A comprehensive multicentric study spanning 15 years has provided valuable insights into the rare but significant manifestation of chronic interstitial lung disease (ILD) in systemic lupus erythematosus (SLE) patients. This retrospective analysis published in Respirology, focusing on patients with SLE-ILD from the OrphaLung and French SLE networks, offers crucial data for rheumatologists managing this complex subset of patients.
Out of the 89 patients, nearly half (47.2%) tested positive for anti-ribonuclear protein antibodies, and over half (50.6%) were positive for anti SSA/Ro antibodies. Additionally, 65.2% of patients had other connective tissue diseases, such as Sjögren’s syndrome (37.1%) and systemic sclerosis (15.7%). ILD was diagnosed concurrently with SLE in 28.1% of patients, while others developed ILD with a median of six years post-SLE diagnosis. The most common CT pattern observed was non-specific interstitial pneumonia. After a median follow-up of over seven years, 20.2% of patients had died, and 6.7% underwent lung transplantation. The study reported a 96% 5-year transplantation-free survival rate and an 87% 10-year rate; however, nearly half (49.4%) experienced ILD progression. Cutaneous manifestations and Raynaud’s phenomenon were associated with better survival rates, while forced vital capacity was significantly linked to both survival and ILD progression.
A study by Mageau et al. utilized a nationwide French database to investigate the incidence and prevalence of ILD among SLE patients. Analyzing data from SLE inpatients admitted between 2011 and 2012, the study identified 134 (1.2%) patients with ILD at baseline. The frequency of ILD was notably higher in patients with associated autoimmune diseases such as Sjögren’s syndrome or systemic sclerosis. ILD was found to significantly increase the risk of death in SLE patients, with a hazard ratio of 1.992. Additionally, among 31,029 SLE patients without initial ILD, 795 (2.6%) developed ILD between 2013 and 2020, indicating an incidence rate of 10.26 per 1000 patient-years. The findings underscore that while ILD in SLE is rare, it is often linked to other autoimmune disorders and significantly heightens mortality risk.
The research highlights that while ILD is a rare complication of SLE, it carries a generally favorable prognosis despite the risk of progression. It also emphasizes the frequent overlap of SLE with other connective tissue diseases, necessitating vigilant monitoring and comprehensive care for affected patients. This study underscores the importance of early detection and management of ILD in SLE patients. By understanding the factors influencing prognosis, healthcare providers can better tailor treatment strategies to improve patient outcomes.
Reference
- Deneuville L, Mageau A, Debray MP, Sacre K, Costedoat-Chalumeau N, Hachulla E, Uzunhan Y, Le Tallec E, Cadranel J, Marchand Adam S, Montani D, Rémi-Jardin M, Reynaud-Gaubert M, Prevot G, Beltramo G, Crestani B, Cottin V, Borie R; OrphaLung Network. Chronic interstitial lung disease associated with systemic lupus erythematosus: A multicentric study of 89 cases. Respirology. 2024 Aug;29(8):713-721.
- Mageau A, Borie R, Crestani B, Timsit JF, Papo T, Sacre K. Epidemiology of interstitial lung disease in systemic lupus erythematosus in France: A nation-wide population-based study over 10 years. Respirology. 2022 Aug;27(8):630-634.