List of 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)
Asbestosis
Sarcoidosis
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)Source----------------------------------------------------
Note: The first one listed is IPF (idiopathic pulmonary fibrosis)—As you may recall, United Therapeutics bought SM04646, a small molecule Wnt pathway inhibitor specifically designed for inhalation, for IPF (idiopathic pulmonary fibrosis), back in 2018. Deal terms included $10 million up-front, plus up to $340 million in developmental milestones and up to low double-digit royalties.
Now, who in the world will United Therapeutics want to use for the inhalation system and technology for delivering this very promising therapy to the lungs? My guess is the same company they partnered with for the Treprostinil inhalation system and technology who also just happened to change their pipeline focus to orphan lung diseases. 😎