ERN ReCONNET–SLICC–SLEuro consensus recommendations for rare SLE manifestations

Recommendations from the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ERN ReCONNET)–Systemic Lupus International Collaborating Clinics (SLICC)–European Lupus Society (SLEuro) international consensus were published in The Lancet Rheumatology by Arnaud et al. The aim of this consensus was to provide expert-informed therapeutic strategies for 22 rare manifestations of systemic lupus erythematosus (SLE), including pulmonary, cutaneous, renal, neurological, gastrointestinal (GI), and musculoskeletal manifestations.

Key data: Across numerous severe manifestations, combination strategies including methylprednisolone infusions followed by oral glucocorticoids + cyclophosphamide, then mycophenolate mofetil (MMF) or azathioprine are recommended. The combination is recommended alone for GI manifestations; with plasmapheresis for diffuse pulmonary hemorrhage; ± calcineurin- and angiotensin-targeted therapies for renal manifestations; with low-dose aspirin or anticoagulation for some neurologic manifestations; and with rituximab for some refractory cutaneous manifestations and lupus-related catatonia. Across cutaneous manifestations and some neurological and musculoskeletal manifestations, glucocorticoid combination strategies with biologics and/or immunosuppressants are recommended. Treatment adaptations or alternatives are also provided for non-severe manifestations.

Key learning: These expert-informed consensus strategies offer a practical, severity-stratified framework to guide treatment decisions across rare SLE manifestations, and may serve as a foundation for future registry-based studies and pragmatic clinical trials.