Restrictive Lung Disease part 1: intro and fibrosing diseases Flashcards
Restrictive lung disorders occur in what two general conditions?
- Chronic interstitial fibrosis
2. Chest wall disorders
How do we characterize chronic interstitial pulmonary disease?
Inflammation and fibrosis of the pulmonary interstitium
What are the 5 major categories of chronic interstitial lung disease?
Fibrosis, granulomatous, eosinophilic, smoking related and other.
What are the 3 specific diseases that fall under the category of pneumonia she wants us to know?
Usual interstitial pneumonia, non specific interstitial pneumonia, and crypto genie organizing pneumonia
What is another name for usual interstitial pneumonia and how do we characterize it?
Idiopathic pulmonary fibrosis. It is characterized by progressive interstitial pulmonary fibrosis and respiratory failure.
What are the top 3 risk factors for IPF?
Smoking, genetic mutations involving telomerase, surfactant and MUC5B, and increasing age.
3 hallmark features of IPF on CT or biopsy?
Patchy fibrosis, fibroblast foci, and peripheral and subpleural honeycombing
What is a hallmark symptom of IPF and what will be find on physical exam?
Dyspnea. When we listen to the lungs we will hear crackles in the lungs, almost like pulling Velcro apart.
What are some differences between NSIP and UIP and really any other interstitial lung disease?
No fibroblastic foci, honeycombing, hyaline membranes or granulomas are present. NSIP has better prognosis than UIP as well. Really this is just either interstitial inflammation or interstitial fibrosis.
How does the patient present with Cryptogenic organizing pneumonia?
Dyspnea, cough, and pneumonia like consolidation
What is also different about COP than IPF?
No interstitial fibrosis or honeycombing lung. The fibrosis is found in the alveolar ducts and alveoli themselves called Masson bodies.
What are three autoimmune/systemic diseases that can manifest as ILD?
RA, sclerosis, and lupus
