Cryoglobulinemic vasculitis and glomerulonephritis

Abstract

Objective:Cryoglobulinemia often causes systemic vasculitis, thereby damaging to skin and internal organs including kidneys, even life-threatening. This review aimed to introduce the advances in understanding, detection, and treatment of this disease in recent years, with a particular concern to clinical practice.Data sources:All the data in this review were from the English or Chinese literature in the PubMed and China National Knowledge Infrastructure databases as of March 2019.Study selection:This review selected important original articles, meaningful reviews, and some reports on cryoglobulinemia published in recent years and in history, as well as the guidelines for treatment of underlying diseases which lead to cryoglobulinemia.Results:Diagnosis of cryoglobulinemia relies on serum cryoglobulin test, in which to ensure that the blood sample temperature is not less than 37°C in the entire pre-analysis phase is the key to avoid false negative results. Cryoglobulinemic vasculitis (Cryo Vas), including cryoglobulinemic glomerulonephritis (Cryo GN), usually occurs in types II and III mixed cryoglobulinemia, and can also be seen in type I cryoglobulinemia caused by monoclonal IgG3 or IgG1. Skin purpura, positive serum rheumatoid factor, and decreased serum levels of C4 and C3 are important clues for prompting types II and III Cryo Vas. Renal biopsy is an important means for diagnosis of Cryo GN, while membranous proliferative GN is the most common pathological type of Cryo GN. In recent years, great advances have been made in the treatment of Cryo Vas and its underlying diseases, and this review has briefly introduced these advances.Conclusions:Laboratory examinations of serum cryoglobulins urgently need standardization. The recent advances in the diagnosis and treatment of Cryo Vas and GN need to be popularized among the clinicians in related disciplines.

Delayed diagnosis of Angioimmunoblast T-cell lymphoma presenting with type II Cryoglobulinemia and acute kidney injury: a case report and narrative review of the literature

“…Cryoglobulinemia was classified by the Brouet criterion into 3 types, typeI, II, and III subtype. Their incidence respectively accounts for approximately 10–15%, 60–70% and 20–30% of cryoglobulinemic syndrome [ 39 ]. Kidney is frequently affected in mixed (Type II and III) cryoglobulinemia, and to a lesser degree in typeIcryoglobulinemia [ 40 , 41 ].…”

“…We suspect that the rapid deterioration of her glomerular filtration function was the result of capillary clog as well as a strong inflammatory reaction in the glomeruli. Although membranoproliferative glomerulonephritis (MPGN) is the predominant pathological phenotype accounting for 70 to 90% of all CN cases [ 39 ], other pathological types including endocapillary proliferative GN, mesangial proliferative GN, minimal change disease, and focal segmental glomerular sclerosis have been reported also [ 9 , 19 , 42 ], and they might correspond to a lower grade of inflammation and proliferation [ 43 ]. Interestingly, cellular phenotypic analysis has revealed that the endocapillary hypercellularity and capillary wall duplication in CN are sometimes the results of monocytic infiltration, rather than actual endothelial proliferation and mesangial cell and matrix interposition [ 43 ].…”

A Case of Cryoglobulinemia after Successful Hepatitis C Virus Treatment

“…Such patients can also exhibit cardiac ischemia, neuropathy, and gastrointestinal bleeding. Over 30% of patients also develop membranoproliferative glomerulonephritis and in some cases pulmonary vasculitis, which results in a worse prognosis [2].…”

“…Mixed cryoglobulinemia is associated with HCV in 70% to 90% of cases [2]. Less than 100 cases of mixed cryoglobulinemic vasculitis following SVR after HCV infection have been reported in the literature.…”

“…In most cases, the disease is characterized by mixed cryoglobulinemiain which abnormal immune complexes, termed cryoglobulins, composed of IgM, IgA, and/or rheumatoid factor aggregate in the blood. Cryoglobulins can be found in chronic infections, lymphoproliferative diseases, and connective tissue diseases, such as Sjögrens and systemic lupus erythematosus (SLE) [2]. Cryoglobulins are proinflammatory and can cause several symptoms, including purpura, arthralgias, fever, hemoptysis, hypercoagulable states, and glomerulonephritis.…”