Diffuse panbronchiolitis

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Diffuse panbronchiolitis

ORPHA:171700Disease

What it is

Diffuse panbronchiolitis is a rare chronic inflammatory obstructive pulmonary disease primarily affecting the respiratory bronchioles throughout both lungs and inducing sinobronchial infection. Onset occurs in the second to fifth decade of life and manifests by chronic cough, exertional dyspnea, and sputum production. Most patients also have chronic paranasal sinusitis

Key facts

Prevalence
<1 / 1 000 000 (Europe)
Age of onset
Adult
Inheritance
Multigenic/multifactorial
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Genes

MUC5BMajor susceptibility factor
MUCL3Major susceptibility factor

ICD-10 codes

J44.8filed under a broader ICD-10 category — shared with 2 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 8526MEDDRA 10062952MESH C536174MONDO 0011490OMIM 604809UMLS C0878555

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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