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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.
 
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).
Proteinuria
MedGen UID:
10976
Concept ID:
C0033687
Finding
Increased levels of protein in the urine.
Abnormal renal physiology
MedGen UID:
508816
Concept ID:
C0151746
Pathologic Function
An abnormal functionality of the kidney.
Chronic kidney disease
MedGen UID:
473458
Concept ID:
C1561643
Disease or Syndrome
Functional anomaly of the kidney persisting for at least three months.
Hypertensive disorder
MedGen UID:
6969
Concept ID:
C0020538
Disease or Syndrome
The presence of chronic increased pressure in the systemic arterial system.
Abnormality of blood and blood-forming tissues
MedGen UID:
163092
Concept ID:
C0850715
Finding
An abnormality of the hematopoietic system.
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.
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.
Elevated circulating creatinine concentration
MedGen UID:
148579
Concept ID:
C0700225
Finding
An increased amount of creatinine in the blood.

Term Hierarchy

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

Professional guidelines

PubMed

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
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
Nwannunu CE, Shah R, Limmer AL
Skin Therapy Lett 2020 May;25(3):5-8. PMID: 32510891

Recent clinical studies

Etiology

Misra DP, Sharma A, Karpouzas GA, Kitas GD
Best Pract Res Clin Rheumatol 2023 Mar;37(1):101831. Epub 2023 Jun 9 doi: 10.1016/j.berh.2023.101831. PMID: 37302927
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
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
Corouge M, Vallet-Pichard A, Pol S
Liver Int 2016 Jan;36 Suppl 1:28-33. doi: 10.1111/liv.13022. PMID: 26725894
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

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
Maltseva N, Borzova E, Fomina D, Bizjak M, Terhorst-Molawi D, Košnik M, Kulthanan K, Meshkova R, Thomsen SF, Maurer M; COLD-CE Steering Committee
Allergy 2021 Apr;76(4):1077-1094. Epub 2020 Dec 24 doi: 10.1111/all.14674. PMID: 33249577
Kolopp-Sarda MN, Miossec P
Curr Opin Rheumatol 2021 Jan;33(1):1-7. doi: 10.1097/BOR.0000000000000757. PMID: 33186245
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
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

Therapy

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
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
Maltseva N, Borzova E, Fomina D, Bizjak M, Terhorst-Molawi D, Košnik M, Kulthanan K, Meshkova R, Thomsen SF, Maurer M; COLD-CE Steering Committee
Allergy 2021 Apr;76(4):1077-1094. Epub 2020 Dec 24 doi: 10.1111/all.14674. PMID: 33249577
Fuentes A, Mardones C, Burgos PI
Curr Rheumatol Rep 2019 Nov 19;21(11):60. doi: 10.1007/s11926-019-0859-0. PMID: 31741077
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

Prognosis

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
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
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
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
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

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
De Vita S, Gandolfo S
Expert Rev Clin Immunol 2019 Sep;15(9):929-938. Epub 2019 Sep 12 doi: 10.1080/1744666X.2019.1649596. PMID: 31347413
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
Lopalco G, Rigante D, Venerito V, Emmi G, Anelli MG, Lapadula G, Iannone F, Cantarini L
Curr Rheumatol Rep 2016 Jun;18(6):36. doi: 10.1007/s11926-016-0580-1. PMID: 27118389
Lally L, Spiera R
Curr Opin Rheumatol 2016 Jan;28(1):15-20. doi: 10.1097/BOR.0000000000000235. PMID: 26599379

Recent systematic reviews

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
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
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
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
Cacoub P, Delluc A, Saadoun D, Landau DA, Sene D
Ann Rheum Dis 2008 Mar;67(3):283-7. Epub 2007 Jul 20 doi: 10.1136/ard.2006.065565. PMID: 17644544

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