Chilling Complications: A Case of Cryoglobulinemic Vasculitis in a Patient With Newly Diagnosed Sjögren's Syndrome.

In rare circumstances, Sjögren's syndrome can cause systemic illness in the form of cryoglobulinemic vasculitis (CV). Diagnostic workup is of the utmost importance to prevent severe end-organ damage and even death, especially in the case of rapidly progressive disease. In this case, a 47-year-old male presented to the hospital with shortness of breath, unilateral leg swelling, and a lower extremity wound worsening over a three-month period. During this admission, the patient was found to have diffuse lymphadenopathy with a negative workup for malignancy. The patient met the criteria for Sjögren's syndrome and was initiated on outpatient disease-modifying antirheumatic drug (DMARD) therapy. The patient was readmitted to the hospital in undifferentiated shock and was found to have CV, with significant end-organ damage. He was treated with pulse-dose steroids, plasmapheresis, and rituximab infusions. After a lengthy three-month admission, he was finally discharged in stable condition. CV can present very similarly to antiphospholipid syndrome and antineutrophil cytoplasmic antibody (ANCA) vasculitis. Biopsies prove invaluable in the diagnosis. This manifestation of Sjögren's syndrome is uncommon, and it is often difficult to assess which patients may be more at risk for the complication. This case illustrates the importance of early identification of patients with Sjögren's syndrome who may be at risk for the development of vasculitis.