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Kidney Week

Abstract: TH-PO142

Atypical Multiorgan Disease Possibly Associated with IgG4RD

Session Information

Category: Trainee Case Report

  • 103 AKI: Mechanisms


  • Leger, Kathleen, Yale University School of Medicine, New Haven, Connecticut, United States
  • Huston, John, Yale University School of Medicine, Yale New Haven Hospital, New Haven, Connecticut, United States
  • Krishnan, Namrata, Yale University School of Medicine, Yale New Haven Hospital, New Haven, Connecticut, United States
  • Perazella, Mark A., Yale School of Medicine, Cheshire, Connecticut, United States
  • Belcher, Justin Miles, Yale University, New Haven, Connecticut, United States

IgG4 related disease (IgG4RD) is associated with multi-system disease processes. Hallmarks include lymphoplasmacytic infiltration with a predominance of IgG4+ plasma cells with associated storiform fibrosis and elevated serum IgG4 levels. We report a patient with multiple yet atypical manifestations suspicious for IgG4RD.

Case Description

38yo man with UC, T2DM, proteinuric CKD-2 (Cr-1.2) and hematuria was admitted for AKI and hyperkalemia. Chief complaints were chronic diarrhea and fatigue. Labs showed K-6.3, HCO3-9, Cr-2.6 and alk phos-861. U/A revealed hematuria and low-grade proteinuria. Renal u/s demonstrated right kidney-13cm and left-14cm. Abdominal u/s revealed dilated intrahepatic ducts. MRCP was unremarkable except for hepatomegaly and splenomegaly. CT abdomen/pelvis 3 months prior demonstrated enlarged retroperitoneal lymph nodes but no hepatomegaly/splenomegaly. Kidney biopsy revealed diabetic nephropathy and plasma cell rich interstitial infiltrate with 20% IgG4+ (>50/HPF) without storiform fibrosis. Serum IgG was 2424 (694-1618) with IgG1-1117 (382-929) and IgG4-338 (4-86). Liver biopsy revealed minimal plasma cell infiltrate with negative IgG4 staining and no sclerosing cholangitis. CT revealed increased retroperitoneal and inguinal lymphadenopathy. Prednisone 60 mg was initiated. He was subsequently re-admitted for brawny LE edema and SOB. Repeat CT demonstrated bilateral cavitary lung lesions and increased retroperitoneal/pelvic lymphadenopathy. Blood cultures were positive for MSSA, TEE revealed normal leaflets and no vegetations. Repeat kidney biopsy again revealed plasma rich interstitial infiltrate (>30 IgG4+ cells/HPF) and diabetic kidney disease. Lymph node biopsy was recommended but patient left prior to procedure.


IgG4RD is challenging to diagnose due to multiple disease manifestations. In our patient, findings consistent with IgG4RD include those on kidney biopsy along with retroperitoneal lymphadenopathy and possible fibrosis. However, not all diagnostic criteria were met and he did not respond to a course of steroids. Other diseases potentially associated with IgG4+ plasma cells within tissues (drugs, sarcoidosis, syphilis, GPA) were excluded in our patient. Our case may represent an atypical case of IgG4RD or manifestation of a yet to be diagnosed disease.