Rheumatoid arthritis (RA) is considered a systemic inflammatory disease marked by polyarthritis which affects the joints symmetrically, leading to progressive damage of the bone structure and eventually joint deformity. Lung involvement is the most prevalent extra-articular feature of RA, affecting 10–60% of patients with this disease. In this review, we aim to discuss the patterns of RA interstitial lung disease (ILD), the molecular mechanisms involved in the pathogenesis of ILD in RA, and also the therapeutic challenges in this particular extra-articular manifestation. The pathophysiology of RA-ILD has been linked to biomarkers such as anti-citrullinated protein antibodies (ACPAs), MUC5B mutation, Krebs von den Lungen 6 (KL-6), and other environmental factors such as smoking. Patients at the highest risk for RA-ILD and those most likely to advance will be identified using biomarkers. The hope is that finding biomarkers with good performance characteristics would help researchers better understand the pathophysiology of RA-ILD and, in turn, lead to the development of tailored therapeutics for this severe RA manifestation.

Interstitial lung disease (ILD) is one of the common and potentially lethal manifestations of systemic autoimmune rheumatic diseases (SARDs). ILD’s prevalence, clinical patterns, imaging, and natural history are variable. Each of the representative diseases—systemic sclerosis (SSc), idiopathic inflammatory myopathies (IIMs), rheumatoid arthritis (RA), Sjӧgren’s syndrome (SjS), mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE)—have distinct clinical, paraclinical and evolutionary features. Risk factors with predictive value for ILD have been identified. This review summarizes, from the clinician’s perspective, recent data from the literature regarding the specificity of ILD for each of the autoimmune rheumatic diseases, with an emphasis on the role of the multidisciplinary team in early diagnosis, case management, as well as the particularities of the clinical approach to the progressive phenotype of ILD in SARDs.