Lupus Nephritis in Thrombotic Thrombocytopenic Purpura with Low ADAMTS13 Activity

This protocol addresses lupus nephritis presenting alongside thrombotic microangiopathy confirmed by low ADAMTS13 activity, microangiopathic haemolytic anaemia, and thrombocytopenia — a combination that signals thrombotic thrombocytopenic purpura (TTP) as a concurrent or causally related process.

Clinical Scenario

The finding of low ADAMTS13 activity in a patient with lupus nephritis confirms TTP. This presentation — thrombotic microangiopathy, microangiopathic haemolytic anaemia, and thrombocytopenia — requires a management approach that addresses both the renal lupus and the TTP simultaneously. Plasma exchange is a recommended early step, followed by immunosuppressive treatment directed at the underlying lupus nephritis.

Treatment Approach (Partial)

In cases where TTP does not respond adequately, a targeted anti-von Willebrand factor agent may be considered as part of the escalated approach — details on sequencing, eligibility criteria, and the full treatment algorithm are in the complete protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.ard.2025.09.007

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.

The presence of low ADAMTS13 activity confirms a diagnosis of TTP, wherein plasma exchange is recommended, followed by immunosuppressive treatment for LN.

Caplacizumab, a bivalent variable domain-only immunoglobulin fragment against von Willebrand factor approved for the treatment of acquired TTP, can be considered in nonresponding TTP patients, although data in patients with SLE are currently limited to a few case reports.

View source ↗