Membranous nephropathy and lupus-like syndrome after hematopoietic cell transplantation: a case report

Abstract

Introduction: The kidney is increasingly recognised as a target organ of chronic graft-versus-host disease after hematopoietic cell transplantation in the context of the development of the nephrotic syndrome. Chronic graft-versus-host disease is associated with autoimmune phenomena similar, but not identical, to those observed in various rheumatologic disorders, implicating autoimmunity as an important component of the disease.

Case presentation: We report the case of a 57-year-old Caucasian man who developed the nephrotic syndrome due to membranous nephropathy in association with recurrent chronic graft-versus-host disease, along with a lupus-like syndrome manifested with pancytopenia, hair loss, positive anti-DNA antibodies and sub-epithelial and mesangial immune deposits. To the best of our knowledge, this is the first case reported in the literature. The nephrotic syndrome subsided soon after he was treated with a short course of cyclosporin with steroids. Unfortunately he died seven months later due to a relapse of leukemia.

Conclusions: Our case report confirms the notion that chronic graft-versus-host disease is characterized by the appearance of autoimmune phenomena similar, but not identical, to those seen in autoimmune diseases. The decision for more immunosuppression has to be weighed against the need for preservation of the graft versus leukemia phenomenon.

Figure 1

A. Mild thickening and rigidity of the glomerular capillary walls with slight dilatation of the capillary lumens [JMSx400]; B. Focus of tubular atrophy and interstitial fibrosis (white arrow), global wrinkling and sclerosis of a glomerulus (white arrowhead) [JMSx200]; C. IgG immunohistochemical expression in glomerular capillary walls (black arrows) [IgGx400]; D. Sub-epithelial dense deposits (white arrow), effacement of epithelial foot processes and segmental spike formation (×13000).