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Orphanet Journal of Rare Diseases

, 3:25

First Online: 16 September 2008Received: 26 February 2008Accepted: 16 September 2008DOI: 10.1186-1750-1172-3-25

Cite this article as: Ferri, C. Orphanet J Rare Dis 2008 3: 25. doi:10.1186-1750-1172-3-25

Abstract

Mixed cryoglobulinemia MC, type II and type III, refers to the presence of circulating cryoprecipitable immune complexes in the serum and manifests clinically by a classical triad of purpura, weakness and arthralgias. It is considered to be a rare disorder, but its true prevalence remains unknown. The disease is more common in Southern Europe than in Northern Europe or Northern America. The prevalence of -essential- MC is reported as approximately 1:100,000 with a female-to-male ratio 3:1, but this term is now used to refer to a minority of MC patients only. MC is characterized by variable organ involvement including skin lesions orthostatic purpura, ulcers, chronic hepatitis, membranoproliferative glomerulonephritis, peripheral neuropathy, diffuse vasculitis, and, less frequently, interstitial lung involvement and endocrine disorders. Some patients may develop lymphatic and hepatic malignancies, usually as a late complication. MC may be associated with numerous infectious or immunological diseases. When isolated, MC may represent a distinct disease, the so-called -essential- MC. The etiopathogenesis of MC is not completely understood. Hepatitis C virus HCV infection is suggested to play a causative role, with the contribution of genetic and-or environmental factors. Moreover, MC may be associated with other infectious agents or immunological disorders, such as human immunodeficiency virus HIV infection or primary Sjögren-s syndrome. Diagnosis is based on clinical and laboratory findings. Circulating mixed cryoglobulins, low C4 levels and orthostatic skin purpura are the hallmarks of the disease. Leukocytoclastic vasculitis involving medium- and, more often, small-sized blood vessels is the typical pathological finding, easily detectable by means of skin biopsy of recent vasculitic lesions. Differential diagnoses include a wide range of systemic, infectious and neoplastic disorders, mainly autoimmune hepatitis, Sjögren-s syndrome, polyarthritis, and B-cell lymphomas. The first-line treatment of MC should focus on eradication of HCV by combined interferon-ribavirin treatment. Pathogenetic treatments immunosuppressors, corticosteroids, and-or plasmapheresis should be tailored to each patient according to the progression and severity of the clinical manifestations. Long-term monitoring is recommended in all MC patients to assure timely diagnosis and treatment of the life-threatening complications. The overall prognosis is poorer in patients with renal disease, liver failure, lymphoproliferative disease and malignancies.

AbbreviationsIgimmunoglobulin

RFrheumatoid factor

MCmixed cryoglobulinemia

HCVhepatitis C virus

MPGNmembranoproliferative glomerulonephritis

B-CLLB-cell chronic lymphocytic leukemia

Icimmunocytoma

MLDUSmonotypic lymphoproliferative disorder of undetermined significance

HBVhepatitis B virus

PCRpolymerase chain reaction

VLDLvery low-density lipoprotein

MALTmucosa-associated lymphoid tissue

HIVhuman immunodeficiency virus

RArheumatoid arthritis

pSSprimary Sjögren-s syndrome

LAC-dietlow-antigen-content diet.

Electronic supplementary materialThe online version of this article doi:10.1186-1750-1172-3-25 contains supplementary material, which is available to authorized users.

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Autor: Clodoveo Ferri

Fuente: https://link.springer.com/







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