1. Mixed cryoglobulinaemia: a cross-road between autoimmune and lymphoproliferative disorders
- Author
-
L. La Civita, Giampiero Pasero, Al Zignego, Giovanni Longombardo, and Clodoveo Ferri
- Subjects
030203 arthritis & rheumatology ,business.industry ,Hepatitis C virus ,Autoantibody ,Lymphoproliferative disorders ,Glomerulonephritis ,030204 cardiovascular system & hematology ,medicine.disease_cause ,medicine.disease ,Hepatitis C ,Lymphoproliferative Disorders ,Autoimmune Diseases ,Lymphoma ,03 medical and health sciences ,0302 clinical medicine ,Cryoglobulinemia ,Rheumatology ,Rheumatoid arthritis ,Immunology ,medicine ,Mixed cryoglobulinaemia ,autoimmunity ,lymphoproliferation ,Humans ,business ,Vasculitis ,Systemic vasculitis - Abstract
Mixed cryoglobulinaemia (MC) is a systemic vasculitis, secondary to the deposition in small and medium-sized blood vessels of circulating immune complexes, mainly the cryoglobulins, and complement. MC is characterised by a typical clinical triad (purpura, weakness, arthralgias) and by one or more organ involvement: chronic hepatitis, glomerulonephritis, peripheral neuropathy, skin ulcers and diffuse vasculitis. In a limited number of MC patients, a malignancy, that is B-cell non-Hodgkin's lymphoma or hepatocellular carcinoma, may also develop. Hepatitis C virus (HCV) infection has been found in the majority of patients with MC; the frequency of HCV markers (91%) was significantly higher than other rheumatic diseases (6.4%), namely systemic lupus, Sjùgren's syndrome, rheumatoid arthritis and systemic sclerosis, or healthy controls (1.2%). The HCV infection of lymphoid tissues may represent the remote event leading to B-lymphocyte proliferation responsible for autoantibodies and immune-complex production. In a similar way, HCV infection may also be involved in the pathogenesis of other autoimmune (glomerulonephritis, thyroiditis, lung fibrosis, autoimmune hepatitis, porphyria cutanea tarda) and lymphoproliferative disorders (monoclonal gammopathies, B-cell lymphomas). MC shares numerous clinico-serological and pathological features with the above disorders. HCV seems to be their common etiological agent; however, a variable combination of unknown co-factors (infectious, genetic, environmental) should be determinant for the appearance of different clinical patterns.
- Published
- 1998