Cryoglobulinemia

A condition characterized by the presence of abnormal quantities of CRYOGLOBULINS in the blood. Upon cold exposure, these abnormal proteins precipitate into the microvasculature leading to restricted blood flow in the exposed areas.

Also Known As:

Networked: 1236 relevant articles (88 outcomes, 75 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Infection
2.	Vasculitis (Vasculitides)
3.	Hepatitis C
4.	Glomerulonephritis
5.	Membranoproliferative Glomerulonephritis (Membranoproliferative Glomerulonephritis, Type II)

Experts

1.	Cacoub, Patrice: 24 articles (09/2015 - 01/2002)
2.	Ferri, Clodoveo: 20 articles (03/2014 - 06/2004)
3.	Antonelli, Alessandro: 19 articles (12/2014 - 06/2004)
4.	Fallahi, Poupak: 17 articles (12/2014 - 12/2007)
5.	Zignego, Anna Linda: 16 articles (10/2015 - 08/2003)
6.	Ferrari, Silvia Martina: 15 articles (12/2014 - 09/2008)
7.	Saadoun, David: 14 articles (09/2015 - 09/2005)
8.	Cacoub, P: 13 articles (04/2013 - 02/2000)
9.	Sansonno, Domenico: 11 articles (01/2015 - 05/2003)
10.	Ferri, C: 11 articles (08/2014 - 01/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Cryoglobulinemia:

1.	rituximab (Mabthera)FDA Link 09/01/2011 - "Several studies have demonstrated that the infusion of the anti-CD20 monoclonal antibody rituximab is highly effective for refractory mixed cryoglobulinemia, with a clinical response in approximately 80% of patients, although the relapse rate is high. " 10/01/2007 - "Rituximab at 375 mg/m(2) x 4 is effective for refractory HCV-related mixed cryoglobulinemia. " 01/01/2006 - "Rituximab is an effective treatment for mixed cryoglobulinemia. " 07/01/2009 - "In this open prospective study, rituximab appeared to be effective and safe in the treatment of patients with type II cryoglobulinemia-associated neuropathy." 09/01/2011 - "Based on a prior pilot study, we designed a phase II single-arm two-stage study (EUDRACT n. 2008-000086-38) to evaluate the efficacy of a lower dosage of rituximab, 250mg/m(2) given twice, for refractory mixed cryoglobulinemia. "
2.	Interferon-alpha (Interferon Alfa)FDA Link 07/01/1997 - "The efficacy of interferon-alpha treatment in the presence of cryoglobulinemia, particularly the rate of sustained responders, has not yet been well defined. " 12/01/1994 - "Treatment with interferon-alpha was found to be effective in mixed cryoglobulinemia even in the presence of HBe-minus HBV mutants.(ABSTRACT TRUNCATED AT 250 WORDS)" 01/01/2003 - "The efficacy of alpha interferon (IFN-alpha) in the treatment of severe type II essential mixed cryoglobulinemia (EMC) has been reported previously. " 01/01/1999 - "The association with cryoglobulinemia is clear and cryoglobulinemia-related symptoms are usually improved by interferon alpha treatment, although patients often relapse after the end of treatment. " 11/15/1997 - "We have prospectively studied patients with type II cryoglobulinemia since 1985 to assess the efficacy of treatment with interferon-alpha at cumulative doses ranging from 234 to 849 MU. "
3.	Ribavirin (Virazole)FDA LinkGeneric 06/01/1998 - "Patients treated with ribavirin had prompt decrease in serum aminotransferase levels and marked improvement of manifestations of cryoglobulinemia within a few weeks. " 09/01/2002 - "Experience with IFN-ribavirin in this entity is limited, but has shown promise in hepatic disease and has shown efficacy in HCV-associated cryoglobulinemia. " 11/01/2011 - "The aim of the present study is to provide information on clinical outcome of the patients affected by HCV-positive mixed cryoglobulinemia (MC) treated with PEG-IFN and Ribavirin for 6 or 12 months according to the HCV genotype. " 08/01/2015 - "Role of IL28B genotyping in patients with hepatitis C virus-associated mixed cryoglobulinemia and response to PEG-IFN and ribavirin treatment." 06/01/2013 - "In summary, combination antiviral therapy (pegylated IFN plus ribavirin) gives satisfactory response in more than the half of patients with symptomatic mixed cryoglobulinemia associated with HCV. "
4.	InterferonsIBA 01/01/2009 - "We found that interferon was highly effective in an HCV-positive patient with true essential type II mixed cryoglobulinemia. " 08/01/2001 - "This case report indicates that long-term maintenance interferon therapy was effective in the treatment of symptomatic mixed cryoglobulinemia and its renal complications and resulted in a clinical, biochemical, and virologic sustained response. " 11/01/2015 - "Hereby, we describe the case of a 40-year old man with severe hepatitis C virus-associated cryoglobulinemia, effectively treated with an interferon-free combination regimen. " 12/01/2013 - "Interferon mono-therapy for symptomatic HCV-associated mixed cryoglobulinemia : meta-analysis of clinical studies." 09/01/2011 - "High serum levels of CXCL11 in mixed cryoglobulinemia are associated with increased circulating levels of interferon-γ."
5.	CryoglobulinsIBA 02/01/2012 - "In the cryoglobulinemia patients there was excellent clearance of cryoglobulins during each treatment (mean decrease of 78.2 (SD 14.1)% per treatment). " 01/01/1997 - "These data were confirmed by studies in serial biopsy samples taken from individual patients with type II mixed cryoglobulinemia; the loss of lymphoid nodules with progression to more advanced histological stages of disease in these patients was accompanied by a decrease of the serum levels of cryoglobulins (although not statistically significant). " 01/01/2014 - "Raised cryoglobulins combined with a positive autoantibody screen suggested mixed cryoglobulinemia secondary to Sjögren syndrome. " 11/01/2013 - "Cryoglobulinemia is a unique model of human disease for several reasons: (1) cryoglobulins are detected using a simple technical approach that is based on in vitro laboratory observation of cold precipitation in serum; (2) cryoglobulinemic organ damage may be produced by two different etiopathogenic mechanisms (accumulation of cryoglobulins and autoimmune-mediated vasculitic damage); and (3) cryoglobulinemia is associated with a wide range of etiologies, symptoms, and outcomes, and is considered a disease that combines elements of autoimmune and lymphoproliferative diseases. " 05/01/2013 - "In conclusion, all types of cryoglobulins were found in patients with HCV-associated cryoglobulinemia, with type II being the most frequently identified. "
6.	glucuronyl glucosamine glycan sulfate (Vessel)IBA 04/01/1986 - "Immune deposits have been detected in vessel walls of forearm normal skin of patients with essential mixed cryoglobulinemia. " 04/01/1986 - "Abnormalities in dermal vessel walls of normal skin of patients with essential mixed cryoglobulinemia. " 02/01/1977 - "Both C3 and immunoglobin were regularly found in the vessel walls of 2 cases of cryoglobulinemia and 1 case of SLE. " 11/01/2014 - "Cryoglobulinemic vasculitis is a typical small vessel disease associated with cryoglobulinemia commonly bearing typical purpuric skin lesions. " 12/01/2007 - "Mixed cryoglobulinemia (MC) is a systemic small-vessel vasculitis; B-cell expansion is the biological substrate of the disease. "
7.	Prednisone (Sone)FDA LinkGeneric 07/01/1998 - "Recently, we have observed that most patients affected with HCV-associated mixed cryoglobulinemia (13 out of 15, 86.7%), that were at baseline normoglycemic, developed DM following corticosteroid treatment (prednisone > 25 mg/daily) for at least three months. " 07/01/1994 - "The authors report the results of clinical trials of a high capacity cryoglobulin filter (Cryofilter) in seven patients with cryoglobulinemia unresponsive to high doses of prednisone or immunosuppressive drugs who required plasmapheresis. " 12/01/1995 - "Thirty-three patients with II type mixed essential cryoglobulinemia (MEC) were randomized into two groups: one to receive combined therapy including prednisone plus interferon, the other to receive prednisone therapy. " 01/01/1991 - "A patient with leukocytoclastic vasculitis (LCV) with secondary mixed cryoglobulinemia was treated by cryofiltration in an effort to stop the progression of the disease despite the use of high dose prednisone. " 10/01/2015 - "A 60-year-old male with history of essential mixed cryoglobulinemia 12 years ago treated successfully with six months of cyclophosphamide and prednisone presented with bilateral lower extremity pupuric rash and swelling. "
8.	Erythema elevatum diutinumIBA 01/01/1991 - "Rapid improvement in a patient with leukocytoclastic vasculitis with secondary mixed cryoglobulinemia treated with cryofiltration." 03/01/2013 - "We report about a 39-year-old female patient with severe essential mixed cryoglobulinemia of type III with leukocytoclastic vasculitis. " 01/01/2011 - "Although leukocytoclastic vasculitis is more characteristic of mixed cryoglobulinemia, it can be a presenting manifestation of type 1 cryoglobulinemia." 01/01/2011 - "Leukocytoclastic vasculitis in a patient with type 1 cryoglobulinemia." 05/01/2009 - "Although it is not well known, this is not always the case because mixed cryoglobulinemia may histopathologically present itself as intravascular eosinophilic deposits without leukocytoclastic vasculitis. "
9.	AntigensIBA 11/01/1989 - "The effects of a low-antigen-content diet (LAC diet) versus a standard normocaloric diet on the signs and symptoms of mixed cryoglobulinemia (MC) were compared in a crossover randomized study. " 09/01/2015 - "Although the specific antigen involved is unknown, it is likely that the mixed cryoglobulinemia in Sjögren syndrome is triggered by the long-term B-cell stimulation, resulting in clonal proliferation of B cells. " 01/01/1994 - "The binding specificities identified appear to be different from the RFs isolated from patients with mixed cryoglobulinemia and may reflect a different antigen selection mechanism." 11/01/1989 - "Low-antigen-content diet in the treatment of patients with mixed cryoglobulinemia." 09/01/1981 - "A shared cryoglobulin antigen in familial cryoglobulinemia."
10.	Antigen-Antibody Complex (Immune Complex)IBA 01/01/2012 - "The pathogenesis of mixed cryoglobulinemia is mediated by immune complexes that are the most important cause of the vasculitic phenomena, typical of the disease. " 05/01/2011 - "Mixed cryoglobulinemia (MC) is a hepatitis C virus (HCV)-related immune complex disorder. " 01/01/2008 - "ACA were searched in serum and cryoprecipitate of 27 HCV infected patients with cryoglobulinemia--before and after dissociation of immune complexes. " 09/01/2007 - "In 20-30% of HCV-infected patients, RFs share characteristics of high affinity molecules, are monoclonal in nature, and result in the production of cold-precipitating immune complexes and mixed cryoglobulinemia (MC). " 03/01/2000 - "Mixed essential cryoglobulinemia is a disorder with deposition of circulating immune complexes in small and medium blood vessels. "

Therapies and Procedures

1.	Plasmapheresis 04/01/2014 - "The long-term use of plasmapheresis may be a well-tolerated treatment option for therapeutically challenging cases of cryoglobulinemia." 01/01/2015 - "Due to its high reproducibility, operator independence, low cost, and results obtained within 2 hours, the rapid test can be used as a "real time" monitoring of cryoglobulinemia related diseases and for the evaluation of plasmapheresis efficacy." 02/01/2012 - "There may be a role for cryofiltration in the treatment of cryoglobulinemia or where the application of other forms of plasmapheresis or immunoadsorption is limited. " 06/01/2010 - "The goal of this paper was to review over the past 20 years the medical literature for evidence supporting or refuting the reported use of plasmapheresis for cryoglobulinemia (January 1988 through June 2008). " 09/01/2007 - "In cryoglobulinemia, plasmapheresis can be used as effective further therapy to minimize cutaneous, renal and/or neurologic involvement."
2.	Plasma Exchange 06/01/2010 - "Although plasma exchange is an accepted treatment for cryoglobulinemia, there are no large multicentre randomized controlled trials of plasma exchange versus placebo or versus immunosuppressive therapy. " 06/01/2010 - "We included all reported literature of the use of plasma exchange for the treatment of cryoglobulinemia that included at least five patients. " 06/01/2010 - "Plasma exchange for treating cryoglobulinemia: a descriptive analysis." 07/01/2004 - "[From the Mailing List of the Italian Society of Nephrology: Pharmacological therapy and plasma exchange in the treatment of HCV-associated cryoglobulinemia]." 10/01/2003 - "The elective treatment for hyperviscosity syndrome, whether associated with monoclonal, mixed, or polyclonal cryoglobulinemia, is plasma exchange. "
3.	Aftercare (After-Treatment) 12/01/1997 - "Recurrence of cryoglobulinemia was observed in two thirds of the patients who were observed after treatment with IFN-alpha. " 05/01/2006 - "Association between mixed cryoglobulinemia, translocation (14;18), and persistence of occult HCV lymphoid infection after treatment." 03/15/2002 - "Regression of lymphoproliferative disorder after treatment for hepatitis C virus infection in a patient with partial trisomy 3, Bcl-2 overexpression, and type II cryoglobulinemia." 02/01/1997 - "We describe two patients with chronic hepatitis secondary to hepatitis C virus complicated by mixed cryoglobulinemia and membranoproliferative glomerulonephritis who developed severe renal failure which resolved after treatment with standard doses of IFN-alpha 2b." 08/01/2008 - "Cryoglobulinemia has also been described as occurring after treatment with rituximab. "
4.	Blood Component Removal (Apheresis) 07/01/1995 - "Cryofiltration apheresis (CA) is a specific therapy for treatment of patients with cryoglobulinemia. " 03/01/1980 - "Where an etiology for cryoglobulinemia is known and specific treatment exists, pheresis can be used as effective adjunct therapy." 03/01/1980 - "Pheresis can be used as primary therapy for reduction of cryoglobulin levels in cases of symptomatic essential cryoglobulinemia. " 01/01/2009 - "Immunoadsorption apheresis and immunosuppressive drug therapy in the treatment of complicated HCV-related cryoglobulinemia." 06/01/2003 - "Immunoadsorption apheresis (Selesorb) in the treatment of chronic hepatitis C virus-related type 2 mixed cryoglobulinemia."
5.	Injections 09/01/2000 - "Peri-lesional injections of granulocyte-macrophage colony-stimulating factor in the management of chronic leg ulcers in type II mixed cryoglobulinemia."