Cryoglobulinemic vasculitis in a patient with CREST syndrome.

Abstract

Cryoglobulinemic vasculitis is a rare entity. Although it has been reported in diffuse systemic sclerosis, it has not been reported in calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia (CREST) syndrome. We report a patient with cryoglobulinemic vasculitis with CREST syndrome who did not have typical clinical features of vasculitis. This 58-year-old woman presented with mild generalized weakness and a diagnosis of CREST syndrome, which included Raynaud's syndrome, dysphagia and telangiectasias. She was positive for serum cryoglobulins, which led to a sural nerve biopsy. The biopsy results were consistent with cryoglobulinemic vasculitis. Cryoglobulinemic vasculitis has not been previously reported in CREST syndrome to our knowledge. Additionally, the patient also had limited clinical symptoms. Our patient displays the importance of checking for cryoglobulins and obtaining a nerve biopsy when the serum is positive. Both of these diagnostic tests were integral for directing appropriate treatment for this patient.

• Authors: Rebecca L Hurst, Florentina Berianu, William W Ginsburg, Christopher J Klein, Janean K Englestad, Kathleen D Kennelly
• Journal: Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia (J Clin Neurosci) Vol. 21 Issue 10 Pg. 1821-3 (Oct 2014) ISSN: 1532-2653 [Electronic] Scotland
• PMID: 24852904 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2014 Elsevier Ltd. All rights reserved.

Chemical References

• Cryoglobulins

Topics

• CREST Syndrome (complications, pathology)
• Cryoglobulins (metabolism)
• Female
• Humans
• Middle Aged
• Sural Nerve (pathology)
• Vasculitis (complications, drug therapy, metabolism, pathology)