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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

Diagnosis and treatment of calciphylaxis in patients with chronic kidney disease.
Lucca LJ, Moysés RMA, Lima Neto AS
J Bras Nefrol 2021;43(4 Suppl 1):665-668. Epub 2021 Dec 3 doi: 10.1590/2175-8239-JBN-2021-S111. PMID: 34910803Free PMC Article
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
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: 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: 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.
Zhou Q, Neubauer J, Kern JS, Grotz W, Walz G, Huber TB
Lancet 2014 Mar 22;383(9922):1067. Epub 2014 Feb 28 doi: 10.1016/S0140-6736(14)60235-X. PMID: 24582472

Prognosis

Common and critical inflammatory dermatoses every pathologist should know.
Billings SD
Mod Pathol 2020 Jan;33(Suppl 1):107-117. Epub 2019 Nov 1 doi: 10.1038/s41379-019-0400-z. PMID: 31676787
Pigmented purpura and cutaneous vascular occlusion syndromes.
Lamadrid-Zertuche AC, Garza-Rodríguez V, Ocampo-Candiani JJ
An Bras Dermatol 2018 Jun;93(3):397-404. doi: 10.1590/abd1806-4841.20187459. PMID: 29924250Free PMC Article
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

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
Common and critical inflammatory dermatoses every pathologist should know.
Billings SD
Mod Pathol 2020 Jan;33(Suppl 1):107-117. Epub 2019 Nov 1 doi: 10.1038/s41379-019-0400-z. PMID: 31676787
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.
Daudén E, Oñate MJ
Dermatol Clin 2008 Oct;26(4):557-68, ix. doi: 10.1016/j.det.2008.05.006. PMID: 18793990
Panniculitis: recent developments and observations.
White WL, Wieselthier JS, Hitchcock MG
Semin Cutan Med Surg 1996 Dec;15(4):278-99. doi: 10.1016/s1085-5629(96)80042-3. PMID: 9069596

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
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
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
Calciphylaxis: a systematic review of existing and emerging therapies.
Vedvyas C, Winterfield LS, Vleugels RA
J Am Acad Dermatol 2012 Dec;67(6):e253-60. Epub 2011 Aug 6 doi: 10.1016/j.jaad.2011.06.009. PMID: 21821309

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