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Case report: IgG4-related renal disease co-existing with retroperitoneal fibrosis.

ABSTRACT: A 51-year-old woman visited our clinic for orthopnea, chest discomfort, and facial and leg edema. Acute kidney injury and pleural effusion were diagnosed and she received hemodialysis. This is a rare case of IgG4-related disease diagnosed by renal biopsy in the patient with retroperitoneal fibrosis and severe acute kidney injury, which progressed rapidly and required hemodialysis without markedly elevated serum IgG4 level and urinary tract obstruction. Immunostaining of a kidney biopsy revealed IgG4-related renal disease and magnetic resonance imaging showed retroperitoneal fibrosis in the paraspinal and presacral areas. After 3 days of glucocorticoid treatment, renal function and bilateral pleural effusion were restored. Another 2 months of glucocorticoid treatment decreased the extent of retroperitoneal fibrosis on follow-up abdominal computed tomography. IgG4-related disease is a recently recognized fibro-inflammatory condition with a dense lymphoplasmacytic infiltration of IgG4-positive plasma cells. The kidney and retroperitoneum are the frequently associated organs in IgG4-related disease. This case shows that IgG4-related disease can involve the kidney and/or other organs and be diagnosed by kidney biopsy.

SUBMITTER: Kim YJ

PROVIDER: S-EPMC7215037 | biostudies-literature | 2020 Apr

REPOSITORIES: biostudies-literature

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Case report: IgG4-related renal disease co-existing with retroperitoneal fibrosis.

Kim Young Jin YJ Kim Ga-Eon GE Ma Seong Kwon SK Kim Soo Wan SW Bae Eun Hui EH

Translational andrology and urology 20200401 2

A 51-year-old woman visited our clinic for orthopnea, chest discomfort, and facial and leg edema. Acute kidney injury and pleural effusion were diagnosed and she received hemodialysis. This is a rare case of IgG4-related disease diagnosed by renal biopsy in the patient with retroperitoneal fibrosis and severe acute kidney injury, which progressed rapidly and required hemodialysis without markedly elevated serum IgG4 level and urinary tract obstruction. Immunostaining of a kidney biopsy revealed ...[more]

PMID: 32420186

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Project description:BackgroundChest pain is a common reason for admission to the Emergency Department and aortic disease is a relatively frequent cause amongst the total number of admissions due to chest pain. IgG4-related disease (IgG4-RD) is a multi-organ immune-mediated condition that mimics several malignant, infectious, and inflammatory disorders.Case summaryWe report a rare case of IgG4-related aortitis complicated with severe aortic regurgitation and multivessel coronary artery disease in a 64-year-old man with a history of atypical chest pain. The diagnosis was made performing transthoracic echocardiography, transoesophageal echocardiography, and left heart catheterization; the aortitis was an incidental finding discovered by computed tomography angiography. Unusually, the positron emission tomography-computed tomography (PET-CT) scans did not reveal metabolic activity in the aortic wall. This last finding prompted us to exclude more aggressives arteritis (such as Horton's disease or Takayasu arteritis); syphilis infection and other infective or autoimmune diseases were excluded with laboratory tests. The patient underwent cardiac surgery with replacement of both the aortic valve and the ascending aorta, also performing a coronary aortic bypass graft (CABG). Despite the PET-CT scans were negative in the aortic wall, the histological specimens showed diffuse lymphoid infiltration, fibro-atheromatosis lesions, and medium-interstitial hyperplasia compatible with aortic atherosclerosis and IgG4-RD. The post-surgery course was free of complications and the patient was discharged in good clinical condition. He was referred to the Rheumatologic Department and a corticosteroid therapy has been started.DiscussionThe growing recognition of IgG4-related systemic disease as a clinical entity underscores the importance of considering this diagnosis in patients with any type of idiopathic aortitis and aortic valve disease. Noteworthy, the PET-CT scans could be negative in this disease, so histological exams are mandatory to make the diagnosis; in our case an unusual post-surgery histopathological finding prompted us to diagnose a rare, unrecognized disease and allowed us to treat the patient properly.

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Case report: IgG4-related renal disease co-existing with retroperitoneal fibrosis.

Publications

Case report: IgG4-related renal disease co-existing with retroperitoneal fibrosis.

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