ERN ReCONNET-SLICC-SLEuro expert consensus on the therapeutic management of rare systemic lupus erythematosus manifestations.

Existing guidelines for systemic lupus erythematosus (SLE) predominantly focus on common and major organ involvements. An international taskforce involving experts from three SLE expert groups (ie, the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases, the Systemic Lupus Erythematosus International Collaborating Clinics group, and the European Lupus Society) was established. A total of 119 participants contributed to the development of consensus therapeutic strategies for 24 rare SLE manifestations, using a multistep process. For SLE enteritis and pancreatitis, experts recommended hydroxychloroquine, glucocorticoids, and cyclophosphamide or mycophenolate mofetil. Rare lung conditions such as pneumonitis were also managed with cyclophosphamide if severe or with mycophenolate mofetil if not severe. SLE for myocarditis with hydroxychloroquine, glucocorticoids, and cyclophosphamide or mycophenolate mofetil, are recommended based on severity. For CNS manifestations, hydroxychloroquine, glucocorticoids, and cyclophosphamide or mycophenolate mofetil were common choices for treatment. For rare skin manifestations, the preferred strategy was a combination of hydroxychloroquine and glucocorticoids with anifrolumab or mycophenolate mofetil. This expert-based consensus provides a valuable framework for guiding therapeutic decisions where the available recommendations might be insufficient or inapplicable.