%0 Journal Article
%T Chronic interstitial lung disease associated with systemic lupus erythematosus: A multicentric study of 89 cases.
%A Deneuville L
%A Mageau A
%A Debray MP
%A Sacre K
%A Costedoat-Chalumeau N
%A Hachulla E
%A Uzunhan Y
%A Le Tallec E
%A Cadranel J
%A Marchand Adam S
%A Montani D
%A Rémi-Jardin M
%A Reynaud-Gaubert M
%A Prevot G
%A Beltramo G
%A Crestani B
%A Cottin V
%A Borie R
%A  
%J Respirology
%V 29
%N 8
%D 2024 Aug 17
%M 38494831
%F 6.175
%R 10.1111/resp.14703
%X <B>OBJECTIVE: </B>Chronic interstitial lung disease (ILD) occurs rarely with systemic lupus erythematosus (SLE) as compared with other connective tissue diseases. This multicentric retrospective study of patients with SLE-ILD from the OrphaLung and French SLE networks during 2005-2020 aimed to describe the characteristics of patients with SLE-ILD and analyse factors associated with prognosis.<BR><B>METHODS: </B>We analysed data for 89 patients with SLE-ILD (82 women, 92.1%) (median age at SLE diagnosis: 35 years [interquartile range 27-47]). All patients met the 2019 EULAR/ACR criteria for the diagnosis of SLE.<BR><B>RESULTS: </B>Forty two (47.2%) patients were positive for anti-ribonuclear protein antibodies and 45 (50.6%) for anti SSA/Ro antibodies. A total of 58 (65.2%) patients had another connective tissue disease: Sjögren's syndrome (n = 33, 37.1%), systemic sclerosis (n = 14, 15.7%), inflammatory myopathy (n = 6, 6.7%), or rheumatoid arthritis (n = 6, 6.7%). ILD was diagnosed along with SLE in 25 (28.1%) patients and at a median of 6 (0-14) years after the SLE diagnosis. The most frequent CT pattern was suggestive of non-specific interstitial pneumonia (n = 41, 46.0%) with or without superimposed organizing pneumonia. After a median follow-up of 86.5 [39.5-161.2] months, 18 (20.2%) patients had died and 6 (6.7%) underwent lung transplantation. The median 5-year and 10-year transplantation-free survival were 96% (92-100) and 87% (78-97). In total, 44 (49.4%) patients showed ILD progression. Cutaneous manifestations and Raynaud's phenomenon were associated with better survival. Only forced vital capacity was significantly associated with survival and ILD progression.<BR><B>CONCLUSIONS: </B>ILD is a rare manifestation of SLE with good overall prognosis but with possible risk of ILD progression. Patients with SLE-ILD frequently have another connective tissue disease.