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Cryoglobulins: putative effectors of adaptive immune response


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11

 

  1. Area Diagnostica di Laboratorio, Fondazione Policlinico Universitario 'A. Gemelli', I.R.C.C.S, Rome, Italy.
  2. Dipartimento di Medicina e Chirurgia Traslazionale, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario “A. Gemelli”, I.R.C.C.S, Rome, Italy.
  3. Dipartimento di Medicina e Chirurgia Traslazionale, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario “A. Gemelli”, I.R.C.C.S, Rome, Italy. mariapaola.marino@unicatt.it
  4. Laboratorio di Patologia Clinica, Ospedale Madre Giuseppina Vannini, Rome, Italy. dottfgulli@gmail.com
  5. Department of Translational and Precision Medicine, Sapienza University of Rome, Italy.
  6. Department of Translational and Precision Medicine, Sapienza University of Rome, Italy.
  7. Dipartimento di Medicina e Chirurgia Traslazionale, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario “A. Gemelli”, I.R.C.C.S, Rome, Italy.
  8. Dipartimento di Medicina di Laboratorio, Università di Tor Vergata, Rome, Italy.
  9. Dipartimento di Medicina e Chirurgia Traslazionale, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario “A. Gemelli”, I.R.C.C.S, Rome, Italy.
  10. Dipartimento di Medicina e Chirurgia Traslazionale, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario “A. Gemelli”, I.R.C.C.S, Rome, Italy.
  11. Department of Translational and Precision Medicine, Sapienza University of Rome, Italy.

CER13372
2021 Vol.39, N°2 ,Suppl.129
PI 0171, PF 0179
Review

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PMID: 33124568 [PubMed]

Received: 27/03/2020
Accepted : 17/07/2020
In Press: 29/10/2020
Published: 19/05/2021

Abstract

Cryoglobulinaemia consists of circulating monoclonal and/or polyclonal immunoglobulins with rheumatoid factor (RF) activity that precipitate at temperatures <37°C. Cryoglobulinaemic syndrome, characterised by clinical signs of systemic vasculitis, is associated with chronic infection of hepatitis C virus (HCV) and might evolve in B-cell malignancies. In about one third of all HCV infection cases, serum autoantibodies are commonly found. This is probably due directly to the transformation of infected B cells but, also, indirectly, to the viral chronic stimulation of a pool of autoreactive B cells. The pattern of IgG subclasses seems to contribute to the worsening progression of HCV infection into lymphoproliferative and/or autoimmune diseases. Many evidences showed that B cells circulating in patients with HCV-associated mixed cryoglobulinaemia (MC) are profoundly abnormal; moreover, in most of cases, normal B cells are replaced by expanded clonal B cells characterized by the low expression of CD21. After viral eradication, these cells persist in circulation and their occurrence does not correlate with serum cryoglobulins nor with vasculitis response or relapse. It is probably due to the persistence of monoclonal B cells producing RF, that in course of MC can be reactivated by circulating immune complexes, highly produced during infections or tumours. Here, we aimed to review current literature focusing the pathogenesis of MC referring to specificity and immunochemical characteristics of the immunoglobulins involved in cryoprecipitation.

Rheumatology Article