ERN ReCONNET-SLICC-SLEuro consensus on the therapeutic management of rare systemic lupus erythematosus manifestations (part 2).

Abstract

Systemic lupus erythematosus (SLE) guidelines predominantly focus on common major organ involvement. An international taskforce from three SLE expert groups (European Reference Network on Connective Tissue and Musculoskeletal Diseases, Systemic Lupus International Collaborating Clinics, and the European Lupus Society) previously developed consensus therapeutic strategies for 24 rare SLE manifestations. Here, 77 participants contributed to the development of consensus therapeutic strategies for 22 additional rare SLE manifestations, including diffuse pulmonary haemorrhage, rare cutaneous manifestations (bullous lupus, chilblain lupus, lupus tumidus, erythema multiforme, and toxic epidermal necrolysis-like lupus erythematosus), renal manifestations (interstitial nephritis and lupus podocytopathy), rare neurological manifestations (chorea, small fibre neuropathy, catatonia, and intracranial hypertension), rare gastrointestinal manifestations (protein-losing enteropathy, lupus hepatitis, intestinal pseudo-obstruction, and peritonitis), musculoskeletal manifestations (myositis and Jaccoud's arthropathy), and other rare manifestations such as uveitis, angioedema due to anti-C1 esterase inhibitor antibodies, interstitial cystitis, and lupus mastitis. These expert-based therapeutic strategies provide a framework for guiding therapeutic decisions where evidence-based recommendations might be insufficient.