List of rare lung diseases

You will find below the list of the rare lung diseases recorded in SIOLD registries:

Idiopathic interstitial pneumonias 

  • idiopathic pulmonary fibrosis
  • desquamative interstitial pneumonia (DIP)
  • respiratory bronchiolitis interstitial lung disease (RBILD)
  • acute interstitial pneumonia (AIP)
  • nonspecific interstitial pneumonia (NSIP)
  • cryptogenic organizing pneumonia (COP = idiopathic BOOP)
  • secondary organizing pneumonia (BOOP)
  • lymphoid interstitial pneumonia (LIP)
  • idiopathic interstitial pneumonia : unspecified

Hypereosinophilic lung diseases

  • chronic idiopathic eosinophilic pneumonia
  • acute idiopathic eosinophilic pneumonia
  • idiopathic hypereosinophilic syndrome with pulmonary manifestations
  • hypereosinophilic lung disease: other (specify)

Allergic bronchopulmonary aspergillosis (ABPA)

Pulmonary vasculitis

  • Wegener's granulomatosis
  • microscopic polyangiitis
  • Churg-Strauss syndrome
  • pulmonary vasculitis : unspecified

Alveolar hemorrhage syndromes

  • Goodpasture syndrome
  • idiopathic pulmonary hemosiderosis
  • alveolar hemorrhage syndrome of undetermined origin
  • alveolar hemorrhage syndrome of determined origin

Pulmonary lymphangioleiomyomatosis (LAM)

  • Sporadic pulmonary lymphangioleiomyomatosis (S-LAM)
  • Pulmonary lymphangioleiomyomatosis in tuberous sclerosis (TSC-LAM)

Alveolar proteinosis

Pulmonary amyloidosis

Pulmonary langerhans cell histiocytosis (histiocytosis x)

Primary pulmonary lymphoma

Primary ciliary dyskinesia (without or with situs inversus)

Rare cause of hypersensitivity pneumonitis (all causes other than farmer's lung disease and pigeon breeder's lung disease)

Pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia (HHT)

Pulmonary manifestations of gastro-intestinal disorders

  • pulmonary manifestations in inflammatory bowel diseases
  • severe hepatopulmonary syndrome (pa02 < 55 mmHg)

Interstitial lung disease in connective tissue diseases

  • interstitial lung disease in systemic sclerosis
  • interstitial lung disease in rheumatoid arthritis
  • interstitial lung disease in idiopathic inflammatory myopathies (polymyositis, dermatomyositis, anti-synthetase syndrome)
  • interstitial lung disease in Sjögren syndrome
  • interstitial lung disease in mixed connective tissue disease (MCTD)
  • interstitial lung disease in overlap syndromes
  • interstitial lung disease in undifferentiated connective tissue disease

Bronchiolitis obliterans (in non-transplanted patients)



Chronic beryllium disease

Waterproofing spray pneumonitis

Combined pulmonary fibrosis and emphysema

  • combined pulmonary fibrosis and emphysema without associated connective tissue disease
  • combined pulmonary fibrosis and emphysema with connective tissue disease

Alpha-1-antitrypsin deficiency emphysema

Other rare diseases considered of interest (but not mentioned above)

 Last updated on 22/06/2018 at 09:52