U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Cryoglobulinemic vasculitis

MedGen UID:
343814
•Concept ID:
C1852456
•
Disease or Syndrome
Synonyms: Cryoglobulinemia, familial mixed; MELTZER SYNDROME
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0007407
OMIM®: 123550
Orphanet: ORPHA91138

Definition

A rare immune complex-mediated vasculitis characterized by the presence of circulating cryoprecipitable immune complexes in the serum, manifesting clinically with the classical triad of purpura, weakness and arthralgia. [from ORDO]

Clinical features

From HPO
Hematuria
MedGen UID:
5488
•Concept ID:
C0018965
•
Disease or Syndrome
The presence of blood in the urine. Hematuria may be gross hematuria (visible to the naked eye) or microscopic hematuria (detected by dipstick or microscopic examination of the urine).
See: Feature record | Search on this feature
Proteinuria
MedGen UID:
10976
•Concept ID:
C0033687
•
Finding
Increased levels of protein in the urine.
See: Feature record | Search on this feature
Abnormal renal physiology
MedGen UID:
508816
•Concept ID:
C0151746
•
Pathologic Function
An abnormal functionality of the kidney.
See: Feature record | Search on this feature
Chronic kidney disease
MedGen UID:
473458
•Concept ID:
C1561643
•
Disease or Syndrome
Functional anomaly of the kidney persisting for at least three months.
See: Feature record | Search on this feature
Hypertensive disorder
MedGen UID:
6969
•Concept ID:
C0020538
•
Disease or Syndrome
The presence of chronic increased pressure in the systemic arterial system.
See: Feature record | Search on this feature
Abnormality of blood and blood-forming tissues
MedGen UID:
163092
•Concept ID:
C0850715
•
Finding
An abnormality of the hematopoietic system.
See: Feature record | Search on this feature
Cryoglobulinemia
MedGen UID:
3673
•Concept ID:
C0010403
•
Disease or Syndrome
Increased level of cryoglobulins in the blood. Cryoglobulins are abnormal immunoglobulins, especially IGG or IGM, that precipitate spontaneously when serum is cooled below 37 degrees Celsius.
See: Feature record | Search on this feature
Anasarca
MedGen UID:
101794
•Concept ID:
C0151603
•
Pathologic Function
An extreme form of generalized edema with widespread and massive edema due to effusion of fluid into the extracellular space.
See: Feature record | Search on this feature
Elevated circulating creatinine concentration
MedGen UID:
148579
•Concept ID:
C0700225
•
Finding
An increased amount of creatinine in the blood.
See: Feature record | Search on this feature
Show allHide all

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCryoglobulinemic vasculitis

Professional guidelines

PubMed

Recent updates in the diagnosis and management of cryoglobulinemic vasculitis.
Treppo E, Quartuccio L, De Vita S
Expert Rev Clin Immunol 2023 Jul-Dec;19(12):1457-1467. Epub 2023 Sep 12 doi: 10.1080/1744666X.2023.2249609. PMID: 37698547
Management of mixed cryoglobulinemia with rituximab: evidence and consensus-based recommendations from the Italian Study Group of Cryoglobulinemia (GISC).
Quartuccio L, Bortoluzzi A, Scirè CA, Marangoni A, Del Frate G, Treppo E, Castelnovo L, Saccardo F, Zani R, Candela M, Fraticelli P, Mazzaro C, Renoldi P, Scaini P, Filippini DA, Visentini M, Scarpato S, Giuggioli D, Mascia MT, Sebastiani M, Zignego AL, Lauletta G, Fiorilli M, Casato M, Ferri C, Pietrogrande M, Pioltelli PE, De Vita S, Monti G, Galli M
Clin Rheumatol 2023 Feb;42(2):359-370. Epub 2022 Sep 28 doi: 10.1007/s10067-022-06391-w. PMID: 36169798Free PMC Article
Diagnosis and management of leukocytoclastic vasculitis.
Fraticelli P, Benfaremo D, Gabrielli A
Intern Emerg Med 2021 Jun;16(4):831-841. Epub 2021 Mar 13 doi: 10.1007/s11739-021-02688-x. PMID: 33713282Free PMC Article

Recent clinical studies

Etiology

Management of mixed cryoglobulinemia with rituximab: evidence and consensus-based recommendations from the Italian Study Group of Cryoglobulinemia (GISC).
Quartuccio L, Bortoluzzi A, Scirè CA, Marangoni A, Del Frate G, Treppo E, Castelnovo L, Saccardo F, Zani R, Candela M, Fraticelli P, Mazzaro C, Renoldi P, Scaini P, Filippini DA, Visentini M, Scarpato S, Giuggioli D, Mascia MT, Sebastiani M, Zignego AL, Lauletta G, Fiorilli M, Casato M, Ferri C, Pietrogrande M, Pioltelli PE, De Vita S, Monti G, Galli M
Clin Rheumatol 2023 Feb;42(2):359-370. Epub 2022 Sep 28 doi: 10.1007/s10067-022-06391-w. PMID: 36169798Free PMC Article
The wide spectrum of cryoglobulinemic vasculitis and an overview of therapeutic advancements.
Dammacco F, Lauletta G, Vacca A
Clin Exp Med 2023 Jun;23(2):255-272. Epub 2022 Mar 28 doi: 10.1007/s10238-022-00808-1. PMID: 35348938Free PMC Article
A Review on Extrahepatic Manifestations of Chronic Hepatitis C Virus Infection and the Impact of Direct-Acting Antiviral Therapy.
Mazzaro C, Quartuccio L, Adinolfi LE, Roccatello D, Pozzato G, Nevola R, Tonizzo M, Gitto S, Andreone P, Gattei V
Viruses 2021 Nov 9;13(11) doi: 10.3390/v13112249. PMID: 34835054Free PMC Article
Management of Primary Small-Vessel Vasculitis.
Nwannunu CE, Shah R, Limmer AL
Skin Therapy Lett 2020 May;25(3):5-8. PMID: 32510891
Cryoglobulinemia Vasculitis.
Cacoub P, Comarmond C, Domont F, Savey L, Saadoun D
Am J Med 2015 Sep;128(9):950-5. Epub 2015 Mar 30 doi: 10.1016/j.amjmed.2015.02.017. PMID: 25837517

Diagnosis

Recent updates in the diagnosis and management of cryoglobulinemic vasculitis.
Treppo E, Quartuccio L, De Vita S
Expert Rev Clin Immunol 2023 Jul-Dec;19(12):1457-1467. Epub 2023 Sep 12 doi: 10.1080/1744666X.2023.2249609. PMID: 37698547
CRYOGLOBULINEMIC VASCULITIS.
Grugan BM, Nelson ME, Dyer SP
J Emerg Med 2022 Sep;63(3):e77-e79. Epub 2022 Oct 20 doi: 10.1016/j.jemermed.2022.09.004. PMID: 36272838
Diagnosis and management of leukocytoclastic vasculitis.
Fraticelli P, Benfaremo D, Gabrielli A
Intern Emerg Med 2021 Jun;16(4):831-841. Epub 2021 Mar 13 doi: 10.1007/s11739-021-02688-x. PMID: 33713282Free PMC Article
Cryoglobulinemic vasculitis: pathophysiological mechanisms and diagnosis.
Kolopp-Sarda MN, Miossec P
Curr Opin Rheumatol 2021 Jan;33(1):1-7. doi: 10.1097/BOR.0000000000000757. PMID: 33186245
New insights in cryoglobulinemic vasculitis.
Silva F, Pinto C, Barbosa A, Borges T, Dias C, Almeida J
J Autoimmun 2019 Dec;105:102313. Epub 2019 Aug 2 doi: 10.1016/j.jaut.2019.102313. PMID: 31383568

Therapy

Cryoglobulinemic vasculitis: a 2023 update.
Moretti M, Ferro F, Baldini C, Mosca M, Talarico R
Curr Opin Rheumatol 2024 Jan 1;36(1):27-34. Epub 2023 Nov 1 doi: 10.1097/BOR.0000000000000989. PMID: 37916482
Recent updates in the diagnosis and management of cryoglobulinemic vasculitis.
Treppo E, Quartuccio L, De Vita S
Expert Rev Clin Immunol 2023 Jul-Dec;19(12):1457-1467. Epub 2023 Sep 12 doi: 10.1080/1744666X.2023.2249609. PMID: 37698547
The wide spectrum of cryoglobulinemic vasculitis and an overview of therapeutic advancements.
Dammacco F, Lauletta G, Vacca A
Clin Exp Med 2023 Jun;23(2):255-272. Epub 2022 Mar 28 doi: 10.1007/s10238-022-00808-1. PMID: 35348938Free PMC Article
New insights in cryoglobulinemic vasculitis.
Silva F, Pinto C, Barbosa A, Borges T, Dias C, Almeida J
J Autoimmun 2019 Dec;105:102313. Epub 2019 Aug 2 doi: 10.1016/j.jaut.2019.102313. PMID: 31383568
Renal Manifestations of Hepatitis C Virus.
Ferri C, Giuggioli D, Colaci M
Clin Liver Dis 2017 Aug;21(3):487-497. Epub 2017 Apr 27 doi: 10.1016/j.cld.2017.03.005. PMID: 28689588

Prognosis

Cryoglobulinemic vasculitis: a 2023 update.
Moretti M, Ferro F, Baldini C, Mosca M, Talarico R
Curr Opin Rheumatol 2024 Jan 1;36(1):27-34. Epub 2023 Nov 1 doi: 10.1097/BOR.0000000000000989. PMID: 37916482
Management of mixed cryoglobulinemia with rituximab: evidence and consensus-based recommendations from the Italian Study Group of Cryoglobulinemia (GISC).
Quartuccio L, Bortoluzzi A, Scirè CA, Marangoni A, Del Frate G, Treppo E, Castelnovo L, Saccardo F, Zani R, Candela M, Fraticelli P, Mazzaro C, Renoldi P, Scaini P, Filippini DA, Visentini M, Scarpato S, Giuggioli D, Mascia MT, Sebastiani M, Zignego AL, Lauletta G, Fiorilli M, Casato M, Ferri C, Pietrogrande M, Pioltelli PE, De Vita S, Monti G, Galli M
Clin Rheumatol 2023 Feb;42(2):359-370. Epub 2022 Sep 28 doi: 10.1007/s10067-022-06391-w. PMID: 36169798Free PMC Article
Diagnosis and management of leukocytoclastic vasculitis.
Fraticelli P, Benfaremo D, Gabrielli A
Intern Emerg Med 2021 Jun;16(4):831-841. Epub 2021 Mar 13 doi: 10.1007/s11739-021-02688-x. PMID: 33713282Free PMC Article
Renal Manifestations of Hepatitis C Virus.
Ferri C, Giuggioli D, Colaci M
Clin Liver Dis 2017 Aug;21(3):487-497. Epub 2017 Apr 27 doi: 10.1016/j.cld.2017.03.005. PMID: 28689588
Cryoglobulinemia Vasculitis.
Cacoub P, Comarmond C, Domont F, Savey L, Saadoun D
Am J Med 2015 Sep;128(9):950-5. Epub 2015 Mar 30 doi: 10.1016/j.amjmed.2015.02.017. PMID: 25837517

Clinical prediction guides

Management of mixed cryoglobulinemia with rituximab: evidence and consensus-based recommendations from the Italian Study Group of Cryoglobulinemia (GISC).
Quartuccio L, Bortoluzzi A, Scirè CA, Marangoni A, Del Frate G, Treppo E, Castelnovo L, Saccardo F, Zani R, Candela M, Fraticelli P, Mazzaro C, Renoldi P, Scaini P, Filippini DA, Visentini M, Scarpato S, Giuggioli D, Mascia MT, Sebastiani M, Zignego AL, Lauletta G, Fiorilli M, Casato M, Ferri C, Pietrogrande M, Pioltelli PE, De Vita S, Monti G, Galli M
Clin Rheumatol 2023 Feb;42(2):359-370. Epub 2022 Sep 28 doi: 10.1007/s10067-022-06391-w. PMID: 36169798Free PMC Article
Hepatitis C virus-related cryoglobulinemic vasculitis.
Mazzaro C, Mauro E, Ermacora A, Doretto P, Fumagalli S, Tonizzo M, Toffolutti F, Gattei V
Minerva Med 2021 Apr;112(2):175-187. Epub 2020 Nov 16 doi: 10.23736/S0026-4806.20.07120-7. PMID: 33198444
The remarkable history of the hepatitis C virus.
Pol S, Lagaye S
Genes Immun 2019 May;20(5):436-446. Epub 2019 Apr 25 doi: 10.1038/s41435-019-0066-z. PMID: 31019253
Therapy of chronic hepatitis C virus infection in the era of direct-acting and host-targeting antiviral agents.
Conteduca V, Sansonno D, Russi S, Pavone F, Dammacco F
J Infect 2014 Jan;68(1):1-20. Epub 2013 Sep 4 doi: 10.1016/j.jinf.2013.08.019. PMID: 24012819
Vasculitis and infections: contribution to the issue of autoimmunity reviews devoted to "autoimmunity and infection".
Kallenberg CG, Tadema H
Autoimmun Rev 2008 Oct;8(1):29-32. Epub 2008 Aug 12 doi: 10.1016/j.autrev.2008.07.020. PMID: 18703171Free PMC Article

Recent systematic reviews

Efficacy of Therapeutic Apheresis for Cryoglobulinemic Vasculitis Patients with Renal Involvement: A Systematic Review.
Miao J, Krisanapan P, Tangpanithandee S, Thongprayoon C, Cheungpasitporn W
Blood Purif 2024;53(1):1-9. Epub 2023 Oct 18 doi: 10.1159/000534102. PMID: 37852193
Risk factors for relapse in non-infectious cryoglobulinemic vasculitis, including type I cryoglobulinemia: a systematic review.
Rajendran N, Rameli PM, Awad H
Front Immunol 2023;14:1215345. Epub 2023 Jul 7 doi: 10.3389/fimmu.2023.1215345. PMID: 37483620Free PMC Article
Management of mixed cryoglobulinemia with rituximab: evidence and consensus-based recommendations from the Italian Study Group of Cryoglobulinemia (GISC).
Quartuccio L, Bortoluzzi A, Scirè CA, Marangoni A, Del Frate G, Treppo E, Castelnovo L, Saccardo F, Zani R, Candela M, Fraticelli P, Mazzaro C, Renoldi P, Scaini P, Filippini DA, Visentini M, Scarpato S, Giuggioli D, Mascia MT, Sebastiani M, Zignego AL, Lauletta G, Fiorilli M, Casato M, Ferri C, Pietrogrande M, Pioltelli PE, De Vita S, Monti G, Galli M
Clin Rheumatol 2023 Feb;42(2):359-370. Epub 2022 Sep 28 doi: 10.1007/s10067-022-06391-w. PMID: 36169798Free PMC Article
Impact of direct acting antivirals on hepatitis C virus-related cryoglobulinemic syndrome.
Zignego AL, Marri S, Gragnani L
Minerva Gastroenterol (Torino) 2021 Sep;67(3):218-226. Epub 2021 Apr 1 doi: 10.23736/S2724-5985.21.02848-8. PMID: 33793154
Efficacy and safety of Rituximab in vasculitic neuropathy: a systematic review of the literature.
Mena-Vázquez N, Cabezudo-García P, Fuego Varela C, Manrique-Arija S, Fernandez-Nebro A
Reumatol Clin (Engl Ed) 2019 May-Jun;15(3):173-178. Epub 2019 Jan 25 doi: 10.1016/j.reuma.2018.10.007. PMID: 30691946

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • OMIM
      Related records in OMIM
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed
    • PubMed (OMIM)
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...