Type I Pneumocyte
Flattened region of gaseos transport/diffusion, which is vulnerable to injury.
Type II Pneumocyte
Site of surfactant synthesis.
Can proliferate and re-form alveolar epithelial surface.
Non-Pharmacologic Management of ILD
Manage co-morbidities (GERD, OSA (obstructive sleep apnea), CHF)
1. Immuno-modulatory therapies
2. Anti-fibrotic therapies (IPF only)
A multisystem granulomatous disorder of unknown etiology.
Affects individuals worldwide.
Characterized histologically by the presence of non-caseating granulomas in the affected organs.
~50% is detected incidentally on CXR.
Organ Involvement of Sarcoidosis
Lungs - 95%
Skin - 16%
Lymph nodes - 15%
Eye - 12%
Liver - 12%
Erythema Nodosum - 8%
Spleen - 7%
Sarcoidosis - Histology
Granulomatous (non-caseating) inflammation.
Indications for Treatment - Sarcoidosis
60-80% of asymptomatic patients have spontaneous remission.
Consider treatment if it is progressive symptomatic pulmonary, neurologic, ocular, hypercalcemia/renal, or cardiac
Treatment - Pulmonary Sarcoidosis
Glucocorticoids (First line) - prednisone
Signs of Restrictive Spirometry
FEV1/FVC > 70%
Low FVC (<80%)
Low FEV1 (<80%)
Idiopathic Pulmonary Fibrosis (IPF)
The most common of the idiopathic ILD's.
Chronic progressive parenchymal lung disease of unclear origin.
Median survival is 3-5 years from diagnosis.
Usually affects those aged >50
2 therapies now available; pirfenidone and nintedanib