| Literature DB >> 30454884 |
Zao Zhang1, Ting Gong2, Helmut G Rennke3, Rick Hayashi4.
Abstract
Membranous nephropathy (MN) associated with malignancies is a well-known entity. However, its association with benign neoplasm is not broadly recognized. A 69-year-old man with recurrent nephrotic syndrome presented with pedal edema and proteinuria of 5 months' duration. Laboratory results showed hypoalbuminemia and hyperlipidemia. Proteinuria was estimated to be protein excretion of 3.5g/d. Studies were negative for viral hepatitis, syphilis, human immunodeficiency virus, autoimmune diseases, and paraproteinemia. Kidney biopsy disclosed MN with negative phospholipase A2 receptor (PLA2R) staining, favoring a secondary form of MN. Computed tomography detected a 7.6-cm duodenal schwannoma. Elective surgical resection was performed. Pathologic study showed that THSD7A (thrombospondin type 1 domain-containing 7A) was positive in both glomeruli and schwannoma. Commonly, secondary MN is related to underlying conditions, including lupus, hepatitis, and neoplasm, and can be medication induced. The risk for developing a concomitant neoplasm among patients with PLA2R-negative MN is up to 12 times higher than in the general population. Most of these neoplasms are malignancies, and the presence of autoantibodies directed at similar tissue targets is hypothesized as the potential mechanism. In our case, THSD7A may be the autoantibody that has linked the schwannoma and the development of MN. Although benign tumors rarely produce renal manifestations, effective treatment may lead to resolution of nephrotic syndrome.Entities:
Keywords: Secondary membranous nephropathy; autoantigen; benign neoplasm; duodenal schwannoma; kidney biopsy; paraneoplastic syndrome; phospholipase A(2) receptor (PLA(2)R); proteinuria; thrombospondin type 1 domain-containing 7A (THSD7A)
Mesh:
Year: 2018 PMID: 30454884 DOI: 10.1053/j.ajkd.2018.09.003
Source DB: PubMed Journal: Am J Kidney Dis ISSN: 0272-6386 Impact factor: 8.860