Disease Directory Chronic Granulomatous Disease
Immune

Chronic Granulomatous Disease

Also known as: CGD, NADPH oxidase deficiency, chronic granulomatosis, X-linked CGD

Prevalence

1 in 200,000-250,000

Onset

Infancy to early childhood

Type

Primary phagocyte immunodeficiency

Gene

CYBB, NCF1, NCF2, CYBA

About Chronic Granulomatous Disease

Chronic Granulomatous Disease is caused by mutations in components of the NADPH oxidase complex, resulting in failure of phagocytes to generate the oxidative burst needed to kill catalase-positive bacteria and fungi, leaving patients susceptible to a characteristic spectrum of life-threatening infections and granulomatous inflammation. The X-linked form caused by CYBB mutations accounts for approximately 65-70% of cases and generally presents earlier and more severely than autosomal recessive variants. Allogeneic hematopoietic stem cell transplantation is the standard curative approach, and gene therapy with lentiviral vectors has demonstrated promise in trials for patients without suitable donors.

Common Clinical Features

Recurrent deep-seated infections with catalase-positive organisms (Staphylococcus, Aspergillus) Liver, lymph node, and lung abscesses Granulomatous obstruction of GI or urinary tract Inflammatory bowel disease-like colitis Lymphadenopathy and hepatosplenomegaly Perianal abscesses and fistulae Abnormal dihydrorhodamine (DHR) flow cytometry

Clinical Trial Eligibility Tips

What to know before applying to Chronic Granulomatous Disease trials.

DHR flow cytometry or nitroblue tetrazolium (NBT) test confirming NADPH oxidase deficiency is required for all trials; CYBB mutation confirmation is additionally required for X-linked specific gene therapy trials

Active invasive fungal infection (Aspergillus, Candida) typically excludes from transplant conditioning studies until infection is controlled; provide current antifungal therapy details

Prophylactic antibiotic and antifungal regimen history (itraconazole, trimethoprim-sulfamethoxazole) should be documented, as protocol-specified prophylaxis changes during trial participation are common

Patient Resources

Patient Organization

CGD Association

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Natural History Registry

USIDNET Registry

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Orphanet

European reference resource for rare diseases (ORPHA:379)

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NORD

National Organization for Rare Disorders

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Find recruiting Chronic Granulomatous Disease trials

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

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