In a retrospective study of 242 cases of non-infectious cryoglobulinemia-associated vasculitis, corticosteroids and rituximab were efficacious in the treatment of the clinical, kidney and immunologic sequelae of cryoglobulinemia.6
After treatment with a single dose of rituximab and continued maintenance therapy with hydroxychloroquine, azathioprine, prednisone and lisinopril, her kidney function remains normal and her proteinuria decreased to less than 500 mg/day. Complements remained low despite treatment. Her blood pressure is well controlled, and she has had no further LCV.
Unfortunately, subsequent rituximab infusions have been delayed due to insurance coverage, and she coincidentally developed new papillary thyroid cancer, resulting in total thyroidectomy, and cervical intraepithelial neoplasia II/III for which a hysterectomy is planned. After she recovers from her hysterectomy, she will receive further rituximab infusions.
Lindsey MacFarlane, MD, is a second-year rheumatology fellow at Brigham and Women’s Hospital. She is currently pursuing her MPH and has a research interest in osteoarthritis.
Paul Hoover, MD, PhD, is a second-year rheumatology fellow at Brigham and Women’s Hospital, starting his post-doctoral work at the Broad Institute investigating gene regulation in autoimmunity. He graduated from the Medical Scientist Training Program from Stanford University and completed his internal medicine residency at Brigham and Women’s Hospital.
Acknowledgments
The authors would like to thank Helmut Rennke, MD, for supplying the light and electron microscopy images from the kidney biopsy and thoughtful conversations, and Elena Massarotti, MD, for proofreading the manuscript.
References
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- Hervier B, Hamidou M, Haroche J, et al. Systemic lupus erythematosus associated with ANCA-associated vasculitis: An overlapping syndrome? Rheumatol Int. 2012 Oct;32(10):3285–3290.
- Jones RB, Tervaert JW, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med. 2010 Jul 15;363(3):211–220.
- Hochberg MC, Silman AJ, Smolen JS, et al. (July 2014) Rheumatology, Sixth edition. St. Louis: Mosby.
- Garcia-Carrasco M, Ramos-Casals M, Cervera R, et al. Cryoglobulinemia in systemic lupus erythematosus: Prevalence and clinical characteristics in a series of 122 patients. Semin Arthritis Rheum. 2001 Apr;30(5):366–373.
- Terrier B, Krastinova E, Marie I, et al. Management of noninfectious mixed cryoglobulinemia vasculitis: Data from 242 cases included in the CryoVas survey. Blood. 2012 Jun 21;119(25):5996–6004.