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Abstract: FR-PO201

Dual Autoimmune Processes, One IgG4 Stain

Session Information

Category: Acute Kidney Injury

  • 103 AKI: Mechanisms


  • Schretlen, Claire Frances, Johns Hopkins Medicine, Baltimore, Maryland, United States
  • Chow, Timothy M., Johns Hopkins Medicine, Baltimore, Maryland, United States
  • Malvica, Silvia, Johns Hopkins Medicine, Baltimore, Maryland, United States
  • Abifaraj, Farah, Johns Hopkins Medicine, Baltimore, Maryland, United States

IgG4-related disease (IgG4-RD) is an idiopathic immune-mediated fibroinflammatory disease often targeting the pancreas, kidney, retroperitoneum, and salivary glands. Pathology shows lymphoplasmacytic infiltrate with IgG4 plasma cells, storiform fibrosis, and obliterative phlebitis. We present an atypical presentation of a disease still being elucidated.

Case Description

A 30-year-old female with cirrhosis due to autoimmune hepatitis with primary sclerosing cholangitis overlap (AIH-PSC) and CKD stage III due to previous acute kidney injury (AKI) presented with new AKI (creatinine 3.3 mg/dl) during liver transplant evaluation. Serologic studies (table 1) showed elevated IgG with IgG4 predominance. Radiologic findings included retroperitoneal lymphadenopathy and groundglass pulmonary nodules. Although liver biopsy had not shown IgG4 plasma cell predominance, kidney biopsy revealed global glomerulosclerosis, interstitial fibrosis without storiform pattern, IgG4/IgG ratio of at least 30%, and >10 IgG4-plasma cells per high powered field (fig 1). She was treated for IgG4-RD with steroids and rituximab. Kidney function and pulmonary imaging improved dramatically within 6 months.


This is a rare case of IgG4-RD in a young female with AIH-PSC without all characteristic pathologic findings. Diagnosis should be based on clinical, serological, radiological, and pathologic evidence. Underlying autoimmune disease may correlate with development of IgG4-RD.

Serologic TestsResultsNormal range
Anti-neutrophil antibody (ANA) titer>1:640negative
Perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) titer>1:640negative
Myeloperoxidase (MPO) IgG69 units<20.0 units
Proteinase-3 (PR3) IgG139.6 units<20.0 units
Anti-double stranded DNA (Anti-dsDNA) titer1:80negative
Total IgG6249 mg/dl610-1616 mg/dL
IgG4436.9 mg/dl3.9-86.4 mg/dL