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

Area of Science:

• Rheumatology
• Immunology
• Internal Medicine

Background:

• Existing guidelines for systemic lupus erythematosus (SLE) primarily address common organ involvements.
• Rare SLE manifestations often lack specific, evidence-based treatment recommendations.
• An international consensus was needed to guide management of uncommon SLE presentations.

Purpose of the Study:

• To develop consensus-based therapeutic strategies for 24 rare manifestations of systemic lupus erythematosus (SLE).
• To provide clinicians with a framework for managing SLE cases not covered by existing guidelines.

Main Methods:

• An international taskforce comprising experts from three major SLE groups was formed.
• A multistep consensus process involving 119 participants was employed.
• Therapeutic recommendations were developed for 24 rare SLE manifestations.

Main Results:

• Specific treatment regimens were proposed for SLE enteritis, pancreatitis, myocarditis, CNS manifestations, and rare lung/skin conditions.
• Recommended therapies often included hydroxychloroquine, glucocorticoids, cyclophosphamide, mycophenolate mofetil, and anifrolumab, tailored to severity and manifestation.
• Consensus strategies were established for managing diverse and rare SLE presentations.

Conclusions:

• This expert-based consensus offers a valuable framework for therapeutic decisions in rare SLE manifestations.
• The guidelines address critical gaps in managing uncommon SLE presentations, improving patient outcomes.
• The consensus provides practical guidance where standard recommendations are insufficient or inapplicable.