Abstract: TH-PO217

A Unique Case of Sjogren’s Syndrome-Associated Cryoglobulinemic Glomerulonephritis

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Long, Brad, University of Utah Hospital, Salt lake, Utah, United States
  • Morton, Ryan, University of Utah Hospital, Salt lake, Utah, United States
  • Marji, Catreena, University of Utah Hospital, Salt lake, Utah, United States
  • Cho, Monique E., University of Utah Hospital, Salt lake, Utah, United States
  • Al-Rabadi, Laith, University of Utah Hospital, Salt lake, Utah, United States
Background

Cryoglobulinemic Glomerulonephritis is rare and not well described in the absence of Hepatitis C virus. Sjogren’s syndrome continues to be the second most common cause of mixed cryoglobulinemia after hepatitis C virus (HCV). We present a unique case of Sjogren’s disease and cryoglobulinemic glomerulonephritis

Methods

Patient is a 65 year old male patient with medical history relevant for Sjogren’s disease and hepatitis C, in remission after treatment with interferon 10 years ago, presented to the outside hospital with a 5-day history of fatigue, headache, facial swelling and lower extremity rash. His physical exam was relevant for petechial rash involving his low extremities.

Initial investigation revealed well preserved renal function with creatinine of 1 mg/dL. Urine protein was 1346 mg per gram of creatinine. Urine microscopy showed many non-dysmorphic RBCs with no cellular casts. Further workup showed ANA 1:2560, with positive SSA/SSB. The antibodies for GBM, dsDNA , RNP , ASO , Smith Ab, and ANCA were all negative. Complement 3 and 4 levels were 44 mg/dL and undetectable, respectively. HCV PCR was undetectable. Cryoglobulin from outside hospital was negative. Rheumatoid factor was elevated at 411 IU/mL. Immunofixation showed a faint band in the IgM kappa region.

Light microscopy showed 15 glomeruli, many with lobular membranoproliferative pattern, mesangial and endocapillary hypercellularity. There was also evidence of focal intra-capillary eosinophilic material and hyaline pseudothrombi. Immunofluorescence showed granular mesangial and capillary wall staining for the following: 3+ IgM, 1-2+ IgG (segmental), 2+ C3, trace C1q, 2+ Kappa, and trace lambda. Electron microscopy revealed segmental immune-type subendothelial and mesangial electron dense deposits with no definite substructural organization. Repeat Cryoglobulin done at our hospital was positive. Patient was started on prednisone 60 mg and Azathioprine 100 mg daily. He had complete resolution of proteinuria and hematuria in one month

Conclusion

Elevated rheumatoid factor, and very low complement 4 level were the key for suspecting cryoglobulinemic glomerulonephritis despite the absence of impaired GFR. Cryoglobulinemia in this case is likely related to Sjogren’s syndrome, not hepatitis C, given the undetected Hepatitis C PCR. Early treatment is crucial in preserving renal function