MCID: CLC001
MIFTS: 52

Calciphylaxis

Categories: Blood diseases, Bone diseases, Cardiovascular diseases, Metabolic diseases, Rare diseases, Skin diseases

Aliases & Classifications for Calciphylaxis

MalaCards integrated aliases for Calciphylaxis:

Name: Calciphylaxis 12 77 54 60 45 15 74
Idiopathic Calciphylaxis 54

Characteristics:

Orphanet epidemiological data:

60
calciphylaxis
Inheritance: Not applicable; Prevalence: 1-9/100000 (Europe); Age of onset: Adult; Age of death: adult;

Classifications:



External Ids:

Disease Ontology 12 DOID:4734
MeSH 45 D002115
NCIt 51 C84607
ICD10 via Orphanet 35 E83.5
UMLS via Orphanet 75 C0006666
Orphanet 60 ORPHA280062
UMLS 74 C0006666

Summaries for Calciphylaxis

NIH Rare Diseases : 54 Calciphylaxis is a disease in which blood vessels (veins and arteries) become blocked by a build-up of calcium in the walls of the vessels, preventing blood from flowing to the skin or internal organs.  The lack of blood flow (ischemia) damages healthy tissue and causes it to die (necrosis).  The most obvious and frequent symptom of calciphylaxis is damage to the skin, as ulcers can develop and become infected easily.  Calciphylaxis can also affect fat tissue, internal organs, and skeletal muscle, causing infections, pain, and organ failure.  These symptoms are often irreversible, and many individuals with calciphylaxis may not survive more than a few months after they are diagnosed due to infection that spreads throughout the body (sepsis), or organ failure.  The exact cause of calciphylaxis is unknown.  Treatments may include medications to reduce pain, antibiotics to treat infections, and various approaches to preventing the development or worsening of this condition.

MalaCards based summary : Calciphylaxis, also known as idiopathic calciphylaxis, is related to end stage renal failure and hyperparathyroidism. An important gene associated with Calciphylaxis is ALB (Albumin), and among its related pathways/superpathways are Ca, cAMP and Lipid Signaling and Transcription_Role of VDR in regulation of genes involved in osteoporosis. The drugs Warfarin and Anticoagulants have been mentioned in the context of this disorder. Affiliated tissues include skin, kidney and bone, and related phenotypes are skin ulcer and cellulitis

Wikipedia : 77 Calciphylaxis, also known as calcific uremic arteriolopathy (CUA) or “Grey Scale”, is a rare painful... more...

Related Diseases for Calciphylaxis

Diseases related to Calciphylaxis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 162)
# Related Disease Score Top Affiliating Genes
1 end stage renal failure 30.6 ALB PTH
2 hyperparathyroidism 30.1 CASR FGF23 PTH VDR
3 osteitis fibrosa 29.9 CASR PTH
4 fournier gangrene 29.6 ALB CD40LG
5 kidney disease 29.6 ALB CASR FGF23 PTH VDR
6 chronic kidney failure 29.5 ALB CASR FGF23 PTH VDR
7 bone resorption disease 29.5 PTH VDR
8 hypoparathyroidism 29.5 CASR FGF23 PTH
9 hyperphosphatemia 29.2 CASR FGF23 PTH VDR
10 primary hyperparathyroidism 29.2 ALB CASR FGF23 PTH VDR
11 osteoporosis 28.9 CASR FGF23 PTH VDR
12 calciphylaxis cutis 12.2
13 visceral calciphylaxis 12.1
14 systemic lupus erythematosus 10.3
15 lupus erythematosus 10.3
16 secondary hyperparathyroidism 10.3
17 arthritis 10.2
18 poems syndrome 10.2
19 rheumatoid arthritis 10.2
20 calcinosis 10.2
21 hypocalciuric hypercalcemia, familial, type i 10.2 CASR PTH
22 hypocalciuric hypercalcemia, familial, type ii 10.2 CASR PTH
23 hypocalciuric hypercalcemia, familial, type iii 10.1 CASR PTH
24 pseudoxanthoma elasticum 10.1
25 alcoholic liver cirrhosis 10.1
26 thyroid crisis 10.1 ALB PTH
27 hypoparathyroidism, familial isolated 10.1 CASR PTH
28 familial isolated hyperparathyroidism 10.1 CASR PTH
29 familial hypocalciuric hypercalcemia 10.1 CASR PTH
30 oncogenic osteomalacia 10.1 FGF23 PTH
31 hypophosphatemic rickets, x-linked dominant 10.1 FGF23 PTH
32 protein s deficiency 10.1
33 ischemia 10.1
34 vasculitis 10.1
35 impaired renal function disease 10.1 FGF23 PTH
36 pulmonary alveolar microlithiasis 10.1 FGF23 PTH
37 metal metabolism disorder 10.1 CASR PTH
38 arterial calcification of infancy 10.1 FGF23 NT5E
39 hypercalciuria, absorptive, 2 10.1 CASR VDR
40 metaphyseal chondrodysplasia, jansen type 10.1 FGF23 PTH
41 temporal arteritis 10.1
42 nephrogenic systemic fibrosis 10.1
43 parathyroid carcinoma 10.1 CASR PTH
44 xanthinuria, type i 10.0 CASR VDR
45 hypophosphatemic rickets with hypercalciuria, hereditary 10.0 FGF23 PTH
46 idiopathic hypercalciuria 10.0 CASR VDR
47 nephrolithiasis, calcium oxalate 10.0 CASR VDR
48 arteries, anomalies of 10.0
49 breast cancer 10.0
50 myeloma, multiple 10.0

Graphical network of the top 20 diseases related to Calciphylaxis:



Diseases related to Calciphylaxis

Symptoms & Phenotypes for Calciphylaxis

Human phenotypes related to Calciphylaxis:

60 33 (show all 12)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 skin ulcer 60 33 hallmark (90%) Very frequent (99-80%) HP:0200042
2 cellulitis 60 33 hallmark (90%) Very frequent (99-80%) HP:0100658
3 sepsis 60 33 hallmark (90%) Very frequent (99-80%) HP:0100806
4 gangrene 60 33 hallmark (90%) Very frequent (99-80%) HP:0100758
5 cutis marmorata 60 33 hallmark (90%) Very frequent (99-80%) HP:0000965
6 hyperphosphatemia 60 33 hallmark (90%) Very frequent (99-80%) HP:0002905
7 stage 5 chronic kidney disease 60 33 hallmark (90%) Very frequent (99-80%) HP:0003774
8 secondary hyperparathyroidism 60 33 hallmark (90%) Very frequent (99-80%) HP:0000867
9 abnormality of skin physiology 60 33 hallmark (90%) Very frequent (99-80%) HP:0011122
10 arterial calcification 60 33 hallmark (90%) Very frequent (99-80%) HP:0003207
11 ectopic ossification 60 33 hallmark (90%) Very frequent (99-80%) HP:0011986
12 abnormality of metabolism/homeostasis 60 Very frequent (99-80%)

GenomeRNAi Phenotypes related to Calciphylaxis according to GeneCards Suite gene sharing:

27
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased melanin production GR00056-A 8.62 FGF23 NT5E

MGI Mouse Phenotypes related to Calciphylaxis:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 endocrine/exocrine gland MP:0005379 9.85 ALB CASR CD40LG FGF23 PTH VDR
2 cardiovascular system MP:0005385 9.83 CD40LG FGF23 NT5E PTH VDR
3 hematopoietic system MP:0005397 9.8 CASR CD40LG FGF23 NT5E PTH VDR
4 homeostasis/metabolism MP:0005376 9.8 ALB CASR CD40LG FGF23 NT5E PTH
5 digestive/alimentary MP:0005381 9.71 ALB CASR FGF23 VDR
6 mortality/aging MP:0010768 9.63 ALB CASR CD40LG FGF23 PTH VDR
7 renal/urinary system MP:0005367 9.43 ALB CASR CD40LG FGF23 NT5E VDR
8 skeleton MP:0005390 9.02 CASR CD40LG FGF23 PTH VDR

Drugs & Therapeutics for Calciphylaxis

Drugs for Calciphylaxis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 32)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Warfarin Approved Phase 4 81-81-2 54678486 6691
2 Anticoagulants Phase 4
3
Calcium Approved, Nutraceutical Phase 3,Phase 2 7440-70-2 271
4 Protective Agents Phase 3,Phase 2
5 Antioxidants Phase 3,Phase 2
6 sodium thiosulfate Phase 3,Phase 2
7 Anti-Infective Agents Phase 3,Phase 2
8 Anti-Bacterial Agents Phase 3,Phase 2
9 Antitubercular Agents Phase 3,Phase 2
10 Antidotes Phase 3,Phase 2
11 Chelating Agents Phase 3,Phase 2
12 Calcium, Dietary Phase 3,Phase 2
13 Pharmaceutical Solutions Phase 3,Phase 2
14
Calcium Carbonate Approved, Investigational Phase 2 471-34-1
15 Bone Density Conservation Agents Phase 2
16 Antacids Phase 2
17 Hormones Phase 2
18 Gastrointestinal Agents Phase 2
19 Anti-Ulcer Agents Phase 2
20 Menaquinone Approved, Investigational Not Applicable 1182-68-9
21
Phylloquinone Approved, Investigational Not Applicable 84-80-0
22
Menadione Approved, Nutraceutical Not Applicable 58-27-5 4055
23 Hemostatics Not Applicable
24 Antifibrinolytic Agents Not Applicable
25 Coagulants Not Applicable
26 Micronutrients Not Applicable
27 Nutrients Not Applicable
28 Trace Elements Not Applicable
29 naphthoquinone Not Applicable
30 Vitamins Not Applicable
31 Vitamin K Not Applicable
32 Vitamin K 1 Not Applicable

Interventional clinical trials:

(show all 17)
# Name Status NCT ID Phase Drugs
1 DANWARD - Danish Warfarin-Dialysis Study Not yet recruiting NCT03862859 Phase 4 Warfarin
2 A Phase 3 Clinical Trial of Intravenous Sodium Thiosulfate in Acute Calciphylaxis Patients Recruiting NCT03150420 Phase 3 Sodium Thiosulfate;Placebo-Normal Saline
3 Randomized Double Blind Placebo Controlled Trial of Sodium Thiosulfate for the Treatment of Pain Associated With Calcific Uremic Arteriolopath Terminated NCT02527213 Phase 3 Sodium thiosulfate;Placebo
4 Phase 2 Study With SNF472 in Calciphylaxis Patients Completed NCT02790073 Phase 2 SNF472
5 Efficacy and Tolerance of Sodium Thiosulfate Injection After Ultrasound-guided Percutaneous Irrigation of Calcific Tendinopathy of the Rotator Cuff Completed NCT02538939 Phase 2 Sodium thiosulfate
6 Topical Sodium Thiosulfate and Fractional Carbon Dioxide Laser in Treating Dermatomyositis Associated Calcinosis Completed NCT01572844 Phase 2 Sodium thiosulfate
7 Two phosphAte taRGets in End-stage Renal Disease Trial (TARGET) Completed NCT01994733 Phase 2 Calcium carbonate ( Intensive phosphate control);Calcium carbonate (Liberalized phosphate control)
8 A Pilot Study to Evaluate Topical Sodium Thiosulfate Therapy for Calcinosis Cutis Withdrawn NCT01918904 Phase 2 Sodium thiosulfate;Placebo
9 Efficacy of Lanthanum Carbonate in Calciphylaxis Completed NCT01289626 Phase 1 lanthanum carbonate;Lanthanum for calciphylaxis
10 Calciphylaxis : Population, Risk Factors, Diagnostic Practice, Therapeutic and Outcome Unknown status NCT02854046
11 Pathophysiology of Martorell Hypertensive Ischemic Leg Ulcer (HYTILU) Unknown status NCT01578382
12 The Early Administration of Sodium Thiosulfate Should Help to Reduce the Mortality of Dialysis Patients With Calciphylaxis, From a National Cohort Unknown status NCT03146793 Sodium Thiosulfate
13 Partners Calciphylaxis Biobank Recruiting NCT03032835
14 European Calciphylaxis Registry Network Recruiting NCT02635373
15 Evaluation of Vitamin K Supplementation for Calcific Uremic Arteriolopathy Recruiting NCT02278692 Not Applicable
16 Pragmatic Randomised Trial of High Or Standard PHosphAte Targets in End-stage Kidney Disease (PHOSPHATE) Not yet recruiting NCT03573089 Not Applicable Liberal phosphate target;Intensive phosphate target
17 Observational Follow-up to ST-001 Calciphylaxis Pain Treatment With Intravenous Sodium Thiosulfate Terminated NCT03319914

Search NIH Clinical Center for Calciphylaxis

Cochrane evidence based reviews: calciphylaxis

Genetic Tests for Calciphylaxis

Anatomical Context for Calciphylaxis

MalaCards organs/tissues related to Calciphylaxis:

42
Skin, Kidney, Bone, Breast, Skeletal Muscle, Liver, Lung

Publications for Calciphylaxis

Articles related to Calciphylaxis:

(show top 50) (show all 786)
# Title Authors Year
1
Demographics, Comorbid Conditions, and Outcomes of Patients With Nonuremic Calciphylaxis. ( 30601879 )
2019
2
The Effectiveness of Topical Cerium Nitrate-Silver Sulfadiazine Application on Overall Outcome in Patients with Calciphylaxis. ( 30605905 )
2019
3
Hypercalcemia associated with sodium thiosulfate treatment in a calciphylaxis patient. ( 31111826 )
2019
4
Calcific uremic arteriolopathy (calciphylaxis) calls into question the validity of guidelines of diagnosis and treatment. ( 31075991 )
2019
5
A persistent penile ulcer in end stage renal disease: It is not always calciphylaxis. ( 31050098 )
2019
6
Breakthrough invasive Trichosporon asahii infection in an uremic patient with systemic calciphylaxis complicating necrotizing fasciitis during echinocandin therapy for C. tropicalis. ( 31023578 )
2019
7
Calciphylaxis: Diagnosis, Pathogenesis, and Treatment. ( 31008761 )
2019
8
Calciphylaxis: Diagnosis, Pathogenesis, and Treatment. ( 31008757 )
2019
9
Digital Gangrene in a Dialysis Patient Due to Calciphylaxis and Systemic Polyarteritis Nodosa: A Diagnostic Dilemma. ( 30983759 )
2019
10
Phenprocoumon based anticoagulation is an underestimated factor in the pathogenesis of calciphylaxis. ( 30940121 )
2019
11
Successful treatment of extensive uremic calciphylaxis with intravenous sodium thiosulfate and its potential in treating various diseases of pathologic calcification. ( 30901068 )
2019
12
Calciphylaxis as A Rare Cause of A Chronic Wound in An 83-Year-Old Woman. ( 30889873 )
2019
13
Non-uremic calciphylaxis in a patient with multiple rheumatologic diseases. ( 30865406 )
2019
14
A unique clinical and histologic presentation of catastrophic systemic calciphylaxis in a nonuremic patient with systemic lupus erythematosus. ( 30854415 )
2019
15
Non-uremic calciphylaxis: a rare and late adverse reaction of warfarin. ( 30834840 )
2019
16
Calciphylaxis in a renal transplant recipient with normal graft function: A case report and review of literature. ( 30804284 )
2019
17
Resolution of acenocoumarol-associated calciphylaxis with drug withdrawal. ( 30790279 )
2019
18
Treatment of Calciphylaxis in CKD: A Systematic Review and Meta-analysis. ( 30775620 )
2019
19
Bilateral Mastodynia: An Unusual Presentation of Calciphylaxis. ( 30753809 )
2019
20
The use of cinacalcet for the treatment of calciphylaxis in patients with chronic kidney disease: A comprehensive review. ( 30666627 )
2019
21
Calciphylaxis and nephrogenic fibrosing dermopathy with pseudoxanthoma elasticum-like changes: Successful treatment with sodium thiosulfate. ( 30663093 )
2019
22
Sodium thiosulfate in calciphylaxis: Make a long story short. ( 30662931 )
2019
23
Hans Selye and his studies on the role of mast cells in calciphylaxis and calcergy. ( 30456595 )
2019
24
Palliative Care Use and Patterns of End-of-Life Care in Hospitalized Patients With Calciphylaxis. ( 30399415 )
2019
25
Non-uraemic calciphylaxis successfully treated with pamidronate infusion. ( 30393969 )
2019
26
Management of calciphylaxis in a burn center: A case series and review of the literature. ( 30322738 )
2019
27
Review: update on the management of calciphylaxis. ( 30304522 )
2019
28
Factors associated with false-negative pathologic diagnosis of calciphylaxis. ( 30280393 )
2019
29
Non-uremic calciphylaxis associated with alcoholic hepatitis: A case report. ( 30705725 )
2019
30
Chronic Kidney Disease and Calciphylaxis: A Literature Review. ( 30473967 )
2018
31
Calciphylaxis-as a drug induced adverse event. ( 30574812 )
2018
32
Calciphylaxis in haemodialysed patients: diagnostic value of calcifications in cutaneous biopsy. ( 28498603 )
2018
33
A 33year-old woman with calciphylaxis, diabetic kidney disease and abdominal pain. ( 28506812 )
2018
34
Calciphylaxis in patients with chronic kidney disease: A disease which is still bewildering and potentially fatal. ( 30415999 )
2018
35
Rheopheresis for Adjuvant Treatment in Resistant Calciphylaxis. ( 29430867 )
2018
36
Pancreatitis associated with calciphylaxis successfully treated with sodium thiosulfate. ( 29387772 )
2018
37
Non Uremic Calciphylaxis Post Liver Transplantation: A Case Report and Literature Review of an Unusual Presentation of a Rare Disease. ( 29386500 )
2018
38
Evaluating the validity of subclassifying warfarin-associated nonuremic calciphylaxis: a retrospective cohort study. ( 29336027 )
2018
39
Association Between Hypercoagulable Conditions and Calciphylaxis in Patients With Renal Disease: A Case-Control Study. ( 29238798 )
2018
40
3DTEE imaging of a descending aorta floating thrombus in a patient with calciphylaxis. ( 29226430 )
2018
41
Calciphylaxis with pseudoxanthoma elasticum-like changes: A case series. ( 29086985 )
2018
42
Anterior Ischemic Optic Neuropathy Due to Calciphylaxis. ( 29016409 )
2018
43
Systematic review of sodium thiosulfate in treating calciphylaxis in chronic kidney disease patients. ( 28603903 )
2018
44
Rare Case of Hematemesis: Calciphylaxis of the Esophagus. ( 28478301 )
2018
45
Non-Uremic Calciphylaxis: A Rare Diagnosis with Limited Therapeutic Strategies. ( 30755998 )
2018
46
Sodium thiosulfate and pamidronate for treatment of calciphylaxis: case report. ( 30700922 )
2018
47
Update on calciphylaxis etiopathogenesis, diagnosis, and management. ( 30657805 )
2018
48
Removing the problem: parathyroidectomy for calciphylaxis. ( 30580300 )
2018
49
Medication-Related Osteonecrosis of the Jaw in a Patient with Nonuremic Calciphylaxis. ( 30575515 )
2018
50
A Rare Case of Multiorgan Calciphylaxis in a Patient with Stage 5 Chronic Kidney Disease. ( 30420934 )
2018

Variations for Calciphylaxis

Expression for Calciphylaxis

Search GEO for disease gene expression data for Calciphylaxis.

Pathways for Calciphylaxis

GO Terms for Calciphylaxis

Biological processes related to Calciphylaxis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 regulation of signaling receptor activity GO:0010469 9.61 CD40LG FGF23 PTH
2 cellular calcium ion homeostasis GO:0006874 9.43 CASR PTH VDR
3 leukocyte cell-cell adhesion GO:0007159 9.4 CD40LG NT5E
4 vitamin D metabolic process GO:0042359 9.37 FGF23 VDR
5 cellular response to vitamin D GO:0071305 9.32 CASR FGF23
6 phosphate ion homeostasis GO:0055062 9.16 FGF23 PTH
7 response to fibroblast growth factor GO:0071774 8.96 PTH CASR
8 positive regulation of vitamin D 24-hydroxylase activity GO:0010980 8.62 VDR FGF23

Molecular functions related to Calciphylaxis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 drug binding GO:0008144 8.32 ALB

Sources for Calciphylaxis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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