Plasma Exchange as the Rescuing Therapy for Critical Diffuse Alveolar Hemorrhage in Systemic Lupus Erythematosus: Experiences of 28 Patients in One Center

Abstract

Objective: To determine the role of plasma exchange (PE) in patients with systemic lupus erythematosus (SLE) with diffuse alveolar hemorrhage (DAH).Methods: We performed a retrospective analysis to evaluate patients in critical condition with SLE with DAH who underwent rescue plasma exchange (RPE) between February 1985 and January 2012.Results: Our study included 28 patients with SLE with DAH. Twenty-two patients received PE with or without pulse methylprednisolone therapy as rescue therapies and other six patients did not undergo the PE. Overall PEs were performed 189 sessions, with a median of seven sessions for each patient per course (range, 2-33). Four of these patients died at therapies: one of septicemia, one of DAH with acute respiratory failure, and two of cardiac thrombosis with circulation failure. The overall 5-year survival rate was better in patients receiving RPE (64% vs. 33%). No patient developed anaphylaxis. Patients received methylprednisolone pulse therapy (1500-6000 mg) simultaneously with or separately from RPE. In all courses, we evaluated the disease activity with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and the mean SLEDAI scores were 24.9 and 9.1 before and 3 weeks after RPE, respectively.Conclusions: Our data suggest that the patients with SLE in critical condition of DAH not receiving PE may have a higher immediate mortality rate in comparison with those receiving PE with or without concomitant pulse methylprednisolone therapy.