Cryoglobulinemic vasculitis in a patient with CREST syndrome.
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.
Department of Neurology, Mayo Clinic, 4500 San Pablo Road South, Jacksonville, FL 32224, USA. Electronic address: hurst.rebecca@mayo.edu.
Rebecca L
RL
Hurst
Department of Rheumatology, Mayo Clinic, Jacksonville, FL, USA.
Florentina
F
Berianu
Department of Rheumatology, Mayo Clinic, Jacksonville, FL, USA.
William W
WW
Ginsburg
Department of Neurology, Mayo Clinic, Rochester, MN, USA; Peripheral Nerve Center, Mayo Clinic, Rochester, MN, USA.
Christopher J
CJ
Klein
Peripheral Nerve Center, Mayo Clinic, Rochester, MN, USA.
Janean K
JK
Englestad
Department of Neurology, Mayo Clinic, 4500 San Pablo Road South, Jacksonville, FL 32224, USA.
Kathleen D
KD
Kennelly
D017675:CREST Syndrome / Q000150:complications* / Q000473:pathology; D003450:Cryoglobulins / Q000378:metabolism*; D005260:Female; D006801:Humans; D008875:Middle Aged; D013497:Sural Nerve / Q000473:pathology; D014657:Vasculitis / Q000150:complications* / Q000188:drug therapy / Q000378:metabolism* / Q000473:pathology
D003450:Cryoglobulins
CREST; Cryoglobulinemia; Cryoglobulins; Peripheral neuropathy; Sural nerve biopsy; Systemic sclerosis; Vasculitis