Drugs used in the treatment of SLE Figure 1 Treatment of non-renal SLE-recommended drugs with respective grading of recommendation. aPL, aniphospholipid antibodies; AZA, azathioprine; BEL, belimumab; BILAG: British Isles Lupus Assessment Group disease activity index; CNIs, calcineurin inhibitors; CYC, cyclophosphamide; GC, glucocorticoids; HCQ, hydroxychloroquine; IM, intramuscular; MMF, mycophenolate mofetil; MTX, methotrexate; Pre, predinisone; PO, per os; RTX, rituximab, PLTs: Platelets; SLEDAI, Systemic Lupus Erythematosus Disease Activity Index. Fanouriakis A, Kostopoulou M, Alunno A, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosusAnnals of the Rheumatic Diseases 2019;78:736-745. Treatment of non-renal Systemic Lupus Erythematosus Adjunct: Sun protection Vaccinations Exercise No soking Body weight Blood pressure Lipids Glucose Antiplatelets anti-coagulants (in aPL-positive patient) Target Remission SLEDAI = 0 HCQ No GC or Low disease activity SLEDAI ≤ 4 HCQ Pre ≤ 7.5 mg/d Immunosuppressives (in stable doses and well-tolerated) Mild* HCQ GC PO/IM MTX /AZA BEL GC PO/IV st 1 line st 1 line st 1 line Refractory Refractory Refractory CYC RTX Moderate* Severe* CNI MMF Grade A Grade B Grade C Grade D 3 3- Mild: constitutional symptoms / mild arthritis / rash ≤9% BSA/PLTs 50-100 x 10 /mm . SLEDAI≤6: BILAG C or ≤1 BILAG B manifestation Moderate: RA-like arthritis / rash 9-18% BSA/cutaneous vasculitis ≤18% BSA; PLTs 20*50 *103/mm/serosities; SLEDAI 7-12; ≥2 BILAG B manifestations Severe: major organ threatening disease (nephritis, cerebritis, myelitis, pneumonitis, mesenteric vasculitis; thrombocytopenia with platelets <103* 20/mm; TTP-like disease or acute hemophagocytic syndrome; SLEDAI>12; ≥1 BILAG A manifestations 28
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