Restrictive Lung Disease Flashcards
Describe Restrictive Pulmonary Disease
-Inability to completely fill their lungs with air
-Their lungs are “restricted” from fully expanding.
-Characterized by reduced lung volumes.
-Unlike obstructive lung disease (COPD, Asthma), restrictive disease are associated with a decreased total lung capacity.
Restrictive Pulmonary Disease can be divided into what two groups?
- Intrinsic: diseases of the lung parenchyma, inflammation or scarring of lung tissue. (Idiopathic fibrotic disease, Pneumoconioses, and Sarcoidosis)
- Extrinsic: Extra-pulmonary diseases involving the chest wall, pleura, and respiratory muscles. (Obesity, Myasthenia gravis, ALS, Kyphoscoliosis)
A patient presents with progressively worsening shortness of breath, dry hacking cough, fatigue and crackles on exam. No fever or leukocytosis. PFT results are as follows:
*Reduced FVC, but normal or elevated FEV1/FVC ratio
*Reduced DLCO
*Impaired 6 min walk
What is the most likely diagnosis?
Idiopathic Fibrosing Interstitial Pneumonia
What radiographic findings would you expect to see on the patient with Idiopathic Fibrosing Interstitial Pneumonia? (CXR and CT)
- CXR: Most common findings- increase in reticular markings (IPF, CHF)
- CT shows diffuse patchy fibrosis with pleural based honeycombing.
Treatment Options for Idiopathic Fibrosing Interstitial Pneumonia?
Supportive Care: -Supplemental home oxygen -Vaccinations (influenza, pneumococcal) -Outpatient Pulmonary Rehab Programs Medications: -Nintedanib: a tyrosine kinase inhibitor. -Pirfenidone(Esbriet): an anti-fibrotic drug Surgery: -Lung transplantation -Age < 65 -Free of substance abuse (smoking, drugs) -Acceptable BMI range of 20-29
What is Pneumoconiosis?
- “Occupational lung disease”
- Group of interstitial lung diseases caused by the inhalation and deposition of inorganic particles and mineral dust with subsequent reaction of the lung.
What is Clinically important pneumoconiosis?
- Coal worker’s pneumoconiosis
- Silicosis
- Asbestosis
What is Coal Worker’s Pneumoconioses?
- Also called “Black Lung Disease”
- Milder form of the disease is known as Anthracosis.
- Caused by prolonged exposure to coal dust, which is inert and cannot be removed by the body.
- Leads to inflammation, fibrosis, and sometimes necrosis
What is Silicosis?
-Spectrum of pulmonary diseases caused by inhalation of crystalline silica.
-Occupations: mining, masonry, glass manufacturing, foundry work, and sandblasting.
What is Asbestos?
Group of naturally occurring fibrous composed of hydrated magnesium silicates used for variety of construction and insulating purposes.
What is Asbestosis?
Pneumoconiosis caused by inhalational asbestos fibers
Clinical presentation of Asbestosis?
- Usually asymptomatic for at least 20-30 years after initial exposure
- Dyspnea on exertion
- Cough
- Weight loss
What is Sarcoidosis?
-Multisystem granulomatous disorder of unknown etiology.
-Lungs, lymph nodes, eyes, skin, liver, spleen, heart, nervous system, but approximately 90% of patients have lung involvement.
-Characterized pathologically by the presence of non-caseating granuloma.
-More common in young black women and Northern European whites.
Clinical Presentation of Sarcoidosis?
-Cough (dry, hacking)
-Progressive, worsening dyspnea
-Atypical chest discomfort
-Fever/night sweats
-Weight loss
Evaluation for Sarcoidosis?
-Serum blood tests, ACE levels, ESR are non-diagnostic.
-Radiographic Imaging:
CXR: Bilateral hilar adenopathy
CT: Right paratracheal lymphadenopathy along with bilateral diffuse reticular infiltrates
