SLEeping in the Cold
- Glomerular Diseases: From Inflammation to Fibrosis - III
November 04, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1401 Glomerular Diseases: From Inflammation to Fibrosis
- Bonner, Ryan, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
- Jain, Koyal, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States
Glomerular diseases in patients with systemic lupus erythematosus (SLE) can take many forms, however the classification of lupus nephritis by ISN/RPS does not include cryoglobulinemic vasculitis. We describe a patient evaluated for lupus nephritis who was found to have SLE-induced mixed cryoglobulinemic vasculitis.
A 68-year-old woman with SLE and no prior kidney involvement was referred to the nephrology clinic for suspected lupus nephritis. Two months prior to evaluation, she developed edema and exercise intolerance, with workup notable for new proteinuria with urine protein-to-creatinine ratio (UPCR) of 3.8 g/g, mildly elevated serum creatinine (0.75 mg/dL) from baseline (0.60 mg/dL), low C4, and normal C3. There was no evidence of SLE or vasculitis on history or physical exam. Urine sediment microscopy was notable for many acanthocytes.
Kidney biopsy was consistent with membranoproliferative glomerulonephritis and noted cryoglobulin deposits on electron microscopy. Further workup was notable for the presence of type II cryoglobulins (2%), elevated rheumatoid factor (140 IU/mL), negative hepatitis C virus antibodies, and normal serum free light chains. She was diagnosed with cryoglobulinemic vasculitis. Treatment with oral prednisone and mycophenolate mofetil resulted in improved proteinuria (UPCR 0.375 g/g) and decline in rheumatoid factor titers (59 IU/mL), however during her steroid taper the patient developed a purpuric leukocytoclastic rash, prompting the addition of rituximab. Her symptoms have markedly improved since receiving rituximab.
While the most common glomerular disease affecting patients with lupus is lupus nephritis, many glomerular diseases are associated with SLE, including lupus podocytopathy, thrombotic microangiopathy, antiphospholipid antibody syndrome, and cryoglobulinemic vasculitis. Treatment of cryoglobulinemic vasculitis due to SLE is initially focused on treating the SLE itself, however with progressive or persistent disease, rituximab may be added.