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

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.