Restrictive lung diseases Flashcards
Definition of insterstitial lung disease
Hetergenous group of disorders with a combo of inflammation or scarring.
General categories of causes of interstitial lung disease
idiopathic (sarcoidosis or idiopathic interstitial pneumonias), 1. autoimmune, 2. Exposure to inorganic dusts (typically occupational dusts such as silica or asbestos) 3. Exposure to organic molecules that result in hypersensitivity pneumonitis 4. Drug effect
Six classes of idiopathic interstitial pneumonias
Idiopathic pulmonary fibrosis, idiopathic nonspecific interstitial pneumonia, Respiratory bronchiolitis-interstitial lung disease, desquamative interstitial pneumonia, cryptogenic organized pneumonia, acute interstitial pneumonia
Describe approach to diagnosis of ILD
clinical (history and physical) look for evidence of known causes (ie. Autoimmune). Radiographic patterns on high resolution CT. Pathologic pattern from lung biopsy. Etiology alone is not enough, must also know pattern.
Presentation of most ILD
Dyspnea on exertion. Nonproductive cough is common. Rarely, the disease may be sudden in onset. Decreased DLCO
PFTs of most ILDs
Restrictive pattern (decreased lung volume) with reduced diffusing capacity
Physical exam of ILD
end-inspiratory crackles (velcro like), digital clubbing if bad, joint/skin exam to identify collagen vascular diseases
Treatment of ILD
remove exposure, immunosuppressive therapy (steroids), oxygen, transplant, research trial for IPF
Idiopathic pulmonary fibrosis
Idiopathic scarring lung disease with pattern of injury of usual insterstitial pneumonia (UIP).
Idiopathic pulmonary fibrosis physical exam and survival
Cough, DOE, fatigue, basilar predominant velcro crackles, digital clubbing if bad. Survival is 2-3 yrs.
IPF associations
tobacco use, disease of older patients
IPF imaging
Peripheral and basilar predominant reticulation, traction bronchiectasis, honeycombing and ground glass infiltrate
IPF pathology
Heterogenous fibrosis with fibroblast foci and juxtaposition of fibrotic and normal lung
IPF therapy
none
Non specific interstitial pneumonia
Presents similar to other ILD. May be idiopathic, collagen vascular disease related (RA), fibrotic, or drug related. More likely to be female and younger, with better prognosis than IPF
Nonspecific intersitial pneumonia therapy
anti-inflammatory therapy
Nonspecific intersitial pneumonia radiograph
basilar predominant reticular abnormality with volume loss, bronchiectasis, ground glass, no honeycombing
Non specific interstitial pneumonia pathology
homogenous fibrosis with varying inflammation and fibrosis
Smoking related interstitial lung diseases
Respiratory bronchiolitis-ILD, Desquamative interstitial pneumonia (DIP) and pulmonary langerhans cell histiocytosis. RBILD and DIP are both on a continuum. Langerhans is seen in young smokers and it results in cysts with high risk for pneumothorax. Treatment for all is smoking cessation
Organizing pneumonia
noninfectious pneumonia with alveolar filling. May be idiopathic, secondary to cardio disease, drugs, aspiration of stomach acid.
Organizing pneumonia imaging and path
imaging shows ground glass and consolidation. Path shows organizing pneumonia pattern with alveolar filling
Organizing pneumonia treatment
steroids- requires 6-12 months, and can have relapse
Eosinophilic pneumonia
Acute often results in acute respiratory failure, mimics ARDS and shows eosinophils. Chronic is associated with asthma. Both are treated with steroids
Lymphangioleiomyomatosis (LAM)
Cystic lung disease in young women- Has an obstructive pattern on PFTs. Treated with Sirolimus (mTOR inhibitor)
