The Rare Disease Database

The most comprehensive source of rare disease information.

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Top 10 rare pulmonology diseases

Measured by: prevalence

DiseaseEst US PrevalenceInheritanceAge of onset
Sarcoidosis 66,000-158,600
Chronic thromboembolic pulmonary hypertension20,970-82,000
Granulomatosis with Polyangiitis (Wegener's)9,200-30,000
Granulomatosis with polyangiitis (Churg-Strauss)3,630-8,000
Idiopathic pulmonary fibrosis (IPF)62,000-140,100
Behcet's disease9,000-17,200
Alpha-1 Antitrypsin Deficiency60,000-100,000
Cystic fibrosis30,000-35,000
Pulmonary arterial hypertension (PAH)8,900-52,800
Idiopathic Interstitial pneumonias60,000-125,000
Tuberculosis8,580-34,970
Pulmonary Alveolar Proteinosis (PAP) Syndrome
Lymphangioleiomyomatosis (LAM)
Birt-Hogg-Dubé syndrome (BHD)
Pulmonary Langerhans cell histiocytosis (PLCH)
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)
Pulmonary alveolar microlithiasis (PAM)
Lymphangiomatosis or generalized lymphatic anomaly (GLA-now the preferred name)