Journal Article

POS1518 PULMONARY INVOLVEMENT IN LUPUS IS ASSOCIATED WITH ENHANCED MORBIDITY: A MULTICENTRE STUDY

Pellico, M. R., Dagna, L., Cavallo, L., Caporali, R., Bozzolo, E., Ramirez, G. A., Scotti, I., Argolini, L. M., Artusi, C., Gerosa, M., Moroni, L.

2023

Overview

BackgroundAlthough several studies report a high incidence of pulmonary manifestations in SLE, lung involvement is often underestimated in SLE clinical assessment [1]. This is also mirrored by the absence of pulmonary manifestations other than pleurisy in the old and new classification criteria for SLE [2]. Moreover, limited evidence on the management of SLE-related pulmonary manifestations is available.ObjectivesTo assess prevalence and clinical impact of the spectrum of SLE-related pulmonary manifestations and their association with patient autoantibody profiles in a large SLE cohort and to describe the effectiveness of different therapeutic approaches in distinct clinical settings.MethodsPatients followed at the Lupus Clinics of ASST G. Pini-CTO and San Raffaele Hospital (Milan, Italy) were enrolled. Data regarding demographics, disease characteristics, autoantibody profile, pulmonary manifestations, damage accrual and treatment were collected. The following types of lung involvement were recorded: pleurisy, acute lupus pneumonitis, interstitial lung disease (ILD), alveolar haemorrhage, pulmonary embolism, arterial pulmonary hypertension and shrinking lung syndrome.ResultsOf the 471 SLE patients enrolled, we identified 78 patients (16.5%) displaying at least one pulmonary manifestation. Epidemiological data on each manifestation are reported in Table 1. Pleurisy was the most common pulmonary manifestations and manifested at disease onset in most cases (56%).Patient home environment (urban vs countryside) did not seem to impact the risk of developing lung disease. Damage accrual was relevant, as 2/3 of patients displayed at least 1 point increase in SLICC Damage Index (SDI) after the onset of lung involvement in comparison to baseline. All patients received at least one steroid course. Immunosuppressive treatment choices and efficacy differed among distinct manifestations: only half of the patients with pleurisy received immunosuppression, mainly azathioprine, with 100% of improvement, while 80% of cases of ILD received immunosuppression, predominantly mycophenolate, with a 50% risk of non-response.By comparing demographics and clinical characteristics among cases and controls, we found a significantly lower median age at disease onset (p=0.002) and a higher frequency of male sex (18% vs 9%; p=0.07), joint involvement (p=0.02) and constitutional symptoms (p=0.02) in patients with lung involvement, while no differences were observed in the autoantibody profile, including anti-dsDNA and anti-ENA autoantibodies.ConclusionOur study confirms that, in addition to the known epidemiological burden of pleurisy, other types of pulmonary involvement can complicate the disease course and contribute to damage accrual. In particular, ILD can frequently occur and respond to immunosuppressants in only half cases. Consistent with the association of lung involvement with increased morbidity, higher-risk categories for severe disease such as males and subjects with early-onset SLE were more represented among patients with pulmonary manifestations.References[1] Amarnani R, et al. Front. Med. 2021; 7:610257.[2] Aringer M, et al. Ann Rheum Dis. 2019; 78:1151-1159.[3] Smith EMD, Clin Immunol. 2019; 209:108274.[4] Ryu S, et al. Lupus Sci Med. 2017;):e000221.Table 1.Demographic and disease characteristics of SLE patients with lung involvementDemographics and clinical characteristicsFemales, n (%)65 (83)Age at diagnosis (years): median (IQR)29 (22 – 39)Disease duration (years): mean ±DS19 ± 12Subjects with >0 and >1 point SDI increase from baseline: n (%)48 (61.5); 26 (33)Lung involvementN (%)immunosuppressive therapy, %Pleurisy61 (78)49Acute pneumonitis4 (5)100ILD15 (19)80PAH6 (8)50Pulmonary embolism4 (5)50Shrinking lung1 (1)100Alveolar hemorrage4 (5)100Acknowledgements:NIL.Disclosure of InterestsMaria Gerosa: None declared, Giuseppe Alvise Ramirez: None declared, Lorenza Maria Argolini: None declared, Isabella Scotti: None declared, Carolina Artusi: None declared, Luca Moroni: None declared, Enrica Bozzolo: None declared, Maria Rosa Pellico: None declared, Ludovica Cavallo: None declared, Lorenzo Dagna: None declared, Roberto Caporali Speakers bureau: AbbVie, Amgen, BMS, Celltrion, Fresenius, Galapagos, Janssen, Lilly, Novartis, Pfizer, and UCB, Consultant of: AbbVie, Fresenius, Galapagos, Lilly, Novartis, Pfizer, and UCB,

• Publisher: BMJ Publishing Group Ltd and European League Against Rheumatism,Elsevier B.V,Elsevier Limited
• Subject: Age / Alveoli / Anti-DNA antibodies / Autoantibodies / Azathioprine / Demographics / Demography / Embolism / Epidemiology / Hemorrhage / Immunosuppression / Immunosuppressive agents / Lung diseases / Lungs / Lupus / Morbidity / Mycophenolic acid / Patients / Pleurisy / Pneumonitis / Pulmonary embolisms / Scientific Abstracts / Systemic lupus erythematosus