Abstract: SA-PO243
Cry, Cry Again: A Case of Idiopathic Cryoglobulinemia
Session Information
- Trainee Case Reports - V
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Kikani, Nupur, Baylor College of Medicine, Houston, Texas, United States
- Ali, Sehrish, BCM Nephrology, Sugar Land, Texas, United States
- Raghavan, Rajeev, Baylor College of Medicine, Houston, Texas, United States
Introduction
Cryoglobulinemia is characterized by the presence of cryoglobulins in the serum, and it is commonly associated with persistent viral infections, autoimmune diseases, and lymphoproliferative disorders. Although there are standardized treatments for viral or malignancy associated cryoglobulinemia, a protocol for idiopathic non-viral cryoglobulinemia has yet to be described. Literature supports using a combination of high dose glucocorticoid therapy and plasmapheresis. In true idiopathic cryoglobulinemia, Rituximab has improved outcomes in published cases. Here, we present a patient with newly diagnosed idiopathic cryoglobulinemia in whom Rituximab was added to high dose steroids resulting in improvement of renal function
Case Description
A 66-year old male with a history of leukocytoclastic vasculitis diagnosed by biopsy of a right leg rash 7 years prior, presented with 2.5 weeks of new onset lower extremity edema and scrotal swelling. He had new onset hypertension, acute kidney injury, hyperkalemia, and non-anion gap metabolic acidosis. A 24-hour urine collection was consistent with nephrotic range proteinuria. A laboratory work-up showed an undetectable C4, low-normal C3, positive rheumatoid factor, and positive ANA with titer >640. Kidney biopsy revealed immune deposits consistent with cryoglobulinemia and immunofluorescence was negative for other pathology (Figure 1). The remaining viral and autoimmune laboratory work up was negative, including both Hepatitis panel and HCV RNA. The patient received three days of pulse dose steroids followed by a single dose of rituximab at 375 mg/m2. His creatinine, proteinuria, and overall clinical picture improved and has persisted.
Discussion
Guidelines to treat idiopathic non-viral cryoglobulinemia do not exist. Rituximab may be an effective and intuitive adjunct treatment, per literature review and in our patient.