When lupus nephritis is accompanied by thrombotic microangiopathy (TMA) and positive antiphospholipid antibodies — particularly at high titres — the clinical picture points toward APS nephropathy. This combination requires a specific management approach guided by structured evidence.
This protocol applies to lupus nephritis presenting with thrombotic microangiopathy and positive antiphospholipid antibodies. Positivity at high titres establishes the diagnosis of APS nephropathy, which directly determines the therapeutic strategy.
The approach in this setting incorporates anticoagulation as a central component alongside IV pulse corticosteroid therapy. The complete regimen — including agent selection, sequencing, and any additional interventions — is outlined in the full structured protocol.
DOI: 10.1016/j.ard.2025.09.007
Finally, anticoagulation is recommended in the presence of TMA accompanied by positive antiphospholipid antibodies, especially at high titres, which establishes the diagnosis of APS nephropathy.
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.
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