Abstract: FR-PO201
Dual Autoimmune Processes, One IgG4 Stain
Session Information
- AKI: Mechanisms - Case Reports
November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 103 AKI: Mechanisms
Authors
- 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
Introduction
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.
Discussion
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 Tests | Results | Normal range |
| Anti-neutrophil antibody (ANA) titer | >1:640 | negative |
| Perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) titer | >1:640 | negative |
| Myeloperoxidase (MPO) IgG | 69 units | <20.0 units |
| Proteinase-3 (PR3) IgG | 139.6 units | <20.0 units |
| Anti-double stranded DNA (Anti-dsDNA) titer | 1:80 | negative |
| Total IgG | 6249 mg/dl | 610-1616 mg/dL |
| IgG4 | 436.9 mg/dl | 3.9-86.4 mg/dL |