Cryoglobulinemia based on interaction between a gamma macroglobulin and 7S gamma globulin

LoSpalluto, Joseph; Dorward, Barbara; Miller, William E.; Ziff, Morris

doi:10.1016/0002-9343(62)90191-2

Cited by 193 publications

(59 citation statements)

References 23 publications

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“…Mixed cryoglobulins (MCs) are immune complexes that typically consist of an IgM rheumatoid factor and immunoglobulins (most frequently IgG) that can precipitate at temperatures below 37 °C [1]. Associated with acute and chronic infections, lymphoproliferative disorders, and autoimmune diseases, they often have no real pathological significance but, in some cases, they may be responsible for serious and debilitating vasculitis syndromes with organ damage and a sometimes fatal outcome [2].…”

Section: Introductionmentioning

confidence: 99%

Recommendations for the management of mixed cryoglobulinemia syndrome in hepatitis C virus-infected patients

Pietrogrande¹,

Vita²,

Zignego³

et al. 2011

Autoimmunity Reviews

186

155

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Objective: The objective of this review was to define a core set of recommendations for the treatment of HCVassociated mixed cryoglobulinemia syndrome (MCS) by combining current evidence from clinical trials and expert opinion. Methods: Expert physicians involved in studying and treating patients with MCS formulated statements after discussing the published data. Their attitudes to treatment approaches (particularly those insufficiently supported by published data) were collected before the consensus conference by means of a questionnaire, and were considered when formulating the statements. Results: An attempt at viral eradication using pegylated interferon plus ribavirin should be considered the first-line therapeutic option in patients with mild-moderate HCV-related MCS. Prolonged treatment (up to 72 weeks) may be considered in the case of virological non-responders showing clinical and laboratory improvements. Rituximab (RTX) should be considered in patients with severe vasculitis and/or skin ulcers, peripheral neuropathy or glomerulonephritis. High-dose pulsed glucocorticoid (GC) therapy is useful in severe conditions and, when necessary, can be considered in combination with RTX; on the contrary, the majority of conference participants discouraged the chronic use of low-medium GC doses. Apheresis remains the elective treatment for severe, life-threatening hyper-viscosity syndrome; its use should be limited to patients who do not respond to (or who are ineligible for) other treatments, and emergency situations. Cyclophosphamide can be considered in combination with apheresis, but the data supporting its use are scarce. Despite the limited available data, colchicine is used by many of the conference participants, particularly in patients with mild-moderate MCS refractory to other therapies. Careful monitoring of the side effects of each drug, and its effects on HCV replication and liver function tests is essential. A lowantigencontent diet can be considered as supportive treatment in all symptomatic MCS patients. Although there are no data from controlled trials, controlling pain should always be attempted by tailoring the treatment to individual patients on the basis of the guidelines used in other vasculitides. Conclusion: Although there are few controlled randomised trials of MCS treatment, increasing knowledge of its pathogenesis is opening up new frontiers. The recommendations provided may be useful as provisional guidelines for the management of MCS.

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Section: Introductionmentioning

confidence: 99%

Recommendations for the management of mixed cryoglobulinemia syndrome in hepatitis C virus-infected patients

Pietrogrande¹,

Vita²,

Zignego³

et al. 2011

Autoimmunity Reviews

186

155

View full text Add to dashboard Cite

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“…"Mixed" cryoglobulins, in contrast, consist of IgG and IgM in varying proportions and have been described primarily in SLE (6), in a syndrome of purpura, weakness, and arthralgias (8,9), and in other patients with varying clinical pictures (10)(11)(12)(13).…”

Section: Introductionmentioning

confidence: 99%

Studies of Cryoproteins in Systemic Lupus Erythematosus*

Hanauer¹,

Christian²

1967

J. Clin. Invest.

108

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“…more immunoglobulins (mixed cryoglobulinemia), which precipitate at temperatures below 37°C and re-dissolve on re-warming [1,2]. This is an in vitro phenomenon ( Fig.…”

mentioning

confidence: 99%

“…This is an in vitro phenomenon ( Fig. 1), the actual mechanism(s) of cryoprecipitation remains obscure, it could be secondary to intrinsic characteristics of both mono-and polyclonal immunoglobulin (Ig) components, it can be caused as well by the interaction among single components of the cryoprecipitate [1][2][3][4][5][6][7][8][9][10][11][12][13][14].…”

mentioning

confidence: 99%

“…Type I cryoglobulinemia is almost invariably associated with well-known hematological disorders and is frequently asymptomatic per se; similarly, circulating mixed cryoglobulins are commonly detected in a great number of infectious or systemic disorders [1][2][3][4][5][6][7][8][9][10][11][12][13][14]. On the contrary, 'essential' MC represents a distinct disorder [3][4][5][6], which can be classified among systemic vasculitides [3][4][5][6][7][8][9][10].…”

mentioning

confidence: 99%

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