Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
22 Cards in this Set
- Front
- Back
|
Interstitial lung disease: History
|
Cough is a nearly universal symptom,
Wheezing suggests bronchiolitis or other concommitant airway pathology -Usually no wheezing heard Progressive or exertional dyspnea is a common manifestation of any diffuse or severe lung disease Hemoptysis suggests vasculitis Interstitial lung disease can result from occupational exposures |
|
|
Interstitial lung disease: Physical exam
|
Crackles (rales) suggest interstitial lung disease or fibrosis;
-Inspiratory squeaks Reduced volumes, basilar dullness Pleural rub suggests pleurisy or pleuritis Digital Clubbing is uncommon; and non-specific Hypoxia or cor pulmonale is common with advanced disease of any type; |
|
|
Interstitial lung disease: Imaging and lung function tests
|
Chest CT is the best imaging modality and is almost always done
Lung Function tests including arterial blood gas are most useful in measuring severity and estimating prognosis rather than making diagnoses -Diffuse lung disease or pleural disease usually results in pulmonary restriction (reduced lung volumes) -Airway involvement resulting in airflow obstruction is less common and suggests bronchiolitis or bronchiectasis |
|
|
Interstitial lung disease: pathologic diagnosis
|
ILD can be diagnosed without surgical biopsy but precise classification usually requires a surgical biopsy
ILD often occur in association with connective tissue diseases -Scleroderma -Mixed-connective tissue disease -Rheumatoid arthritis -Polymyositis -Lupus (SLE) -Sjogren’s Disease (Sicca syndrome) |
|
|
Pathophysiology of restrictive lung disease
|
Inflammation and fibrosis
-Decrease gas diffusion capacity Compliance of lung decreases |
|
|
Pulmonary function tests: results in ILD
|
Spirometry
-↓ Forced Expiratory Volume in 1 second (FEV1) -↓ Forced Vital Capacity (FVC) -↑ or normal FEV1/FVC Ratio Lung volume measurements -↓ Functional Residual Capacity (FRC) or Thoracic Gas Volume (TGV) -↓ Total Lung Capacity (TLC) Gas Diffusion measurements -↓ DLCO |
|
|
Pulmonary fibrosis, idiopathic pneumonias
|
Group of similar interstitial pneumonia with different histology subtypes
|
|
|
Pathogenesis of pulmonary fibrosis
|
Exposure/injury leads to inflammation
Body doesn't shut off inflammation when insult is gone Also has abnormal remodeling and repair response Inflammation leads to collagen deposition and fibroblastic focus |
|
|
Idiopathic pulmonary fibrosis: prevalence, demographics, symptoms, signs
|
Prevalence estimate = 13-20 cases/100,000
Demographics -Age of onset between 50-70 years old -males > females -More common in smokers Symptoms -Progressive dyspnea, cough for months to years (mean = 2 years) Signs -Velcro rales on exam (90%), cyanosis |
|
|
Idiopathic pulmonary fibrosis: diagnosis
|
Gold Standard is Lung Biopsy
Diagnosis often made on clinical impression and imaging Biopsy especially indicated for younger patients that do not present with class findings |
|
|
Idiopathic pulmonary fibrosis: prognosis and treatment
|
Poor prognosis
-50% 5 year survival Lung transplant: -Probably the best therapeutic option -Many patients are too old (> 65 years) -Many have co-existing heart disease that makes them poor surgical candidates -5 year survival for lung allografts is 60-70% |
|
|
Non-specific interstitial pneumonia: clinical, findings, prognosis
|
Dyspnea, cough
Bilateral infiltrates or consolidation without much honeycombing Occurs in association with hypersensitivity, resolving infection, collagen vascular disease Prognosis better than UIP except for “fibrosing” NSIP |
|
|
Desquamative Interstitial Pneumonitis and Respiratory Bronchiolitis Associated ILD (DIP/RBILD)
|
Usually associated with cigarette smoking
Cough and dyspnea PFTs can show obstruction or restriction Does not improve if cigarette smoking continues |
|
|
Cryptogenic organizing pneumonia
|
Formerly BOOP, Bronchiolitis Obliterans Organizing Pneumonia
Airspace organizing pneumonia occurs in response to infection or other inflammation Expected to respond to corticosteroids |
|
|
Sarcoidosis: cause, demographics, symptoms, extrapulmonary symptoms
|
Idiopathic = Cause Unknown
Demographics -20-40 years old -females > males -African Americans > Caucasians (8:1 in US) Symptoms -1/3 No symptoms (Abnormal Chest x-ray only) -2/3 Dyspnea and dry cough Extrapulmonary symptoms -10% diagnosed from extrapulmonary sites |
|
|
Organs involved in sarcoidosis
|
90% Lung or Abnormal Chest X-ray
75% Lymphadenopathy 30% Skin or erythema nodosum 25% Ocular mostly anterior uveitis 20% Liver and/or Spleen 17% Hypercalcemia (? mechanism) 5% Cardiac (high mortality, sudden death) 5% Central Nervous System < 5% all others |
|
|
Treatment in sarcoidosis
|
Asymptomatic patients
-Observe Abnormal PFTs with no symptoms -Observe and follow (PFT) Pulmonary Symptoms Severe restriction on PFTs Fibrotic Chest X-ray Persistent findings after 6 months observation Ocular, Neurologic, Cardiac, Hypercalcemia -Treat with Corticosteroids, Methotrexate, infliximab |
|
|
Silicosis
|
Results from inflammation or fibrosis as a reaction to inhaled crystalline silica
Variable presentation: asymptomic with radiographic changes to progressive massive fibrosis and respiratory failure “Silicotic nodule” Birefringent particles seen on polarized microscopy Many occupations exposed |
|
|
Asbestosis
|
Pulmonary interstitial fibrosis from inhaled asbestos fibers
Variable presentation Occupations: mining and manufacturing of asbestos products, insulators, shipyard workers, pipefitters (ships, power plants, refineries, seamen, steel workers) Mesothelioma, pleural effusion |
|
|
Coal workers pneumoconiosis
|
Less common with industrial hygeine practices
History is important Variable presentation like for silicosis “coal dust macule” |
|
|
IPF, scleroderma, sarcoid, wegeners granulomatosis: treatment
|
IPF
-Transplant evaluation Scleroderma -Cyclophosphamide Sarcoid -Nothing, prednisone, methotrexate, infliximab Wegener’s Granulomatosis -Cyclophosphamide plus prednisone |
|
|
Summary of ILD
|
History: Occupations, cough, dyspnea, chest pain
Crackles Chest CT PFTs: restrictive, low DLCO Serologies (Antibodies) for vasculitis Biopsy needed for definate diagnosis Treatments: steroids, immunosuppressives, transplant |
