Abstract: TH-PO209
Crescentic C3 Glomerulonephritis in HIV Disease
Session Information
- Fellows/Residents Case Reports: Glomerulonephritis
November 02, 2017 | Location: Hall H, Morial Convention Center
Abstract Time: 10:00 AM - 10:00 AM
Category: Nephrology Education
- 1302 Fellows and Residents Case Reports
Authors
- Jan, Muhammad Yahya, Indiana University School of Medicine, Indianapolis, Indiana, United States
- Modi, Jwalant R., Indiana University School of Medicine, Indianapolis, Indiana, United States
- Phillips, Carrie L., Indiana University School of Medicine, Indianapolis, Indiana, United States
- El-Achkar, Tarek M., Indiana University School of Medicine, Indianapolis, Indiana, United States
- Eadon, Michael T., Indiana University School of Medicine, Indianapolis, Indiana, United States
Background
Crescentic C3 Glomerulonephritis (C3GN), a subtype of C3 glomerulopathy, presents a diagnostic and therapeutic challenge, especially in the setting of HIV infection.
Methods
A 24-year-old African-American male with no past medical history presented with leg swelling, upper respiratory tract symptoms and hematuria of 2 weeks duration. Physical exam showed signs of volume overload and uncontrolled hypertension.
Lab showed serum creatinine of 3.01 mg/dl and urine with dysmorphic RBCs and spot proteinuria of 2.4 g/g. He was found to be HIV+ with viral load of 64,000 copies and CD4 count of 355 cells/μl. C3 and C4 levels were within normal limits.
Clinical course showed worsening kidney injury and proteinuria. Anti-GBM IgG, ANA, ASO titer, hepatitis B and C, ANCA titers, cryoglobulins were all negative. A Kidney biopsy revealed crescentic glomerulonephritis with diffuse mesangial and capillary hypercellularity with patchy basement membrane duplication. Immunofluorescence staining revealed 4+ C3 and 1+ IgG. Work-up for other infections was negative. Alternate complement pathway analysis showed borderline low level of Factor H, normal C3 nephritic factor and low levels of Factor B and I confirming the diagnosis of C3GN. He was initiated on HIV treatment, pulse steroids, and Cellcept®. This lead to stabilization and subsequent improvement of renal function.
Conclusion
Diagnosis of glomerulonephritis with predominant C3 deposits in the setting of HIV disease is very challenging. The differential includes C3GN, infection related GN, and HIV immune complex disease (HIVICK). The overlap between these entities and the relevance to disease pathogenesis and management is still unclear. Correlation between isolated strong C3 staining on biopsy and normal C3 complement levels may be more suggestive of C3 glomerulopathy. Testing for abnormalities in the complement pathway is crucial to establish a firm diagnosis. The low levels of Factors B and I in this case suggest inhibited C3 activation, which could be related to HIV disease itself.
We report a case of crescentic C3GN with altered factors B and I in a patient with HIV disease. Further investigation is required to determine whether HIV may dysregulate complement and precipitate C3 glomerulopathy.