with permission from BMJ Publishing Group Limited. Ann Rheum Dis, Hyldgaard C, et al, Vol. 76, Pages No. 1700–1706, Copyright 2017.

Figure 4

Survival in patients with RA‐ILD (n = 227) in subgroups by (A) disease activity (A), functional status (B), predicted FVC (C), and predicted DLCO at baseline (D). DLco, diffusing capacity for carbon monoxide; FVC, forced vital capacity; RA‐ILD, rheumatoid arthritis–interstitial lung disease. Source: Published by Oxford University Press on behalf of the British Society for Rheumatology 2022.

Figure 5

Survival in patients with newly diagnosed RA‐ILD with a definite or possible UIP pattern or a NSIP pattern on HRCT. HRCT, high‐resolution computed tomography; NSIP, nonspecific interstitial pneumonia; RA‐ILD, rheumatoid arthritis–interstitial lung disease; UIP, usual interstitial pneumonia. Source: Adapted from Respir Med, Vol 126, Yunt ZX et al, High resolution computed tomography pattern of usual interstitial pneumonia in rheumatoid arthritis‐associated ILD: relationship to survival, Pages No. 100–104, Copyright 2017, with permission from Elsevier.