Disease Directory Late-Onset Pompe Disease
Metabolic

Late-Onset Pompe Disease

Also known as: LOPD, late-onset glycogen storage disease type II, adult-onset acid maltase deficiency, GAA deficiency

Prevalence

1-5 per 100,000 (Orphanet)

Onset

Childhood, Adult

Type

Autosomal recessive genetic

Gene

GAA

About Late-Onset Pompe Disease

Late-onset Pompe disease (LOPD) is caused by partial deficiency of acid alpha-glucosidase (GAA), distinguishing it from the more severe infantile-onset form. Glycogen accumulates progressively in skeletal muscle and respiratory muscle, causing limb-girdle weakness and respiratory failure that worsens over years to decades. Alglucosidase alfa (Myozyme/Lumizyme) was the first approved ERT; avalglucosidase alfa (Nexviazyme) and cipaglucosidase alfa with miglustat (Pombiliti+Opfolda) represent next-generation options.

Common Clinical Features

Proximal muscle weakness Respiratory insufficiency Exercise intolerance Lordosis and scoliosis Sleep-disordered breathing Wheelchair dependence Dysphagia

Clinical Trial Eligibility Tips

What to know before applying to Late-Onset Pompe Disease trials.

GAA enzyme activity and GAA genotype are required eligibility confirmations — document residual enzyme activity level

Forced vital capacity (FVC) percent predicted is a primary eligibility criterion — many trials require FVC above a minimum threshold

Document current ERT (alglucosidase alfa vs. avalglucosidase alfa) and anti-GAA antibody titer — antibody-positive patients may be excluded or enrolled in immune tolerance induction sub-studies

Six-minute walk test (6MWT) distance is a key baseline and outcome measure

Patient Resources

Patient Organization

Acid Maltase Deficiency Association

Visit website ↗

Natural History Registry

Pompe Registry

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:308)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Late-Onset Pompe Disease trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Late-Onset Pompe Disease. Updated daily.

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