Monthly pemetrexed treatment had been performed twice at a dose of 500 mg/m2 before this hospitalization, but had been discontinued after serum creatinine level elevated from 0.8 mg/dl to 2.4 mg/dl. Serum immunoglobulin (Ig)G, serum IgG4, and urinary β2 microglobulin

Gefitinib nmr levels were 2552 mg/dl, 227 mg/dl, and 17.35 mg/l, respectively. Computed tomography showed bilateral renal swelling. Renal biopsy revealed tubulointerstitial nephritis with increased IgG4-positive plasma cells and storiform fibrosis. IgG4-related kidney disease was diagnosed definitively using the most suitable diagnostic algorithm, and oral prednisolone was administered at an initial dose of 40 mg/day. Two months after starting therapy, serum creatinine, serum IgG, serum IgG4 and urinary β2 microglobulin levels had decreased to 1.13 mg/dl, 1254 mg/dl, 101 mg/dl and 0.13 mg/l, respectively. Renal re-biopsy PKC412 mouse showed a reduced number of infiltrated plasma cells and fibrotic lesions. IgG4-related disease is a recognized fibroinflammatory condition characterized by tumefactive

lesions, dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and elevated serum concentration of IgG4. The most common feature of the renal involvement in IgG4-related disease, termed IgG4-related kidney disease, is tubulointerstitial nephritis with abundant IgG4-positive plasma cells. Pemetrexed is an antifolate agent for the treatment of advanced lung cancer. Although major side effects of pemetrexed include myelosuppression and neutropenia, some aminophylline cases of renal dysfunction have been reported. Pathological features include acute tubular injury and interstitial nephritis with fibrosis without any information about IgG4-positive plasma

cells. This represents the first case of IgG4-related kidney disease after administration of pemetrexed for adenocarcinoma of the lung. PARTININGRUM DWI L1,2, FARADZ SULTANA MH2, LESTARININGSIH LESTARININGSIH1, VAN DEN HEUVEL LAMBERT3 1Nephrologi-Hypertension Division, Internal Medicine, Medical Faculty Diponegoro University, Semarang – Indonesia; 2Centre for Biomedical Research, Medical Faculty Diponegoro University, Semarang, Indonesia; 3Department of Pediatrics, Institute for Metabolic and Genetic Disease, Radboud University Medical Centre, Nijmegen, The Netherlands Diseases of the glomerular filter of the kidney are a leading cause of end-stage renal failure. Idiopathic Nephrotic Syndrome regarded as sporadic disease, but genetic factors cannot be ignored. Several genes and protein that involved in the maintenance of protein barrier in slit diaphragm have been recognized. CD2AP shows a key role in the kidney where it is essential for the ultrafiltration functions of the slit-diaphragm network.