Abstract
Immune thrombocytopenia (ITP) may lead to membranous nephropathy (MN). Here, we report a case of MN complicated by ITP and validate the hypothesis that circulating antiplatelet antibodies cause MN using immunofluorescence analysis for immunoglobulin (Ig) G subclass and anti-phospholipase A2 receptor (PLA2R) antibodies. A 39-year-old Japanese man with ITP, who had been treated with prednisolone for 10 months, achieved a stable disease condition. However, 4 months after tapering the dose down to 10 mg prednisolone, he developed nephrotic syndrome, with a urinary protein-to-creatinine ratio (U-PCR) of 10.6 g/g Cr and was admitted to our hospital. His platelet count, at 89,000/μL, was lower than the normal range, indicating the recurrence of ITP. Renal biopsy revealed the thickening of the glomerular basement membrane with the deposition of IgG and complement component 3. Predominant deposition of IgG1 and negativity for anti-PLA2R staining indicated secondary MN; however, no typical conditions of secondary MN were evident. Although oral prednisolone and cyclosporine A were administered, he was refractory to treatment. A total of 12 sessions of low-density lipoprotein apheresis (LDL-A) decreased his U-PCR to < 3 g/g Cr. Seven months after discharge, his U-PCR further decreased to 0.54 g/g Cr and platelet count recovered to > 200,000/μL. Our literature review reveals that this condition is refractory to steroid therapy. LDL-A can be an effective treatment in drug-resistant MN complicated by ITP.