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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: 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
Calcinosis Cutis and Calciphylaxis.
Jiménez-Gallo D, Ossorio-García L, Linares-Barrios M
Actas Dermosifiliogr 2015 Dec;106(10):785-94. Epub 2015 Sep 26 doi: 10.1016/j.ad.2015.09.001. PMID: 26394755
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
Calcinosis Cutis and Calciphylaxis.
Jiménez-Gallo D, Ossorio-García L, Linares-Barrios M
Actas Dermosifiliogr 2015 Dec;106(10):785-94. Epub 2015 Sep 26 doi: 10.1016/j.ad.2015.09.001. PMID: 26394755
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: Approach to Diagnosis and Management.
Seethapathy H, Noureddine L
Adv Chronic Kidney Dis 2019 Nov;26(6):484-490. doi: 10.1053/j.ackd.2019.09.005. PMID: 31831126
Pathogenesis and management of vascular calcification in CKD and dialysis patients.
Kakani E, Elyamny M, Ayach T, El-Husseini A
Semin Dial 2019 Nov;32(6):553-561. Epub 2019 Aug 29 doi: 10.1111/sdi.12840. PMID: 31464003
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
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

Clinical prediction guides

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
Calciphylaxis.
Daudén E, Oñate MJ
Dermatol Clin 2008 Oct;26(4):557-68, ix. doi: 10.1016/j.det.2008.05.006. PMID: 18793990

Recent systematic reviews

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
Calciphylaxis in Patients With Normal Renal Function: A Case Series and Systematic Review.
Bajaj R, Courbebaisse M, Kroshinsky D, Thadhani RI, Nigwekar SU
Mayo Clin Proc 2018 Sep;93(9):1202-1212. Epub 2018 Jul 27 doi: 10.1016/j.mayocp.2018.06.001. PMID: 30060958
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
The efficacy and safety of sevelamer and lanthanum versus calcium-containing and iron-based binders in treating hyperphosphatemia in patients with chronic kidney disease: a systematic review and meta-analysis.
Habbous S, Przech S, Acedillo R, Sarma S, Garg AX, Martin J
Nephrol Dial Transplant 2017 Jan 1;32(1):111-125. doi: 10.1093/ndt/gfw312. PMID: 27651467
Calciphylaxis from nonuremic causes: a systematic review.
Nigwekar SU, Wolf M, Sterns RH, Hix JK
Clin J Am Soc Nephrol 2008 Jul;3(4):1139-43. Epub 2008 Apr 16 doi: 10.2215/CJN.00530108. PMID: 18417747Free PMC Article

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