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: 1567 relevant articles (127 outcomes, 100 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

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

Experts

1.	Cacoub, Patrice: 43 articles (01/2021 - 01/2002)
2.	Saadoun, David: 29 articles (01/2021 - 05/2004)
3.	Zignego, Anna Linda: 26 articles (01/2021 - 08/2003)
4.	Ferri, Clodoveo: 22 articles (01/2017 - 06/2004)
5.	Antonelli, Alessandro: 21 articles (12/2019 - 06/2004)
6.	Fallahi, Poupak: 19 articles (12/2019 - 12/2007)
7.	Ferrari, Silvia Martina: 17 articles (12/2019 - 09/2008)
8.	Gragnani, Laura: 16 articles (09/2021 - 08/2003)
9.	Casato, Milvia: 15 articles (04/2022 - 03/2002)
10.	Visentini, Marcella: 14 articles (04/2022 - 10/2007)

Drugs and Biologics

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

1.	Rituximab (Mabthera)FDA Link 03/01/2016 - "Idiopathic Nonviral Cryoglobulinemia Treated Successfully With Rituximab." 03/01/2017 - "Our results suggest that repeated treatment of relapsing mixed cryoglobulinemia with a low-dose rituximab regimen is efficacious, safe, and cost-effective for the long-term management of this disorder." 10/01/2007 - "Rituximab at 375 mg/m(2) x 4 is effective for refractory HCV-related mixed cryoglobulinemia. " 01/27/2006 - "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."
2.	Interferon-alpha (Interferon Alfa)IBA 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.	Antiviral Agents (Antivirals)IBA 10/01/2018 - "Are direct-acting antivirals safe and effective in hepatitis C virus-cryoglobulinemia? " 12/01/2005 - "Our present study first reports the onset of asymptomatic mixed cryoglobulinemia in hepatitis C virus patients after clearance of the virus from blood obtained with a combined antiviral treatment. " 01/01/2022 - "We aimed to investigate the efficacy of antiviral treatment in cryoglobulinemia patients and analyze the associated factors of persistent cryoglobulinemia. " 04/01/2015 - "Limited data are available about the efficacy of antiviral treatment in hepatitis C virus (HCV)-associated mixed cryoglobulinemia (MC), especially concerning the long-term effects of HCV eradication. " 12/01/2022 - "Little is known on the changes in cryoglobulinemia mortality after the introduction of direct-acting antivirals (DAA) for treatment of HCV in 2014 in the USA. "
4.	InterferonsIBA 01/01/2009 - "We found that interferon was highly effective in an HCV-positive patient with true essential type II mixed cryoglobulinemia. " 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. " 01/01/2017 - "Virological and Clinical Response to Interferon-Free Regimens in Patients with HCV-Related Mixed Cryoglobulinemia: Preliminary Results of a Prospective Pilot Study." 09/01/2011 - "High serum levels of CXCL11 in mixed cryoglobulinemia are associated with increased circulating levels of interferon-γ." 01/01/2009 - "Interferon might be anticipated to be effective only in HCV-associated cryoglobulinemias. "
5.	Ribavirin (Virazole)FDA LinkGeneric 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. " 07/01/1996 - "A larger-scale study is warranted to test the effect of ribavirin on post-OLT HCV-associated cryoglobulinemia." 08/01/2015 - "Role of IL28B genotyping in patients with hepatitis C virus-associated mixed cryoglobulinemia and response to PEG-IFN and ribavirin treatment." 08/01/2003 - "Combination therapy of IFN plus ribavirin could be useful for patients with mixed cryoglobulinemia resistant to IFN as monotherapy."
6.	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). " 10/01/2000 - "As there was a recurrence of acrocyanosis after the final vaccination, vaccination with Mo-DCs pulsed with purified cryoglobulin would seem to be a useful treatment for refractory essential monoclonal cryoglobulinemia." 05/01/1980 - "All three responded with a reduction in cryoglobulin level and an improvement in clinical manifestations of cryoglobulinemia. " 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). " 03/22/2023 - "Such elevated tires of cryoglobulins in the context of the patient's CRAO raised suspicion of cryoglobulinemia associated CRAO. "
7.	Adrenal Cortex Hormones (Corticosteroids)IBA 04/01/2003 - "(11) Corticosteroids may be useful in the treatment of symptomatic mixed cryoglobulinemia. " 03/01/2015 - "Hereby, we report a case of atypical RTH at CT with no signal suppression on chemical-shift MRI, arose after corticosteroid treatment for mixed cryoglobulinemia in a 60-year-old woman. " 03/01/1993 - "The outcome was satisfactory following rest only but the existence of a mixed cryoglobulinemia, responsible secondarily for cold-related distal ischemic symptomatology of the lower limbs led to the successful prescription of a short course of corticosteroids." 01/01/1978 - "Treatment with corticosteroids was associated with resolution of the cryoglobulinemia and complete clinical recovery." 01/01/2017 - "The patient presented to an outside hospital and was treated for presumptive mixed cryoglobulinemia with corticosteroid therapy. "
8.	Prednisone (Sone)FDA LinkGeneric 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. " 03/01/2016 - "We report a case of 77-year-old female patient diagnosed with idiopathic cryoglobulinemia, treated successfully with 6 months prednisone tapering and 2 doses of rituximab (1 g each dose). " 01/01/1992 - "Deflazacort (DF) and prednisone (PD) were administered to 31 consecutive symptomatic patients with essential mixed cryoglobulinemia (EMC) in a double-blind randomized study lasting 22 months. " 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. " 01/01/1992 - "Deflazacort versus Prednisone in the Treatment of Essential Mixed Cryoglobulinemia A Between-Patient Controlled Clinical Study."
9.	SteroidsIBA 01/01/2023 - "The presence of other possible causes like cryoglobulinemia could induce avoidance of nerve biopsy thus undertreating patient, since steroid treatment is not expected to be efficient on lymphocytic proliferation. " 01/01/2014 - "We also discuss various concerns regarding steroid treatment in patients with mixed cryoglobulinemia complicated by HBV infection. " 07/25/2013 - "His uveitis, refractory to steroid treatments, was well controlled following treatments for cryoglobulinemia. " 01/01/2008 - "Therefore, the possibility of the latent presence of cryoglobulinemia in cases with HSP having an active rash refractory to steroid treatment should not be overlooked." 09/01/1981 - "A prospective clinical study evaluated the effects of combined chemotherapy, steroids and plasmapheresis on 5 patients with mixed cryoglobulinemia with renal or neurologic and vasculitic manifestations. "
10.	Antigen-Antibody Complex (Immune Complex)IBA 01/01/2023 - "Mixed cryoglobulinemia (MC) is a hepatitis C virus (HCV)-related extrahepatic manifestation that is characterized by the abnormal presence of immune complexes (ICs). " 01/01/2020 - "We provided evidence that kidney lesions are due to immune complex deposition and to glomerular obstruction by cryoglobulinemia and RTX." 05/01/2016 - "The mechanisms through which these develop include those which are immunological, in which the chronic persistence of virus leads to the circulation of immune complexes (mixed cryoglobulinemia) and other autoimmune phenomena, and those which are virological and related to the extrahepatic tropism of the virus to other tissues. " 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. "

Therapies and Procedures

1.	Therapeutics 04/01/2015 - "Effective antiretroviral therapy seems to be protective against cryoglobulinemia development in HIV mono-infected patients. " 03/01/2000 - "These observations suggest that IFN-alpha therapy may be effective against CSS in HCV infected patients with or without cryoglobulinemia." 07/01/2006 - "Many disease associations and therapies have been described but clinical trials are few, and the natural history, causes, therapy, and pathways of cryoglobulinemia require further investigation. " 09/01/2000 - "Therapy for cryoglobulinemia secondary to hepatitis C virus: the need for tailored protocols and multiclinic studies." 01/01/2021 - "Lower C3 levels were noted in SVR patients with than those without post-therapy mixed cryoglobulinemia. "
2.	Plasmapheresis 04/01/2014 - "The long-term use of plasmapheresis may be a well-tolerated treatment option for therapeutically challenging cases of cryoglobulinemia." 02/01/2023 - "Cryoglobulinemia and double-filtration plasmapheresis: Personal experience and literature review." 02/01/2018 - "In non-infectious mixed cryoglobulinemia plasmapheresis is indicated when the disease manifestations are severe, as a second line option. " 01/01/2016 - "Preoperative plasmapheresis before hypothermic coronary bypass can prevent fatal cryoglobulinemia-related complications in patients with active disease." 01/01/2016 - "We report the case of a patient with active cryoglobulinemia who was preoperatively managed with plasmapheresis. "
3.	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. " 08/01/2016 - "Successful use of cryocrit for monitoring response to therapeutic plasma exchange in type 1 cryoglobulinemia." 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." 10/01/2003 - "The elective treatment for hyperviscosity syndrome, whether associated with monoclonal, mixed, or polyclonal cryoglobulinemia, is plasma exchange. "
4.	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." 08/01/1996 - "Additional features not commonly seen in fibromyalgia patients included fluctuating synovitis in 5 patients, biopsy-proven leukocytoclastic vasculitis in 5, sicca symptoms in 3, Raynaud's phenomenon in 3, and cryoglobulinemia in 2. One patient died in multi-organ failure after treatment for systemic vasculitis. "
5.	Liver Transplantation 09/01/2008 - "Cryoglobulinemia is frequent after liver transplant and is symptomatic in approximately 20% of all patients." 09/01/2008 - "To examine the prevalence of cryoglobulinemia and autoimmune markers in stable liver transplant recipients and to determine risk factors and clinical impact. " 09/01/2008 - "Prevalence of cryoglobulinemia and autoimmune markers in liver transplant patients." 07/01/2004 - "Immunocompetence, autoimmunity and clonal expansion of B cell lymphocytes have not been analysed simultaneously in previous reports of patients with cryoglobulinemia after liver transplantation. " 03/01/2000 - "In conclusion, mixed cryoglobulinemia was found in approximately 20% of the HCV+ liver transplant recipients. "