Partners Calciphylaxis Biobank

Massachusetts General Hospital300 enrolled

Overview

Calciphylaxis, a vascular calcification disorder, is a rare and serious disorder characterized by calcification of dermal arterioles. There are significant gaps in the understanding of the pathophysiology and risk factors for calciphylaxis. At present, there is no effective treatment. Uncertain pathobiology, rare incidence and lack of collaborative approach have been some of the major limiting factors towards treating calciphylaxis. The Partners Calciphylaxis Biorepository (PCB) aims to address these gaps within calciphylaxis research by utilizing existing and, when necessary, developing new infrastructure to support the consent of patients and the collection of dedicated samples for a calciphylaxis repository.

Study Type

OBSERVATIONAL

Enrollment

300

Conditions

Calciphylaxis Calcific Uremic Arteriolopathy End Stage Renal Disease

Eligibility

Sex: ALLMin age: 18 Years

Medical Language ↔ Plain English

Inclusion Criteria: 1. Male or female ≥18 years old 2. Skin lesions consistent with calciphylaxis diagnosis as determined by the treating clinician 3. Informed of the investigational nature of the study and sign written Informed consent OR are eligible for surrogate consent process based on impaired decision making Exclusion Criteria: 1. Patients \<18 years of age 2. Prisoners

Outcomes

Primary Outcomes

Number of patients with improvement in calciphylaxis skin lesions

Skin lesions will be assessed by investigators at baseline (entry into study) and at 6 months to determine whether there is improvement. The investigators will determine clinical, laboratory and genetic predictors of this outcome in calciphylaxis patients.

Time frame: 6 months

Secondary Outcomes

Number of patients with >30% improvement in pain severity

Pain severity will be assessed by investigators at baseline (entry into study) and at 4 weeks to determine whether there is improvement. Pain is measured using Wong-Baker Faces pain rating scale. The investigators will examine clinical, laboratory and genetic predictors of this outcome in calciphylaxis patients.

Time frame: 4weeks

Time to death in patients with calciphylaxis

Time to death is defined as time from the diagnosis of calciphylaxis to death (or to the end of follow up). The investigators will examine clinical, laboratory and genetic predictors of this outcome in calciphylaxis patients.

Time frame: Up to 5 years