Background Treatment for SLE is often organ based. The literature lacks trials and consensus for treatment in SLE when standard first or second line care is ineffective.
Objectives To determine expert consensus for SLE treatment using case scenarios and especially for treatment beyond first line therapy.
Methods SLE experts (n=69) were sent three surveys; writing therapies for SLE organ complications assuming inadequate response to each choice and providing a list of secondary choices.
Results The response rate for the first survey where all treatment options were written by the experts was 54%. For each subsequent survey, the response rate decreased. For widespread DLE, first-line: topical steroids or tacrolimus+hydroxychloroquine (HCQ) ± glucocorticoids, then azathioprine (AZA) and switching to mycophenolate mofetil (MMF). For cutaneous vasculitis, first-line was GC ± HCQ ± methotrexate (MTX), followed by adding either AZA or MMF and then IV cyclophosphamide (CYC). For gangrenous vasculitis, first-line was glucocorticoids+CYC, then rituximab (RTX) or plasmapheresis and maintenance with AZA or MMF. For arthritis, first-line therapy was HCQ ± glucocorticoids; adding MTX and then RTX. For pericarditis refractory to NSAIDs, first-line was glucocorticoids ± HCQ, then adding AZA, MMF or MTX and then Belimumab (BLM) or RTX; and if needed pericardial window and/or aspiration. For ILD, induction was glucocorticoids+MMF or CYC, then RTX or IVIG; maintenance with AZA or MMF. For PAH, glucocorticoids+CYC or MMF+endothelin receptor antagonist, adding phosphodiesterase-5 inhibitor and then prostanoid and RTX. First-line therapy was anticoagulation ± HCQ for lupus associated antiphospholipid antibody syndrome. A direct thrombin inhibitor was second-line therapy for venous thrombosis, and adding low dose aspirin or another platelet aggregation inhibitor was a second-line option for arterial thrombosis. For mononeuritis multiplex, and CNS vasculitis, first-line induction was glucocorticoids+CYC followed by maintenance with AZA, or MMF and then RTX, IVIG or plasmapheresis. For LN type III/IV and V first-line was glucocorticoids+MMF, then adding RTX for LN type III/IV or switching to AZA, CYC or RTX for LN type V. Treatment algorithm for organ system involvements by systemic lupus erythematosus:
Conclusions Consensus for SLE treatment had variable agreement but some treatment consensus beyond first line therapy was obtained.
Disclosure of Interest None declared