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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:
Table 1
Organ involvement Treatment options Ancillary therapy % Agreement (median) 1st-line or Induction 2nd-line or failure of induction 3rd-line Maintenance 1. Constitutional symptoms GC, HCQ, IMM or combinations MMF Switching to RTX or BLM N/A N/A 60 2. Generalized DLE HCQ ± GC Adding AZA or switching antimalarial Switching AZA to MMF N/A Sun screening + Topical steroids or Topical tacrolimus 70 3. Uncomplicated digital/cutaneous vasculitis GC ± HCQ ± MTX AZA or MMF Switching to IV CYC N/A N/A 80 4. Gangrenous digital/cutaneous vasculitis GC + IV CYC Adding RTX or PLAX N/A AZA or MMF PGA 90 5. Non-erosive, non-deforming polyarthritis HCQ ± GC Adding MTX Adding RTX N/A N/A 80 6. Lupus pericarditis GC ± HCQ Adding MMF or AZA or MTX Adding BLM or RTX N/A Pericardiocentesis ± window 75 7. Lupus myocarditis GC + IV CYC ± HCQ Adding RTX or BLM or IVIG N/A MMF N/A 90 8. Lupus ILD GC + MMF or IV CYC Adding RTX or IVIG N/A AZA or MMF N/A 90 9. Lupus PAH GC + IV CYC or MMF + ERA Adding RTX, and PDE5i Adding PGA MMF N/A 80 10. Lupus thrombocytopenia GC ± HCQ Adding AZA or MMF Adding RTX or IV CYC or IVIG N/A Splenectomy 50
Conclusions: Consensus for SLE treatment had variable agreement but some treatment consensus beyond first line therapy was obtained.
Disclosure of Interest: None declared
DOI: 10.1136/annrheumdis-2014-eular.2541