Résumé
Objectives: the occurrence of severe infections (SI) in systemic sclerosis (ScS) affects the prognosis of the disease. Infectious risk factors vary across studies. We describe here the occurrence of severe infections (requiring hospitalization or resulting in death) as well as the main suspected risk factors. Methods: data were collected retrospectively from real-world ScS patients followed from 2010 to 2024. We recorded the occurrence of SI episodes and the targeted organ, as well as clinical and biological phenotypes, and treatment exposures. Results: among 111 included patients (64% female with a mean age of 54±16 years at diagnosis), 23 patients (21%) with 35 SI events were identified. Pulmonary infections were predominant (69%), followed by digestive infections (14%) and urinary tracts infections (9%). Two severe opportunistic infections were reported (CMV colitis and atypical Mycobacterial pneumonia). Patients with severe infections had more cardiovascular risk factors at diagnosis (hypertension, dyslipidemia, diabetes), more severe scleroderma involvement (ILD, PAH, renal crisis, cardiac involvement), and had received ≥1 immunosuppressive therapy. The presence of a severe infectious event was associated with higher mortality (43% vs 17%). Conclusion: SI events in ScS patients, especially targeting lung, are common and are associated with an increased risk of morbidity and mortality. Patients with baselines cardiovascular factors, or severe form of the diseases, including ILD, seem particularly at risk. Identifying risk factors and implementing prophylactic measures could impact the clinical trajectory of these patients.