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Calciphylaxis

MedGen UID:
2404
•Concept ID:
C0006666
•
Disease or Syndrome
Synonym: Idiopathic calciphylaxis
SNOMED CT: Calciphylaxis (237900002)
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:0017215
Orphanet: ORPHA280062

Definition

A rare vascular calcification disorder typically characterized by occlusion of microvessels in the cutaneous tissue resulting in painful cutaneous lesions. The disorder is often life-limiting. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCalciphylaxis
Follow this link to review classifications for Calciphylaxis in Orphanet.

Professional guidelines

PubMed

Calciphylaxis: Treatment and outlook-CME part II.
Rick J, Rrapi R, Chand S, Shinohara MM, Strowd L, Pasieka HB, Micheletti R, Kroshinsky D, Ortega-Loayza AG
J Am Acad Dermatol 2022 May;86(5):985-992. Epub 2022 Feb 22 doi: 10.1016/j.jaad.2021.10.063. PMID: 35202773
Calciphylaxis: Diagnosis, Pathogenesis, and Treatment.
Chang JJ
Adv Skin Wound Care 2019 May;32(5):205-215. doi: 10.1097/01.ASW.0000554443.14002.13. PMID: 31008757
Parathyroidectomy in the Management of Secondary Hyperparathyroidism.
Lau WL, Obi Y, Kalantar-Zadeh K
Clin J Am Soc Nephrol 2018 Jun 7;13(6):952-961. Epub 2018 Mar 9 doi: 10.2215/CJN.10390917. PMID: 29523679Free PMC Article

Recent clinical studies

Etiology

Calciphylaxis: Part I. Diagnosis and pathology.
Rick J, Strowd L, Pasieka HB, Saardi K, Micheletti R, Zhao M, Kroshinsky D, Shinohara MM, Ortega-Loayza AG
J Am Acad Dermatol 2022 May;86(5):973-982. Epub 2022 Feb 1 doi: 10.1016/j.jaad.2021.10.064. PMID: 35114300
Calciphylaxis: Diagnosis, Pathogenesis, and Treatment.
Chang JJ
Adv Skin Wound Care 2019 May;32(5):205-215. doi: 10.1097/01.ASW.0000554443.14002.13. PMID: 31008757
Calciphylaxis.
Nigwekar SU, Thadhani R, Brandenburg VM
N Engl J Med 2018 May 3;378(18):1704-1714. doi: 10.1056/NEJMra1505292. PMID: 29719190
Parathyroidectomy in the Management of Secondary Hyperparathyroidism.
Lau WL, Obi Y, Kalantar-Zadeh K
Clin J Am Soc Nephrol 2018 Jun 7;13(6):952-961. Epub 2018 Mar 9 doi: 10.2215/CJN.10390917. PMID: 29523679Free PMC Article
Calciphylaxis: risk factors, diagnosis, and treatment.
Nigwekar SU, Kroshinsky D, Nazarian RM, Goverman J, Malhotra R, Jackson VA, Kamdar MM, Steele DJ, Thadhani RI
Am J Kidney Dis 2015 Jul;66(1):133-46. Epub 2015 May 7 doi: 10.1053/j.ajkd.2015.01.034. PMID: 25960299Free PMC Article

Diagnosis

Calciphylaxis and Kidney Disease: A Review.
Gallo Marin B, Aghagoli G, Hu SL, Massoud CM, Robinson-Bostom L
Am J Kidney Dis 2023 Feb;81(2):232-239. Epub 2022 Aug 12 doi: 10.1053/j.ajkd.2022.06.011. PMID: 35970430
Calciphylaxis: Treatment and outlook-CME part II.
Rick J, Rrapi R, Chand S, Shinohara MM, Strowd L, Pasieka HB, Micheletti R, Kroshinsky D, Ortega-Loayza AG
J Am Acad Dermatol 2022 May;86(5):985-992. Epub 2022 Feb 22 doi: 10.1016/j.jaad.2021.10.063. PMID: 35202773
Calciphylaxis: Part I. Diagnosis and pathology.
Rick J, Strowd L, Pasieka HB, Saardi K, Micheletti R, Zhao M, Kroshinsky D, Shinohara MM, Ortega-Loayza AG
J Am Acad Dermatol 2022 May;86(5):973-982. Epub 2022 Feb 1 doi: 10.1016/j.jaad.2021.10.064. PMID: 35114300
Calciphylaxis: Diagnosis, Pathogenesis, and Treatment.
Chang JJ
Adv Skin Wound Care 2019 May;32(5):205-215. doi: 10.1097/01.ASW.0000554443.14002.13. PMID: 31008757
Calciphylaxis: risk factors, diagnosis, and treatment.
Nigwekar SU, Kroshinsky D, Nazarian RM, Goverman J, Malhotra R, Jackson VA, Kamdar MM, Steele DJ, Thadhani RI
Am J Kidney Dis 2015 Jul;66(1):133-46. Epub 2015 May 7 doi: 10.1053/j.ajkd.2015.01.034. PMID: 25960299Free PMC Article

Therapy

Calciphylaxis: Diagnosis, Pathogenesis, and Treatment.
Chang JJ
Adv Skin Wound Care 2019 May;32(5):205-215. doi: 10.1097/01.ASW.0000554443.14002.13. PMID: 31008757
Calciphylaxis.
Nigwekar SU, Thadhani R, Brandenburg VM
N Engl J Med 2018 May 3;378(18):1704-1714. doi: 10.1056/NEJMra1505292. PMID: 29719190
Parathyroidectomy in the Management of Secondary Hyperparathyroidism.
Lau WL, Obi Y, Kalantar-Zadeh K
Clin J Am Soc Nephrol 2018 Jun 7;13(6):952-961. Epub 2018 Mar 9 doi: 10.2215/CJN.10390917. PMID: 29523679Free PMC Article
Calciphylaxis.
Nigwekar SU
Curr Opin Nephrol Hypertens 2017 Jul;26(4):276-281. doi: 10.1097/MNH.0000000000000328. PMID: 28375870Free PMC Article
Calciphylaxis: risk factors, diagnosis, and treatment.
Nigwekar SU, Kroshinsky D, Nazarian RM, Goverman J, Malhotra R, Jackson VA, Kamdar MM, Steele DJ, Thadhani RI
Am J Kidney Dis 2015 Jul;66(1):133-46. Epub 2015 May 7 doi: 10.1053/j.ajkd.2015.01.034. PMID: 25960299Free PMC Article

Prognosis

Calciphylaxis.
Nigwekar SU, Thadhani R, Brandenburg VM
N Engl J Med 2018 May 3;378(18):1704-1714. doi: 10.1056/NEJMra1505292. PMID: 29719190
Calciphylaxis.
Nigwekar SU
Curr Opin Nephrol Hypertens 2017 Jul;26(4):276-281. doi: 10.1097/MNH.0000000000000328. PMID: 28375870Free PMC Article
Warfarin-Associated Nonuremic Calciphylaxis.
Yu WY, Bhutani T, Kornik R, Pincus LB, Mauro T, Rosenblum MD, Fox LP
JAMA Dermatol 2017 Mar 1;153(3):309-314. doi: 10.1001/jamadermatol.2016.4821. PMID: 28099971Free PMC Article
Calciphylaxis: risk factors, diagnosis, and treatment.
Nigwekar SU, Kroshinsky D, Nazarian RM, Goverman J, Malhotra R, Jackson VA, Kamdar MM, Steele DJ, Thadhani RI
Am J Kidney Dis 2015 Jul;66(1):133-46. Epub 2015 May 7 doi: 10.1053/j.ajkd.2015.01.034. PMID: 25960299Free PMC Article
Calciphylaxis.
Ng AT, Peng DH
Dermatol Ther 2011 Mar-Apr;24(2):256-62. doi: 10.1111/j.1529-8019.2011.01401.x. PMID: 21410615

Clinical prediction guides

Intravenous Sodium Thiosulphate for Calciphylaxis of Chronic Kidney Disease: A Systematic Review and Meta-analysis.
Wen W, Portales-Castillo I, Seethapathy R, Durant O, Mengesha B, Krinsky S, Kroshinsky D, Kalim S, Goverman J, Nazarian RM, Chitalia V, Malhotra R, Kramann R, Malhotra CK, Nigwekar SU
JAMA Netw Open 2023 Apr 3;6(4):e2310068. doi: 10.1001/jamanetworkopen.2023.10068. PMID: 37099293Free PMC Article
Revisiting therapeutic options for calciphylaxis.
Seethapathy H, Nigwekar SU
Curr Opin Nephrol Hypertens 2019 Sep;28(5):448-454. doi: 10.1097/MNH.0000000000000520. PMID: 31169527
Parathyroidectomy in the Management of Secondary Hyperparathyroidism.
Lau WL, Obi Y, Kalantar-Zadeh K
Clin J Am Soc Nephrol 2018 Jun 7;13(6):952-961. Epub 2018 Mar 9 doi: 10.2215/CJN.10390917. PMID: 29523679Free PMC Article
Calciphylaxis: diagnosis and clinical features.
Hayashi M
Clin Exp Nephrol 2013 Aug;17(4):498-503. Epub 2013 Feb 21 doi: 10.1007/s10157-013-0782-z. PMID: 23430392
Calciphylaxis.
Ng AT, Peng DH
Dermatol Ther 2011 Mar-Apr;24(2):256-62. doi: 10.1111/j.1529-8019.2011.01401.x. PMID: 21410615

Recent systematic reviews

Risk factors, treatment modalities, and clinical outcomes of penile calciphylaxis: systematic review.
Wipattanakitcharoen A, Takkavatakarn K, Susantitaphong P
World J Urol 2023 Nov;41(11):2959-2966. Epub 2023 Oct 2 doi: 10.1007/s00345-023-04611-9. PMID: 37782324
Sodium Thiosulfate for Calciphylaxis Treatment in Patients on Peritoneal Dialysis: A Systematic Review.
Gossett C, Suppadungsuk S, Krisanapan P, Tangpanithandee S, Thongprayoon C, Mao MA, Cheungpasitporn W
Medicina (Kaunas) 2023 Jul 14;59(7) doi: 10.3390/medicina59071306. PMID: 37512116Free PMC Article
Intravenous Sodium Thiosulphate for Calciphylaxis of Chronic Kidney Disease: A Systematic Review and Meta-analysis.
Wen W, Portales-Castillo I, Seethapathy R, Durant O, Mengesha B, Krinsky S, Kroshinsky D, Kalim S, Goverman J, Nazarian RM, Chitalia V, Malhotra R, Kramann R, Malhotra CK, Nigwekar SU
JAMA Netw Open 2023 Apr 3;6(4):e2310068. doi: 10.1001/jamanetworkopen.2023.10068. PMID: 37099293Free PMC Article
Local, non-systemic, and minimally invasive therapies for calcinosis cutis: a systematic review.
Nowaczyk J, Zawistowski M, Fiedor P
Arch Dermatol Res 2022 Aug;314(6):515-525. Epub 2021 Jun 24 doi: 10.1007/s00403-021-02264-5. PMID: 34165603Free PMC Article
Systematic review of sodium thiosulfate in treating calciphylaxis in chronic kidney disease patients.
Peng T, Zhuo L, Wang Y, Jun M, Li G, Wang L, Hong D
Nephrology (Carlton) 2018 Jul;23(7):669-675. doi: 10.1111/nep.13081. PMID: 28603903

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