ASN's Mission

ASN leads the fight to prevent, treat, and cure kidney diseases throughout the world by educating health professionals and scientists, advancing research and innovation, communicating new knowledge, and advocating for the highest quality care for patients.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on Twitter

Kidney Week

Abstract: PO0132

A Rare Case of a 27-Year-Old Man with IgG4-Related Disease

Session Information

Category: Trainee Case Report

  • 102 AKI: Clinical, Outcomes, and Trials

Authors

  • Pillarisetty, Sai Shalini, New York City Health and Hospitals Coney Island, Brooklyn, New York, United States
  • Sonde, Sumedha, New York City Health and Hospitals Coney Island, Brooklyn, New York, United States
  • Lee, Winston, New York City Health and Hospitals Coney Island, Brooklyn, New York, United States
Introduction

IgG4-related disease(IgG4-RD) is a rare multisystem autoimmune disease that was first reported in 2010. We present an unusual case of IgG4-RD in a young man demonstrating the heterogeneity of IgG4-RD with multisystem involvement and irreversible organ damage.

Case Description

A 27 year old obese male who presented with 3 month history of vomiting, diarrhea, epigastric pain, left lower extremity edema and severe fatigue. On admission patient appeared toxic, tachycardic and hypertensive with left leg elephantiasis nostras verrucosa. Lab work was significant for: Hgb 4 mg/dl, BUN/Cr 247/33.04, lipase 1100 U/L (13-60),TPO antibodies 43.6 IU/mL (<=34.9), TSH 8.71uIU/mL (0.35-5.50). CT scan confirmed acute pancreatitis with diffuse lymphadenopathy. Hemodialysis was initiated and the patient received multiple blood transfusions (17 units of PRBC) while on Aranesp with no significant improvement in hemoglobin. Blood antibody screening showed IgG4 warm antibodies and elevated serum IgG4 229 mg/dl (2.4- 24), IgG 2305 mg/dl (610-1660) and IgE 4303KU/L (<100). Renal biopsy showed chronic tubulointerstitial nephritis with global and focal segmental glomerulosclerosis and arterial sclerosis. Immunofluorescence stained for IgG, IgA, IgM, C3, C1q, kappa and lamda. Electron microscopy showed plasma cells staining IgG4 and 80% interstitial fibrosis.

Discussion

IgG4-RD is a fibroinflammatory condition characterized by lymphoplasmacytic infiltrates rich in IgG4 and plasma cells with storiform fibrosis and elevated serum IgG4. Our patient presented with interstitial nephritis, pancreatitis, lymphadenopathy, thyroiditis, lower extremity lymphedema and anemia with elevated serum IgG4 levels and histopathology findings of IgG4-RD. The diagnosis was made based on the Japanese comprehensive diagnostic criteria for IgG4-RD. He responded to rituximab and steroids with decreased transfusion requirements. Severe anemia has not been reported with IgG4-RD. In our case the transfusion requirement decreased after initiation of therapy which suggests the anemia may have been secondary to anti-EPO IgG4 or poor response to EPO in the setting of an underlying inflammatory process.