Details of Disease

General Information of Disease (ID: DIS2Y594)

• Disease Name: Calciphylaxis
• Synonyms: idiopathic calciphylaxis
• Disease Class: EB90: Calcific uremic arteriolopathy
• Definition: 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 itto die (necrosis). The most obvious and frequent symptom of calciphylaxis is damage to the skin, as ulcers can developand 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 thana 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.
• Disease Hierarchy:
  DIS6SVEE: Syndromic disease
  DISQP4OR: Calcinosis
  DISVS67S: Vascular disease
  DIS2Y594: Calciphylaxis
• ICD Code: ICD-11: ICD-11: EB90.42
• Disease Identifiers:
  MONDO ID: MONDO_0017215
  MESH ID: D002115
  UMLS CUI: C0006666
  MedGen ID: 2404
  Orphanet ID: 280062
  SNOMED CT ID: 237900002

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 1 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Sodium thiosulfate	DMYBCBX	Approved	Small molecular drug	[1]

This Disease is Treated as An Indication in 1 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
SNF472	DMOQKAD	Phase 3	Small molecular drug	[2]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 2 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
GARS1	OT5B6R9Y	Strong	Biomarker	[3]
MGP	OTZWU3FU	Strong	Biomarker	[4]

References

• [1] Sodium thiosulfate FDA Label
• [2] ClinicalTrials.gov (NCT04195906) Phase 3 Study of SNF472 for Calciphylaxis (Calciphyx). U.S. National Institutes of Health.
• [3] Increased Bone Morphogenetic Protein Signaling in the Cutaneous Vasculature of Patients with Calciphylaxis.Am J Nephrol. 2017;46(5):429-438. doi: 10.1159/000484418. Epub 2017 Nov 9.
• [4] Warfarin in nonvalvular atrial fibrillation-Time for a change?.Semin Dial. 2019 Nov;32(6):520-526. doi: 10.1111/sdi.12829. Epub 2019 Jun 17.