This protocol covers lupus nephritis presenting in the context of thrombotic microangiopathy (TMA) with confirmed low ADAMTS13 activity, accompanied by microangiopathic haemolytic anaemia and thrombocytopenia — a high-acuity combination that requires a distinct management pathway.
Low ADAMTS13 activity in this setting confirms a concurrent thrombotic thrombocytopenic purpura (TTP)-like process alongside lupus nephritis. The triad of microangiopathic haemolytic anaemia, thrombocytopenia, and depressed ADAMTS13 distinguishes this sub-population and drives specific management decisions beyond standard lupus nephritis care alone.
Management centres on plasma exchange as the primary urgent intervention, used in combination with anti-inflammatory therapy; the complete agent selection, sequencing, and subsequent immunosuppressive regimen for lupus nephritis are detailed in the full protocol.
The presence of low ADAMTS13 activity confirms a diagnosis of TTP, wherein plasma exchange is recommended, followed by immunosuppressive treatment for LN.
In patients with features of thrombotic microangiopathy (antiphospholipid syndrome nephropathy, thrombotic thrombocytopenic purpura-like, or complement mediated hemolytic uremic syndrome), glucocorticoids (IV pulse methylprednisolone), complement inhibitors, B-cell depleting agents, caplacizumab, plasma exchange, and/or anticoagulation should be considered.
DOI: 10.1016/j.ard.2025.09.007
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