MCID: CLC001
MIFTS: 50

Calciphylaxis

Categories: Blood diseases, Bone diseases, Cardiovascular diseases, Metabolic diseases, Rare diseases, Skin diseases
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Aliases & Classifications for Calciphylaxis

MalaCards integrated aliases for Calciphylaxis:

Name: Calciphylaxis 11 19 58 75 43 14 71
Idiopathic Calciphylaxis 19

Characteristics:


Age Of Onset:

Adult 58

Classifications:

Orphanet: 58  
Rare circulatory system diseases
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 11 DOID:4734
MeSH 43 D002115
NCIt 49 C84607
SNOMED-CT 68 237900002
ICD10 via Orphanet 32 E83.5
UMLS via Orphanet 72 C0006666
Orphanet 58 ORPHA280062
UMLS 71 C0006666

Summaries for Calciphylaxis

GARD: 19 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 may causes the tissue 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. The exact cause of Calciphylaxis is unknown.

MalaCards based summary: Calciphylaxis, also known as idiopathic calciphylaxis, is related to hyperparathyroidism and secondary hyperparathyroidism. An important gene associated with Calciphylaxis is FGF23 (Fibroblast Growth Factor 23), and among its related pathways/superpathways are Vitamin D receptor pathway and Ca, cAMP and Lipid Signaling. The drugs Magnesium citrate and Phylloquinone have been mentioned in the context of this disorder. Affiliated tissues include skin, kidney and skeletal muscle, and related phenotypes are skin ulcer and cellulitis

Orphanet: 58 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.

Wikipedia: 75 Calciphylaxis, also known as calcific uremic arteriolopathy (CUA) or “Grey Scale”, is a rare syndrome... more...

Related Diseases for Calciphylaxis

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

(show top 50) (show all 386)
# Related Disease Score Top Affiliating Genes
1 hyperparathyroidism 30.7 VDR PTH FGF23 EPO CASR ALB
2 secondary hyperparathyroidism 30.5 VDR PTH FGF23 EPO CASR ALB
3 necrotizing fasciitis 30.2 FN1 ALB
4 primary hyperparathyroidism 30.2 VDR PTH FGF23 CASR ALB
5 calcinosis 30.2 MGP FGF23 AHSG
6 arteriolosclerosis 30.2 CD79A ALB
7 parathyroid adenoma 30.0 VDR PTH CASR ALB
8 decubitus ulcer 30.0 FN1 ALB
9 vascular disease 30.0 MGP FN1 CD40LG ALB
10 pseudoxanthoma elasticum 30.0 NT5E MGP GGCX
11 graves disease 1 30.0 PTH ALB
12 rickets 30.0 VDR PTH FGF23 CASR ALB
13 nutritional deficiency disease 29.9 MGP EPO ALB
14 osteitis fibrosa 29.9 PTH FGF23 CASR
15 hyperphosphatemia 29.8 VDR PTH MGP FGF23 CASR ALB
16 connective tissue disease 29.8 PTH FN1 FGF23 CD40LG ALB
17 hypoparathyroidism 29.8 VDR PTH FGF23 CASR
18 glomerulonephritis 29.8 FN1 CD79A CD40LG ALB
19 urolithiasis 29.8 VDR GGCX CASR ALB
20 vitamin k deficiency bleeding 29.8 PTH MGP GGCX AHSG
21 myocarditis 29.7 FN1 CD40LG ALB
22 myeloma, multiple 29.7 FN1 EPO CD79A CD40LG
23 nephrotic syndrome 29.7 FN1 EPO CD79A ALB
24 iga glomerulonephritis 29.7 FN1 CD79A CD40LG ALB
25 congestive heart failure 29.6 FN1 EPO ALB
26 hypophosphatemia 29.6 VDR PTH FGF23 ALB
27 renal osteodystrophy 29.6 VDR PTH FGF23 CASR
28 hypersensitivity vasculitis 29.5 CD79A ALB
29 cryoglobulinemia 29.5 FN1 CD79A CD40LG
30 autoimmune disease 29.5 VDR FN1 CD79A CD40LG
31 bone disease 29.4 VDR PTH FGF23 EPO CD40LG CASR
32 spondyloarthropathy 1 29.4 PTH CD79A CD40LG
33 acquired immunodeficiency syndrome 29.4 EPO CD79A CD40LG ALB
34 skin disease 29.3 VDR FN1 CD79A CD40LG ALB
35 hypocalcemia, autosomal dominant 1 29.2 VDR PTH FGF23 CASR ALB
36 immune deficiency disease 29.2 VDR NT5E FN1 CD79A CD40LG
37 polycystic kidney disease 29.2 PTH FN1 FGF23 EPO ALB
38 chronic kidney disease 29.1 VDR PTH MGP FGF23 EPO CD79A
39 lipoprotein quantitative trait locus 29.0 PTH FN1 FGF23 CD40LG ALB
40 parathyroid gland disease 29.0 VDR PTH MGP FGF23 EPO CASR
41 nephrolithiasis 28.7 VDR PTH FN1 FGF23 CASR AHSG
42 kidney disease 28.6 VDR PTH MGP FGF23 EPO CD79A
43 uremia 28.6 VDR PTH MGP FGF23 EPO CASR
44 arterial calcification of infancy 28.5 NT5E MGP GGCX FGF23 AHSG
45 diabetes mellitus 28.3 VDR PTH FGF23 EPO CD40LG CASR
46 mineral metabolism disease 27.9 VDR PTH MGP FGF23 EPO CASR
47 osteoporosis 27.2 VDR PTH MGP GGCX FGF23 EPO
48 calciphylaxis cutis 11.3
49 visceral calciphylaxis 11.0
50 end stage renal disease 10.9

Graphical network of the top 20 diseases related to Calciphylaxis:



Diseases related to Calciphylaxis

Symptoms & Phenotypes for Calciphylaxis

Human phenotypes related to Calciphylaxis:

58 30 (show all 12)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 skin ulcer 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0200042
2 cellulitis 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0100658
3 sepsis 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0100806
4 cutis marmorata 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0000965
5 gangrene 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0100758
6 stage 5 chronic kidney disease 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0003774
7 hyperphosphatemia 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002905
8 abnormality of skin physiology 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0011122
9 secondary hyperparathyroidism 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0000867
10 arterial calcification 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0003207
11 ectopic ossification 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0011986
12 abnormality of metabolism/homeostasis 58 Very frequent (99-80%)

MGI Mouse Phenotypes related to Calciphylaxis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.25 AHSG ALB CASR CD40LG CD79A EPO
2 cellular MP:0005384 10.1 ALB CASR CD40LG CD79A EPO FN1
3 renal/urinary system MP:0005367 10.08 ALB CASR CD40LG CD79A FGF23 MGP
4 cardiovascular system MP:0005385 10.07 ALB CD40LG EPO FGF23 FN1 GGCX
5 muscle MP:0005369 10 AHSG ALB CASR EPO FN1 MGP
6 immune system MP:0005387 9.97 ALB CASR CD40LG CD79A EPO FGF23
7 skeleton MP:0005390 9.91 AHSG CASR CD40LG FGF23 FN1 GGCX
8 hematopoietic system MP:0005397 9.85 CASR CD40LG CD79A EPO FGF23 FN1
9 mortality/aging MP:0010768 9.7 ALB CASR CD40LG CD79A EPO FGF23
10 integument MP:0010771 9.17 CASR CD40LG FGF23 FN1 GGCX NT5E

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
Magnesium citrate Approved Phase 3 3344-18-1
2
Phylloquinone Approved, Investigational Phase 3 84-80-0 5284607
3
Sodium citrate Approved, Investigational Phase 3 68-04-2 23431961
4
Menadione Approved, Nutraceutical Phase 3 58-27-5 4055
5
Citric acid Approved, Nutraceutical, Vet_approved Phase 3 77-92-9 311
6 Menaquinone Investigational Phase 3 1182-68-9
7 Cathartics Phase 3
8 Vitamins Phase 3
9 Vitamin K 1 Phase 3
10 Vitamin K Phase 3
11 Laxatives Phase 3
12 Trace Elements Phase 3
13 Antifibrinolytic Agents Phase 3
14 Anticoagulants Phase 3
15 Hemostatics Phase 3
16 Gastrointestinal Agents Phase 3
17
Naphthoquinone Phase 3
18 Micronutrients Phase 3
19 Coagulants Phase 3
20 Citrate Phase 3
21 Calcium, Dietary Phase 3
22
Sodium thiosulfate Phase 3
23 Anti-Bacterial Agents Phase 3
24 Anti-Infective Agents Phase 3
25 Antidotes Phase 3
26 Antitubercular Agents Phase 3
27 Antioxidants Phase 3
28 Protective Agents Phase 3
29 Chelating Agents Phase 3
30 Pharmaceutical Solutions Phase 3
31
Calcium Nutraceutical Phase 3 7440-70-2 271
32
Warfarin Approved 81-81-2, 129-06-6 54678486

Interventional clinical trials:

(show all 16)
# Name Status NCT ID Phase Drugs
1 Better Evidence and Translation for Calciphylaxis Recruiting NCT05018221 Phase 3 Vitamin K1;Magnesium citrate;Sodium Thiosulfate;Placebo injection (normal saline);Placebo capsule (Vitamin K1);Placebo tablet (Magnesium citrate)
2 A Phase 3, Randomized, Double-Blind, Placebo-Controlled Study to Assess the Efficacy and Safety of SNF472 When Added to Background Care for the Treatment of Calciphylaxis Active, not recruiting NCT04195906 Phase 3 Experimental: SNF472;Placebo Comparator: Placebo;Experimatenl SNF472 (Open-label)
3 A Phase 3, Intravenous Sodium Thiosulfate for Acute Calciphylaxis Treatment: A Multicenter, Randomized, Double-blind, Placebo-controlled, Clinical Trial Terminated NCT03150420 Phase 3 Sodium Thiosulfate;Placebo-Normal Saline
4 Randomized Double Blind Placebo Controlled Trial of Sodium Thiosulfate for the Treatment of Pain Associated With Calcific Uremic Arteriolopathy Followed by a Single Armed Extension Phase to Prospectively Observe Lesion Progression in the Presence of Sodium Thiosulfate Treatment Terminated NCT02527213 Phase 3 Sodium thiosulfate;Placebo
5 Phase 2 Open Label Single Arm Repeat Dose Study to Assess the Effect of SNF472 on Wound Healing in Uraemic Calciphylaxis Patients Completed NCT02790073 Phase 2 SNF472
6 Evaluation of Efficacy of Lanthanum Carbonate (Fosrenol) in Patients With Calciphylaxis Completed NCT01289626 Phase 1 lanthanum carbonate;Lanthanum for calciphylaxis
7 European Calciphylaxis Registry Network Unknown status NCT02635373
8 National Follow-up of Dialysis Patients With Sodium Thiosufate-treated Calciphylaxis Under Temporary Use Authorization Unknown status NCT03146793 Sodium Thiosulfate
9 Pathophysiology of Martorell Hypertensive Ischemic Leg Ulcer (HYTILU): A Comparison of Martorell HYTILU, Calciphylaxis and Venous Ulcer (Control) Completed NCT01578382
10 Calciphylaxis : Population, Risk Factors, Diagnostic Practice, Therapeutic and Outcome : a Multicentric, Retrospective Cohort Completed NCT02854046
11 Evaluation of Vitamin K Supplementation for Calcific Uremic Arteriolopathy Completed NCT02278692
12 Partners Calciphylaxis Biobank and Patient Registry Recruiting NCT03032835
13 Effects of Human Amniotic Mesenchymal Stem Cells (hAMSCs) on Wound Healing in Uremic Calciphylaxis Patients: an Open Label Single Arm Study Active, not recruiting NCT04592640
14 Efficacy of Rheopheresis as Adjuvant Treatment of Calciphylaxis in Hemodialysis Patients : a Prospective Randomized Controled Single-blind Trial Not yet recruiting NCT04654000
15 Retrospective Study Evaluating the Contribution of Rheopheresis in the Treatment of Uremic Calciphylaxis Not yet recruiting NCT05633121
16 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

Organs/tissues related to Calciphylaxis:

MalaCards : Skin, Kidney, Skeletal Muscle, Bone, Breast, Liver, Heart

Publications for Calciphylaxis

Articles related to Calciphylaxis:

(show top 50) (show all 1459)
# Title Authors PMID Year
1
Erythema nodosum with incidental calciphylaxis secondary to zoledronic acid and denosumab. 62
36184744 2022
2
Mechanistic insights into CKD-MBD-related vascular calcification and its clinical implications. 62
36336124 2022
3
Non-uremic calciphylaxis: case series. 62
36473774 2022
4
Complete resolution of calciphylaxis in a renal transplant patient with Calcifediol. 62
34001663 2022
5
Use of the optimized sodium thiosulfate regimen for the treatment of calciphylaxis in Chinese patients. 62
35634730 2022
6
Calciphylaxis in an adolescent without renal failure. 62
35840314 2022
7
Challenges management in penile calciphylaxis. 62
36131716 2022
8
Calciphylaxis arising following bariatric surgery: A case series. 62
36090194 2022
9
Identifying subcutaneous tissue microcalcification by Fluo-3 AM imaging in cutaneous calciphylaxis. 62
35441735 2022
10
An Intensive Multidisciplinary Approach in Management of Extensive Nonuremic Calciphylaxis of the Bilateral Lower Extremities with Angioinvasive Fungus and Mold. 62
36269818 2022
11
Calciphylaxis and its co-occurrence with connective tissue diseases. 62
36274216 2022
12
Rapidly Progressive and Catastrophic Calciphylaxis After Mechanical Valve Replacement: An Anticoagulation Dilemma. 62
36406917 2022
13
Penile calciphylaxis in patients with end-stage kidney disease undergoing dialysis: Invasive treatment and pain management. 62
34984854 2022
14
Chronic skin ulcer in a maintenance hemodialysis patient: pyoderma gangrenosum or calciphylaxis? 62
35349003 2022
15
Ulcer in a dialysis patient: Calciphylaxis or something simpler. 62
36172333 2022
16
The Role of Iron in Calciphylaxis-A Current Review. 62
36233647 2022
17
Xeroradiography as a Noninvasive Tool in Diagnosing Calciphylaxis. 62
35793077 2022
18
Manifestation of Nonuremic Calciphylaxis in the Extremities: Case Report and Review. 62
34876341 2022
19
Refractory wounds induced by normal-renal calciphylaxis: An under-recognised calcific arteriolopathy. 62
36068658 2022
20
Suggestions for a New Clinical Classification Approach to Panniculitis Based on a Mayo Clinic Experience of 207 Cases. 62
35849324 2022
21
Clinical characteristics and comorbidities of the most common atypical wounds in Northern Finland in 1996-2019: A retrospective registry study. 62
36189416 2022
22
Painless Scrotal Ulcers Become Something Unexpected: A Rare Case of Scrotal Calciphylaxis. 62
36237806 2022
23
Are Opioids Contraindicated for the Palliative Care Patient with Hypotension? 62
35196131 2022
24
Cutaneous vascular calcification. Perieccrine calcification as a diagnostic key for calciphylaxis. 62
35357702 2022
25
Calciphylaxis and Kidney Disease: A Review. 62
35970430 2022
26
Role of myoinositol hexaphosphate (SNF 472) in calciphylaxis: an update. 62
35192233 2022
27
Characterization of Risk Factors for Calciphylaxis in Hemodialysis Patients in the Fraser Health Renal Program - A Matched Case-Control Retrospective Review. 62
36036088 2022
28
Multicentric Study on High-Frequency Ultrasound Characterization of Calcium Deposits in Dermal and Subcutaneous Calciphylaxis and Calcinosis. 62
34755910 2022
29
A Rare Presentation of Penile Calciphylaxis Requiring Partial Penectomy. 62
36158391 2022
30
Local, non-systemic, and minimally invasive therapies for calcinosis cutis: a systematic review. 62
34165603 2022
31
[Multidisciplinary regenerative treatment and mechanisms for rescuing a severe calciphylaxis patient with human amnion-derived mesenchymal stem cells]. 62
35872588 2022
32
Rituximab Used for the Treatment of Nonuremic Calciphylaxis: A Complication of Prolonged Steroid Use in Lupus Nephritis. 62
35795578 2022
33
Calciphylaxis in End-Stage Renal Disease: A Rare Condition With High Mortality. 62
35967146 2022
34
Retinal artery and vein occlusion in calciphylaxis. 62
35372715 2022
35
Nonuremic calciphylaxis manifesting with diffuse dermal angiomatosis. 62
35518276 2022
36
A novel long-term intravenous combined with local treatment with human amnion-derived mesenchymal stem cells for a multidisciplinary rescued uremic calciphylaxis patient and the underlying mechanism. 62
35142858 2022
37
[Uremic calciphylaxis]. 62
35514006 2022
38
Calciphylaxis: utility of skin biopsy within a diagnostic algorithm. 62
36259809 2022
39
[Left atrial appendage occlusion as replacement of coumarin anticoagulants in calciphylaxis]. 62
35819038 2022
40
Calciphylaxis: Part I. Diagnosis and pathology. 62
35114300 2022
41
Management of chronic urinary retention in glanular penile calciphylaxis. 62
35530543 2022
42
Comments on "Ultrasound findings of calciphylaxis". 62
35461600 2022
43
Images - Penile pain in the setting of end-stage renal disease: An unusual anatomic location for calciphylaxis. 62
34941493 2022
44
Penectomy to manage penile gangrene caused by calciphylaxis, a case report. 62
35530551 2022
45
Understanding the zebras of wound care: an overview of atypical wounds. 62
35839157 2022
46
Synergy of sodium thiosulphate treatment and expanded hemodialysis in the management of calciphylaxis? A case report. 62
36153291 2022
47
Calciphylaxis: Treatment and outlook-CME part II. 62
35202773 2022
48
The Relevance of Skin Biopsies in General Internal Medicine: Facts and Myths. 62
35430724 2022
49
Rheopheresis: A narrative review. 62
35389284 2022
50
Hemodialysis Patient with Streptococcal Toxic Shock Syndrome and Penile Necrosis: A Case Report. 62
35650128 2022

Variations for Calciphylaxis

Expression for Calciphylaxis

Search GEO for disease gene expression data for Calciphylaxis.

Pathways for Calciphylaxis

GO Terms for Calciphylaxis

Cellular components related to Calciphylaxis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 platelet alpha granule lumen GO:0031093 9.1 FN1 ALB AHSG

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

# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 10.1 VDR PTH FN1 FGF23 CASR
2 ossification GO:0001503 9.91 MGP CASR AHSG
3 negative regulation of bone mineralization GO:0030502 9.78 FGF23 AHSG
4 phosphate ion homeostasis GO:0055062 9.76 PTH FGF23
5 acute-phase response GO:0006953 9.73 FN1 EPO AHSG
6 cellular response to vitamin D GO:0071305 9.67 CASR FGF23
7 response to fibroblast growth factor GO:0071774 9.62 PTH CASR
8 calcium ion homeostasis GO:0055074 9.55 PTH NT5E FGF23
9 positive regulation of vitamin D 24-hydroxylase activity GO:0010980 9.26 VDR FGF23
10 regulation of bone mineralization GO:0030500 9.02 MGP FGF23 AHSG

Sources for Calciphylaxis

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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