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How do you approach the initial treatment for a patient presenting with MAS/HLH as the initial manifestation of SLE?
Patient presenting with seizure, rash, abnormal brain MRI, inflammatory CSF, ferritin >20k, leukocytosis, abnormal LFTs, positive ANA, positive Smith. Patient has a partial response to high dose steroids. Would you consider anakinra or more SLE specific therapy?
Answer from: at Community Practice
For rare manifestions of systemic lupus erythematosus (SLE) such as macrophage activation syndrome/hemophagocytic lymphohistiocytosis (MAS/HLH), I like to refer to the recent paper by the Members of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases, th...
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Answer from: at Community Practice
Severe SLE flares can be complicated by MAS, but it is also important to determine whether there is another inciting trigger, such as herpes virus infection (EBV, CMV, HSV). Absent that, the majority of MAS episodes in the context of SLE flare will usually respond to aggressive SLE-directed therapy....