Clinical-pathological features and prognosis of thrombotic thrombocytopenic purpura in patients with lupus nephritis

Am J Med Sci. 2009 Nov;338(5):343-7. doi: 10.1097/MAJ.0b013e3181b0c872.


Background: We investigated the clinical-pathological features and the prognosis of thrombotic thrombocytopenic purpura (TTP) in patients with lupus nephritis (LN).

Methods: A retrospective analysis was performed on the clinical-pathological data and prognosis in 8 patients with LN complicating with TTP.

Results: Thrombocytopenia and hemolytic anemia, neurologic symptoms, and renal dysfunction were the clinical manifestations in 8 patients. Six patients had fever. Eight patients presented with rapid progressive glomerulonephritis, and 1 patient with continuous gross hematuria. The histologic features of the 8 patients were thrombotic microangiopathy lesions. Immune-suppressive therapies were administrated in all patients, and blood purification therapy was applied in 7 patients. Three cases involved plasma exchange and/or immunoabsorption. Seven patients received a median follow-up of 12 months. One patient died, 3 cases received peritoneal dialysis, and 1 case failed to follow-up. During follow-up, 1 case was able to stop peritoneal dialysis, and 1 case changed to hemodialysis. The other 3 patients continued with stable renal function.

Conclusion: The patients with LN with TTP have severe clinical-pathological changes. Active treatment including renal replacement therapy, plasma exchange, and immunoabsorption are promising.

MeSH terms

  • Adolescent
  • Adult
  • Combined Modality Therapy
  • Female
  • Follow-Up Studies
  • Humans
  • Immunosorbent Techniques
  • Immunosuppressive Agents / therapeutic use
  • Kidney / blood supply
  • Kidney / pathology*
  • Lupus Nephritis / complications
  • Lupus Nephritis / diagnosis*
  • Lupus Nephritis / pathology
  • Lupus Nephritis / therapy
  • Male
  • Middle Aged
  • Plasma Exchange
  • Prognosis
  • Purpura, Thrombotic Thrombocytopenic / complications
  • Purpura, Thrombotic Thrombocytopenic / diagnosis*
  • Purpura, Thrombotic Thrombocytopenic / pathology
  • Purpura, Thrombotic Thrombocytopenic / therapy
  • Renal Replacement Therapy
  • Retrospective Studies
  • Young Adult


  • Immunosuppressive Agents