| Idiopathic Pulmonary Fibrosis
| Diffuse Parenchymal Lung disease
| Asbestos Related Lung disease
| Coal Worker's Pneumoconiosis
| Farmer's Lung
| Pulmonary Alveolar Proteinosis
| Cryptogenic Organising Pneumonia (COP-BOOP)
| Extrinsic Allergic alveolitis (Hypersensitivity)
| Cor Pulmonale
|Chest X Ray Interpretation
Pulmonary Alveolar Proteinosis is a rare cause of cyanosis and finger clubbing.
- It is a rare lung disorder
- It is a potentially treatable disease
- Impaired processing of surfactant which accumulates in the alveoli of proteinaceous material rich in surfactant protein and its component
- Defective GM-CSF signalling
- Abnormal accumulation of surfactant -derived lipoprotein compounds within the alveoli of the lung.
- Primary PAP : most typical and the most common form.
- Secondary PAP : possible to identify a specific factor that actually caused the condition, such as leukaemia or lung cancer, dust or fume inhalation, infections
- Congenital PAP:extremely rare and is caused by a genetic disorder
- Presents with progressive dyspnoea on exertion
- Progressive fatigue, weight loss, and low-grade fever
- Fine end-inspiratory crackles and clubbing are seen
- Very rarely cyanosis and clubbing
- Serum lactate dehydrogenase (LDH) level is usually elevated
- CXR - bilateral alveolar shadowing
- HRCT scan - ground glass appearance, Crazy-paving appearance in a geographic distribution is a characteristic feature of this disease visible
- Bronchoalveolar lavage - large foamy macrophages and a milky appearance
- Lung biopsy: typically shows partial or complete filling of alveoli with periodic-acid-Schiff-positive granular and eosinophilic material in preserved alveolar architecture
- Analysis shows PAS +ve staining
- Antibodies to granulocyte-macrophage colony-stimulating factor are found
- Patients with minimal symptoms are managed conservatively, whereas patients with hypoxemia require a more aggressive approach.
- Whole-lung lavage is the most widely accepted therapy for symptomatic pulmonary alveolar proteinosis. It most often results in a dramatic response.
- Correction of GM-CSF deficiency with exogenous GM-CSF is an alternative therapy
- Smoking cessation is important. Most resolve. Superinfection can occur.