Interstitial Lung Disease

(Do you fancy this bird?)

An organised approach to Interstitial lung diseases:

Systemic Diseases: the most common of these are sarcoidosis and the Collagen Vascular Diseases - with scleroderma being the prototype. Also consider mixed connective tissue diseases. ILD is not as commonly in SLE and Rheumatoid arthritis.

Here is a good review article on sarcoidosis.

Exposures: these are classically divided up into organic and inorganic.

1. Organic: aka hypersensitivity pneumonitis. Think about Bird Fancier's Lung (proteins in bird excrement), Cheese Washers Lung, Farmer's Lung, and many many more.


2. Inorganic: aka pneumoconiosis. This would include exposure to dust from asbestos, silica, and coal.
   

Drugs: Exposure to alkylating chemotherapeutic agents (eg bleomycin), and commonly used meds like amiodarone, methotrexate, nitrofurantoin, and sulfa drugs.

Idiopathic: This is a strange category with bizarre acronyms. It doesn't help that there are new and old acronyms for some of the pathologies, but all are still commonly used. Here's a sample.

• Usual Interstitial Pneumonia (UIP), also known as Interstitial Pulmonary Fibrosis (IPF). This is the most common of the idiopathic interstitial lung diseases. Here is a good review article.
• Acute Interstitial Pneumonia (AIP) also known as the Hamman-Rich Syndrome. Just like UIP, but more rapid and severe.
• Cryptogenic Organising Pneumonia (COP), but if the etiology is known, it will be called Bronchiolitis Obliterans with Organizing Pneumonia (BOOP). Lots of granulation tissue in the small airways here.
• Desquamative Interstitial Pneumonia (DIP). Many macrophages in alveoli. Common in middle-aged smokers. A milder version of this may be referred to as Respiratory Bronchilitis-Associated Interstitial Lung Disease (RBILD).
• Others: Lymphocytic Interstitail Pneumonia (LIP), Nonspecific Interstitial Pneumonia (NSIP).
  

Other Rare Causes: Alveolar hemmorhage, Pulmonary Infiltrates with Eosinophilia (PIE), Pulmonary Alveolar Proteinosis (PAP), Lymphangioleiomyomatosis (LAM).

Mimickers of ILD: Congestive heart failure, certain infections like Pneumocystis Carini Pneumonia, and lymphangitic carcinomatosis can cause interstitial pattersns on chest X-rays.

This cheese looks dirty....it may need a good washing.