Pulmonary- Restrictive/ Interstitial Flashcards
Define restrictive lung disease
Restricted lung expansion causes decreased lung volumes with preserved FEV1/FVC ratio.
Causes: -Mechanical Muscle weakness: polio, MG, GBS Structural: Scoliosis, obesity -Interstitial
What are the causes of interstitial lung disease
Fibrosis (Idiopathic/ Pneumoconiosis/ Drugs) Sarcoidosis Hypersensitivity Pneumonitis Pulmonary Hypertension BOOP/COP Vasculitis
What drugs can cause interstitial lung disease?
Bleomycin Busulfan Amiodarone Methysergide Nitrofurantoin Methotrexate
What is pulmonary fibrosis?
thickening of the interstitial septum of the lung between the arteriolar space and the alveolus.
Interferes with gas exchange in both directions
Characteristics of coal workers pneumoconiosis:
- Exposure
- X-ray findings
- Location
- Risks
- Exposure: Coal
- CXR: fine nodular opacifications
- Location: upper lobes
- Risk: Caplan syndrome
Characteristics of silicosis:
- Exposure
- X-ray findings
- Location
- Risks
- Exposure: sandblasting, rock mining, tunneling, foundries
- CXR: eggshell calcifications & opacities
- Location: upper lobes
- Risk: TB, Caplan Syndrome
Characteristics of Asbestosis:
- Exposure
- X-ray findings
- Location
- Risks
- Exposure: shipyard, plumbing (pipes), insulators, roofing
- CXR: diffuse bilateral infiltrates; calcified supradiaphragmatic & pleural plaques.
- Location: Lower lobes
- Risk: mesothelioma, bronchogenic carcinoma (worse with smoking), pleural effusions
Characteristics of Byssinosis:
- Exposure
- X-ray findings
- Location
- Risks
- exposure: cotton
- CXR: diffuse haziness
- Location: lower lobes
- Risk: n/a
Characteristics of Berylliosis:
- Exposure
- X-ray findings
- Location
- Risks
- exposure: electronic manufacturing, aerospace manufacturing
- CXR: reticular interstitial pattern (similar to sarcoidosis)
- Location: upper lobes
- Risks: cor pulmonale, cancer, TB, Caplan syndrome
Characteristics of Bagassosis:
- Exposure
- X-ray findings
- Location
- Risks
- Exposure: moldy sugar cane
- CXR: diffuse infiltrates, ground glass honeycomb (advanced)
- Location: Mid to upper lobes
- Risk: cor pulmonale
Signs and symptoms of pulmonary fibrosis:
Dyspnea (worse w/ exertion) Short shallow breaths Fine rales or crackles Loud P2 heart sounds clubbing of the fingers
Diagnostic tests for pulmonary fibrosis
BIT: CXR or high resolution CT (more accurate)
Most accurate: lung biopsy
PFT: decreased DLCO, increased A-a gradient
Echo: pulmonary HTN, right ventricular hypertrophy
What is Caplan Syndrome?
Pneumoconiosis in combinations with Rheumatoid Arthritis and intrapulmonary nodules
Tx pulmonary fibrosis
Prednisone or Steroids (berylliosis most likely to respond) Azithioprine (long term alt. to steroids) Cyclophosphamime s(no response to steroids) Pirfenidone/ Nintedanib (slow fibrosis)
How do pirfenidone and nintedanib work?
Pirfenidone: inhibits collagen synthesis
ninetedanib: tyrosine kinase inhibitor, blocks ffibrogenic growth factors and inhibits fibroblast
Define sarcoidosis
an idiopathic inflammatory disorder predominantly of the lungs but can affect whole body. Widespread noncaseating granulomas
Signs and symptoms of sarcoidosis
Young African American women SOB with fine rales Erythema nodosum Lymphadenopathy Parotid gland enlargement Facial palsy Heart block and restrictive cardiomyopathy CNS involvement Iritis and Uveitis
Diagnostic tests sarcoidosis
BIT: CSR (hilar adenopathy)
Most accurate: lymphadenopathy biopsy (noncaseating granuloma)
Hypercalcemia
Elevated ACE
BAL: increased helper cells
Tx sarcoidosis
Prednisone
Define pulmonary hypertension (PHTN)
Pulmonary systolic BP >20mmHg.
Causes:
- Pulmonary arterial hypertension (idiopathic)
- Hypoxemia (COPD, Fibrosis, chronic lung disease)
Mechanism of hypoxia causing PHTN
Hypoxemia causes vasoconstriction of the pulmonary circulation. Shunting blood away from areas of the lung with poor oxygen. causing full system constriction resulting in more hypoxemia (feedback)
Signs and symptoms of PHTN
Dyspnea Fatigue Syncope Chest pain Wide splitting of S2 w/ loud P2
Diagnostic tests for PHTN
BIT: CXR (dilation of proximal pulmonary arteries, narrowing “pruning” distal vessels)
Most accurate: Swan Ganz catheter
EKG/Echo: right hypertrophy
Polycythemia
Tx PHTN
Correct underlying disease. Endothelin antagonist (Bosnian) Phosphodiesterase inhibitors (sildenafil) Prostacyclin analogue vasodilators CCB
Lung transplant (curative for idiopathic)
What drugs are prostacyclin analogue vasodilators?
Epoprostenol Treprostinil Iloprost Beraprost Selexipag
Define BOOP/COP
Bronchiolitis obliterans organized pneumonia (old name)
Cryptogenic Organized Pneumonia (new name)
Patchy process with proliferation of granulation tissue in small airways and ducts. Presents similar to CAP.
Diagnostic test and Tx of BOOP/COP
Most accurate test: lung biopsy
Tx: glucocorticoids
What is hypersensitivity pneumonitis?
An exaggerated immunological response to repeat exposure to antigens such as actinomycetes, fungi, molds and bird droppings. (Farmers Lung)
S&S acute hypersensitivity pneumonitis
Sudden fever, cough, dyspnea Fine crackles Leukocytosis Recurrent episodes Symptoms decrease after expose (e.g.when traveling)
Diagnosis tests
CXR: (Acute) scattered micro nodular interstitial opacities
BAL: high relative lymphocyte count
Lung biopsy: lymphocytic infiltrate
What is eosinophilic pneumonia?
Pneumonia that presents with 1-2wks of fever, cough, SOB and processes to respiratory failure.
Hx: cancer Drugs, parasitic infections
What medications can precipitate eosinophilic pneumonia
Amiodarone NSAIDs Nitrofurantoin Phenytoin DAptomycin
What parasites can precipitate eosinophilic pneumonia
Strongyloidiasis
Ascariasis
Trichinellosis
Schistosomiasis
Diagnostic test eosinophilic pneumonia
Most accurate test: BAL or biopsy (eosinophilic presence)
Common causes of increased A-a gradient
Diffusion limitation (emphysema, fibrosis)
V/Q mistmatch (PE, lobar pneumonia)
Intrapulmonary shunt (p. edema)
Dead-space ventilation (PE, R–> L cardio shunt)
Tx hypersensitivity pneumonitis
glucocorticoids if persistent after antigen exposure removal.
azathioprine (alt. chronic steroids)
Acute HP: self resolving
S&S chromic hypersensitivity pneumonitis
Progressive cough Dyspnea Fatigue, weight loss Hypoxemia worse with exertion CXR: diffuse reticular interstitial opacities
Late phase reaction asthma
Hours after immediate response in asthma exacerbation . Characterized by proinflamatory gene expression, de novo synthesis of Th2 cytokines, further recruitment of leukocytes. Worsening bronchospasm
Tx late phase asthma reaction
systemic glucocorticoids
