Mixed cryoglobulins (MCs) are proteins that precipitate from cooled serum, and are composed of a polyclonal immunoglobulin G (IgG) bound to another immunoglobulin that acts as an anti-IgG rheumatoid factor (RF). In type II mixed cryoglobulinemia, the antiglobulin component, usually of the IgM class, is monoclonal; it is polyclonal in type III mixed cryoglobulinemia. The majority of MCs are found in patients with connective tissue diseases, infectious or lymphoproliferative disorders, hepatobiliary diseases, or immunologically mediated glomerular diseases (secondary MCs). The etiology is not clear for 30% of all MCs, and this type of cryoglobulinemia is called "essential." There is a common clinical syndrome in types II and III essential mixed cryoglobulinemia (EMC) characterized by purpura, weakness, and arthralgia. In type II EMC only, in which an IgMk is the monoclonal RF, a membranoproliferative glomerulonephritis (MPGN) occurs with some peculiar morphologic and clinical features; this is termed "cryoglobulinemic GN." Glomerulonephritis can be differentiated from idiopathic MPGN, especially in the acute stage, which is characterized by an acute nephritic syndrome, by the following findings: (1) the presence of large deposits filling the capillary lumen that sometimes are shown to have a characteristic fibrillar or crystalloid structure by electron microscopy; (2) the extent of the exudative component consequent to the frequently massive infiltration of monocytes; (3) a more diffuse and evident thickening of the glomerular basement membrane, which has a double-contoured appearance that is mainly due to the peripheral interposition of monocytes, with less evident mesangial expansion; and (4) possibly some vasculitis in small and medium-sized renal arteries without concomitant features of segmental necrotizing GN or crescentic GN.(ABSTRACT TRUNCATED AT 250 WORDS)
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http://dx.doi.org/10.1016/0272-6386(95)90095-0 | DOI Listing |
Cureus
December 2024
Nephrology, Ibn Sina Hospital, Rabat, Rabat, MAR.
Cryoglobulinemic vasculitis is a rare small-vessel vasculitis leading to multi-organ dysfunction, often associated with chronic infections like hepatitis C virus (HCV), and autoimmune disorders. Most cases involve mixed monoclonal or polyclonal immunoglobulins, presenting symptoms such as purpura, arthralgias, and weakness. Severe organ involvement, particularly cardiac, is rare but potentially life-threatening.
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January 2025
Department of Nephrology and Dialysis, Tokyo Metropolitan Institute for Geriatrics and Gerontology, 35-2 Sakae-Cho, Itabashi, Tokyo, 173-0015, Japan.
Expert Rev Anti Infect Ther
January 2025
Department of Biomedical Sciences, Humanitas University, Milan, Italy.
Introduction: Hepatitis C virus (HCV) infection represents a significant global health burden, particularly due to its extrahepatic immune-mediated manifestations, such as mixed cryoglobulinemia, associated vasculitis (CryoVas), and non-Hodgkin B-cell lymphoma (B-NHL), which pose significant challenges. The advent of direct-acting antiviral (DAA) has changed the therapeutic landscape for HCV-related complications.
Areas Covered: This review explores the evolving epidemiology and management of HCV extrahepatic manifestation and lymphoproliferative disorders in the era of DAAs.
Hepatol Int
December 2024
Division of Hepatology, Department of Gastroenterology and Hepatology, Chang Gung Memorial Hospital at Linkou, No 5, Fu Hsing Street, Kuei Shan, Taoyuan, Taiwan.
Background: The phenotype of cryoglobulinemia in hepatitis B virus (HBV) carriers remains elusive.
Methods: A 7-year prospective cohort of 648 hepatitis B e antigen (HBeAg)-negative Taiwanese HBV carriers [males: 344 (53%)] was conducted.
Results: Among 648, 189 (29.
Front Immunol
December 2024
Department of Internal Diseases, Nephrology and Dialysis, Military Institute of Medicine-National Research Institute, Warsaw, Poland.
Cryoglobulinemia is a rare disease characterized by the presence of cryoglobulins in the blood serum. It is usually caused by autoimmune, lymphoproliferative, or infectious factors. The pathogenesis of cryoglobulinemia is not well understood, therefore, genetic testing is very important.
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