Restrictive Lung Disease Flashcards
Restrictive lung diseases
Characterized by loss of lung compliance. They result in increased lung stiffness and decreased lung expansion.
Etiologies are AINT. The lungs AINT compliant
Alveolar - edema, hemorrhage, pus
Interstitial lung disease (Idiopathic interstitial pneumonias), Inflammatory (sarcoid, cryptogenic organizing pneumonias), Idiopathic
Neuromuscular - MG, phrenic nerve palsy, myopathy
Thoracic wall - kyphoscoliosis, obesity, ascites, pregnancy, ankylosing spondylitis
Interstitial lung disease
Heterogenous group of disorders characterized by inflammation and/or fibrosis of interalveolar spectum**
In advanced disease, cystic spaces develop in lung periphery (honeycombing)
Causes include:
1) idiopathic interstitial pneumonias
2) collagen vascular diseases
3) Granulomatous disorders
4) Drugs (amiodarone, busulfan, nitrofurantoin, bleomycin, radiation, long term high O2 concentrations - ventilators)
5) hypersensitivity disorders
6) Pneumoconiosis
7) Eosinophilic pulmonary syndromes
IPF is also included in this category tho it’s a diagnosis of exclusion
History and physical in ILD
1) Presents with shallow, rapid breathing. Dyspnea with exercise and a nonproductive cough
2) Patients may have cyanosis, inspiratory squeaks, fine or velcro-like crackles, clubbing or right heart failure
Dx of ILD
1) CXR/CT: Reticular, nodular, or ground glass pattern. Honeycomb pattern in severe disease
2) PFTs: Low TLC, Low FVC, Low DL of CO (may be normal if cause is extrapulmonary), normal FEV/FVC. Serum markers of connective tissue diseases should be obtained if indicated
3) Surgical biopsy is often indicated to confirm dx of IPF with evidence of interstitial inflammation and fibrosis. Alveolar thickening.
Tx of ILD
1) Supportive. Avoid exposure to causative agents. Some inflammatory diseases respond to steroids or other anti-inflammatory/immunosuppressive agents
2) Lung transplant may be indicated in late stage of IPF.
Types of pneumoconioses
1) Asbestosis
2) Coal miner’s disease
3) Silicosis
4) Berylliosis
Asbestosis
1) History: Work involving manufacture of tile or brake linings, insulation, construction, demolition or shipbuilding. Presents 15-20 years after initial exposure
2) Diagnosis: CXR with linear opacities at lung bases and interstitial fibrosis. Calcified pleural plaques are indicative of benign pleural disease
3) Complications: Increased risk of mesothelioma (rare) and lung cancer. The risk of lung cancer is higher in smokers
Coal miner’s disease
1) History: Work in underground coal mines
2) Diagnosis: CXR shows small nodular opacities less than 1cm in upper lung zones. Spirometry consistent with restrictive disease
3) Complications: Progressive massive fibrosis
Silicosis
1) History: Work in mines or quarries or with glass, pottery or silica.
2) Diagnosis: CXR with small (less than 1cm) nodular opacities in upper lung zones and Eggshell calcifications. Spirometry consistent with restrictive disease
3) Complications: Increased risk of TB. Need annual TB skin test. Progressive massive fibrosis.
Berylliosis
1) History: Work in high tech fields such as aerospace, nuclear and electronics plants. Ceramics industries. Foundries. Plating facilities. Dental material sites. Dye manufacturing.
2) Diagnosis: CXR shows diffuse infiltrates and hilar adenopathy
3) Complications: Requires chronic corticosteroid treatment
Systemic sarcoidosis
Multisystem disease of unknown etiology characterized by noncaseating granulomas. Most commonly found in African American females and northern european caucasians. Most often arises in third or fourth decades of life.
History and physical for sarcoid
1) Can present with fever, cough, malaise, weight loss, dyspnea, and arthritis
2) The lungs, liver, eyes, skin (erythema nodosum, violaceous skin plaques), nervous system, heart and kidney may be affected
The features of sarcoid are GRUELING
Granulomas aRthritis Uveitis Erythema nodosum LAD Interstitial fibrosis Negative TB test Gammaglobulinemia
Dx of sarcoid
1) CXR/CT: Radiographic findings of LAD and nodules are used to stage the disease
2) Biopsy: LN bx or transbronchial/VAT lung bx reveals noncaseating granulomas
3) PFTs: Restrictive or obstructive pattern and reduced diffusion capacity
4) Other findings: Increased serum ACE levels (neither sensitive nor specific). Hypercalcemia, hypercalciuria. Increased Alk Phos (with liver involvement), Lymphopenia, cranial nerve deficitis, arrhythmias
Treatment for sarcoid
Systemic corticosteroids are indicated for deteriorating respiratory function, constitutional symptoms, hypercalcemia or extrathoracic organ involvement
Hypersensitivity pneumonitis
Alveolar thickening and granulomas secondary to environmental exposure
