Interstitial lung disease throughout the rheumatoid arthritis disease course

Abstract

Purpose of reviewTo summarize the current understanding of rheumatoid arthritis-associated interstitial lung disease (RA-ILD) throughout the rheumatoid arthritis (RA) disease course from preclinical to established disease. Recent findingsILD is a serious extra-articular manifestation of RA. Multiple studies have demonstrated a high prevalence of both subclinical and clinical ILD throughout the RA disease course. Investigations of patients without RA have demonstrated an association between RA-related autoantibodies like anticitrullinated protein antibodies (ACPA) and interstitial abnormalities on lung imaging. A significant proportion of RA-ILD patients develop ILD prior to articular manifestations, suggesting that the lung plays a central role in RA development, perhaps through ACPA production. RA-ILD also occurs in early RA, when exuberant autoantibody production and systemic inflammation may propagate pulmonary disease activity. In patients with established RA, a high burden of subclinical and clinical ILD results in significant morbidity, mortality, and healthcare expenditure. Multiple epidemiologic and genetic risk factors, as well as serum biomarkers, have been associated with RA-ILD.

Lifetime risk of rheumatoid arthritis-associated interstitial lung disease in MUC5B mutation carriers

“…Prevalence of RA-ILD shows high variability in the literature depending on the population, diagnostic methods and disease definitions used 13. Our lifetime risks compare well with previous estimates of clinically significant disease, reported to occur in up to 5%–10% of patients with RA 2–4.…”

Towards clinical significance of the MUC5B promoter variant and risk of rheumatoid arthritis-associated interstitial lung disease

“…As also noted in other studies, RA-ILD risk seems to be most pronounced in older patients 16 17. More research is needed to understand how MUC5B and the ageing process may impact RA-ILD risk 18…”

Dominating Cause of Pulmonary Hypertension May Change Over Time—Diagnostic and Therapeutic Considerations in a Patient with Pulmonary Hypertension Due to Rheumatoid Arthritis with Lung Involvement

“…On the contrary, female sex is a risk factor for PAH, both idiopathic and connective tissue associated. Lung involvement in RA concerns genetically susceptible individuals with coexisting inducing factors, such as environmental exposure to dusts or cigarette smoke [10,11]. The pathological autoantibodies directed against mucosal surfaces of the lung initiate a chronic inflammatory reaction, activating neutrophils and forming neutrophil extracellular traps (NETs) [11].…”

“…In the case of lung involvement, the final diagnosis relies on the results of high-resolution computed tomography of the lungs (HRCT) and pulmonary function assessment. The extension of Diagnostics 2021, 11,1931 2 of 9 lung disease seen on HRCT calculated as <20% and FVC > 70% allows the exclusion of significant lung disease in PAH CTD [2]. Such criteria are used in most randomised clinical trials and are considered clinically reliable.…”