Disease Directory VCP Disease
Neuromuscular

VCP Disease

Also known as: IBMPFD, inclusion body myopathy with Paget disease and frontotemporal dementia, VCP-related multisystem proteinopathy

Prevalence

Less than 1 in 1,000,000

Onset

Adulthood (typically 4th–5th decade)

Type

Autosomal dominant

Gene

VCP

About VCP Disease

VCP Disease (also called Multisystem Proteinopathy or IBMPFD) is caused by heterozygous gain-of-function mutations in the VCP gene encoding valosin-containing protein, a ubiquitous AAA+ ATPase involved in protein homeostasis, autophagy, and DNA repair. It is a multisystem disorder that variably involves skeletal muscle (inclusion body myopathy with rimmed vacuoles), bone (Paget disease of bone), and brain (frontotemporal dementia). ALS-like motor neuron involvement occurs in a subset.

Common Clinical Features

Progressive proximal and distal muscle weakness with rimmed vacuoles on biopsy Paget disease of bone (bone pain, fractures, elevated alkaline phosphatase) Frontotemporal dementia or behavioural change Scapular winging Respiratory muscle weakness in advanced muscle disease ALS-like features in some patients Cardiomyopathy (less common)

Clinical Trial Eligibility Tips

What to know before applying to VCP Disease trials.

Genetic confirmation of a pathogenic VCP variant is required; the most common hotspot mutations (R155H, R191Q, R155C) account for the majority of cases — targeted sequencing or gene panel is appropriate

Multisystem involvement should be documented across all three domains (muscle, bone, CNS) even if subclinical — bone scan and neuropsychological testing results may be required at screening

Trials targeting the VCP/proteasome pathway may have eligibility criteria excluding patients with advanced dementia; cognitive screening scores (MoCA, MMSE) should be obtained early

Patient Resources

Patient Organization

Myositis Association / Myotonic Dystrophy Foundation

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Orphanet

European reference resource for rare diseases (ORPHA:329478)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

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Find recruiting VCP Disease trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for VCP Disease. Updated daily.

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