Background: Interstitial lung disease (ILD) represents a frequent cause of morbidity and mortality in primary Sjogren syndrome (pSS). However, the prevalence and behavior of pSS-ILD remains incomplete, largely based on retrospective heterogeneous studies. Aim of the study: To investigate the prevalence of progressive pulmonary fibrosis (PPF) in a multicentric cohort of patients with pSS-ILD. Additionally, this study explored possible correlations between PPF and clinical, demographic, and serological features of pSS. Methods: All consecutive patients with pSS-ILD were enrolled in a 6-month period. Clinical, demographic, and serological features of pSS, other than functional and radiological lung features, were collected. Clinical behaviors of ILD other than PPF were also investigated. Results: Seventy-two patients were enrolled. A fibrosing ILD pattern was observed in 65.3% of patients with pSS-ILD; among them, 55.3% showed a PPF. The radiologic pattern (NSIP, UIP, or others) was not associated with PPF; in particular, patients with PFF had UIP in 42.3% of cases and NSIP in 57.7%, without a significant difference with respect to the non-PPF group (p = 0.29). Shorter pSS disease duration, higher age at pSS diagnosis, and lower frequency of antinuclear antibodies were correlated with the PPF subgroup. However, multivariate analysis did not confirm these associations. Discussion: This study provides valuable insights into the prevalence and characteristics of PPF in pSS-ILD. In particular, UIP and NSIP showed a similar evolution towards PPF in patients with pSS; for NSIP, this behavior was more frequent than for other rheumatic diseases. Our results emphasize the importance of early recognition of PPF for timely intervention and careful follow-up. Conclusions: This study provides valuable insights into the prevalence and characteristics of PPF in pSS-ILD. In particular, UIP and NSIP showed a similar evolution towards PPF in patients with pSS; for NSIP, this behavior was more frequent than for other rheumatic diseases. Our results emphasize the importance of early recognition of PPF for timely intervention and careful follow-up.
Introduction: This study evaluates the real-world application of anifrolumab in managing articular involvement in systemic lupus erythematosus (SLE), providing insights into its efficacy and safety in routine clinical practice. Additionally, a systematic review examines anifrolumab’s role specifically in joint manifestations of SLE, consolidating existing real-world data on its therapeutic impact in articular disease. Methods: This monocentric case series presents data from four patients with SLE-related arthritis treated with anifrolumab. Clinical outcomes, including joint symptoms, clinimetric indices (DAS28, SLEDAI-2K, and SLICC), and treatment tolerability, were assessed. Ultrasound evaluation did not represent an outcome since it was not performed regularly. A systematic review was conducted to explore anifrolumab’s real-world application in articular disease manifestations, offering a comparative perspective. Results: All patients achieved complete remission of arthritis and lupus disease activity within four months, with no serious adverse reactions and without treatment discontinuation. Additionally, two patients completely discontinued corticosteroid (GC) therapy within two months, while the remaining two significantly reduced their GC doses. Only three promising relevant articles emerged from the systematic review, underlining the need for further studies to better support the role of anifrolumab in the treatment of arthritis in SLE. Conclusions: These findings highlight anifrolumab’s practical utility in real-world settings, particularly for articular involvement, while the systematic review contextualizes its impact within SLE management. The results underscore anifrolumab’s potential as a valuable treatment option for joint manifestations of SLE, addressing an unmet clinical need in routine practice. This evidence may assist clinicians in selecting the most suitable therapeutic approach based on predominant clinical features, thus enhancing personalized treatment strategies in SLE.