Cryoglobulinemic vasculitis with renal involvement and Sjögren's syndrome

Authors

  • María Emilia Debernardi Dermatology Division, General Acute Hospital Dr. José María Ramos Mejía, Autonomous City of Buenos Aires, Argentina
  • Candela Preti Dermatology Division, General Acute Hospital Dr. José María Ramos Mejía, Autonomous City of Buenos Aires, Argentina
  • Cindy Paula Frare General Acute Hospital Dr. José María Ramos Mejía, Autonomous City of Buenos Aires, Argentina
  • Gabriela Bendjuia Dermatology Division, General Acute Hospital Dr. José María Ramos Mejía, Autonomous City of Buenos Aires, Argentina
  • María C. Corbella Dermatology Division, General Acute Hospital Dr. José María Ramos Mejía, Autonomous City of Buenos Aires, Argentina

DOI:

https://doi.org/10.47196/da.v26i1.2069

Keywords:

Sjögren’s syndrome, Leukocyteclastic vasculitis, Cryoglobulinemic vasculitis, Cryoglobulinemia, Purpura of lower limbs, Glomerulonephritis

Abstract

Cryoglobulinemic vasculitis is a small-sized vasculitis that occurs in the context of serum cryoglobulins. Mixed cryoglobulinemias are the most frequent and are associated with chronic infections, typically hepatitis C and autoimmune diseases, most commonly Sjögren's syndrome. Clinical manifestations include purpura of lower limbs, peripheral neuropathy, arthralgias and glomerulonephritis. The presence of cryoglobulinemic vasculitis in the context of Sjögren's syndrome is a marker of poor prognosis. We present the case of a patient with cryoglobulinemic vasculitis associated to cutaneous and renal involvement that led us to the diagnosis of primary Sjögren's syndrome.

Author Biographies

María Emilia Debernardi, Dermatology Division, General Acute Hospital Dr. José María Ramos Mejía, Autonomous City of Buenos Aires, Argentina

Doctor of the Career of Specialist in Dermatology, University of Buenos Aires. Internal Medicine specialist. Dermatology Division, Ramos Mejía Hospital

Candela Preti, Dermatology Division, General Acute Hospital Dr. José María Ramos Mejía, Autonomous City of Buenos Aires, Argentina

Medical Specialist in Dermatology. Dermatology Division, Ramos Mejía Hospital

Cindy Paula Frare, General Acute Hospital Dr. José María Ramos Mejía, Autonomous City of Buenos Aires, Argentina

Medical Specialist in Dermatology. Dermatology Division, Ramos Mejía Hospital

Gabriela Bendjuia, Dermatology Division, General Acute Hospital Dr. José María Ramos Mejía, Autonomous City of Buenos Aires, Argentina

Specialist in Dermatology and Internal Medicine, Plant Doctor, Collagenopathies Sector. Dermatology Division, Ramos Mejía Hospital

María C. Corbella, Dermatology Division, General Acute Hospital Dr. José María Ramos Mejía, Autonomous City of Buenos Aires, Argentina

Medical Specialist in Pathological Anatomy. Dermatology Division, Ramos Mejía Hospital

References

I. Ramos-Casals M, Stone JH, Cid MC, Bosch X. The cryoglobulinaemias. Lancet 2012;379(9813):348‐360.

II. Brouet JC, Clauvel JP, Danon F, Klein M, et ál. Biologic and clinical significance of cryoglobulins. A report of 86 cases. Am J Med1974;57(5):775‐788.

III. Cacoub P, Comarmond C, Domont F, Savey L, et ál. Cryoglobulinemia vasculitis. Am J Med 2015;128(9):950‐955.

IV. Terrier B, Cacoub P. Cryoglobulinemia vasculitis: an update. Curr Opin Rheumatol 2013;25(1):10‐18.

V. Ferri C, Sebastiani M, Giuggioli D, Cazzato M, et ál. Mixed cryoglobulinemia: demographic, clinical, and serologic features and survival in 231 patients. Semin Arthritis Rheum 2004;33(6):355‐374.

VI. Baldini C, Pepe P, Quartuccio L, Priori R, et ál. Primary Sjogren's syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients. Rheumatology (Oxford) 2014;53(5):839‐844.

VII. Singh AG, Singh S, Matteson EL. Rate, risk factors and causes of mortality in patients with Sjögren's syndrome: a systematic review and meta-analysis of cohort studies. Rheumatology (Oxford) 2016;55(3):450‐460.

VIII. Retamozo S, Gheitasi L, Quartuccio L, Corazza L, et ál. Cryoglobulinaemic vasculitis at diagnosis predicts mortality in primary Sj€ogren syndrome: analysis of 515 patients. Rheumatology 2016;55(8):1443-1451.

IX. Terrier B, Cacoub P. Cryoglobulinemia vasculitis: an update. Curr Opin Rheumato. 2013;25(1):10‐18.

X. Silva F, Pinto C, Barbosa A, Borges T, et ál. New insights in cryoglobulinemic vasculitis. J Autoimmun2019;105:102313.

Published

2020-03-15

Issue

Section

Clinical Cases