ANCA‑Associated Vasculitis with Systemic Thrombotic Microangiopathy: A Literature Review

Abstract / Summary:

This review focuses on patients with ANCA‑associated vasculitis (AAV) who also develop systemic thrombotic microangiopathy (TMA), a rare but severe complication. TMA conditions discussed include hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP).

The authors conducted a literature search covering publications from January 1998 to September 2022. They selected 15 reported cases of concurrent AAV and systemic TMA. The median patient age was 61 years, and approximately two-thirds were female. MPO‑ANCA positivity was more frequent than PR3‑ANCA positivity.

Key Findings:

• Most patients achieved hematological remission after treatment.

• Kidney involvement varied among patients, with some requiring intensive renal support.

• Early recognition of TMA in AAV patients is crucial for effective multidisciplinary management.
  

  
  Significance:
  

This review highlights the complexity of managing AAV when associated with systemic TMA. Clinicians in rheumatology, nephrology, and internal medicine can benefit from understanding these rare but severe presentations to improve outcomes.

Authors:

Indian Journal of Nephrology (2022)

Source / PDF Link:Download PDF – Indian Journal of Nephrology