Restrictive Lung Disease Flashcards
Causes of restrictive lung disease ?
_PAIIINT:
- Pleural (fibrosis, effusions, empyema, pneumothorax).
- Alveolar (edema, hemorrhage, pus)
- Interstitial lung disease (idiopathic interstitial pneumonias),
- Inflammatory (sarcoid, cryptogenic organizing pneumonitis),
- Idiopathic.
- Neuromuscular (myasthenia, phrenic nerve palsy, myopathy)
- Thoracic wall (kyphoscoliosis, obesity, ascites, pregnancy, ankylosing spondylitis)
Interstitial Lung Disease?
A heterogeneous group of disorders characterized by inflammation and/or fibrosis of the interalveolar septum.
In advanced disease, cystic spaces develop in the lung periphery (“honeycombing”).
Causes of Interstitial Lung Disease?
Causes include:
- idiopathic interstitial pneumonias,
- collagen vascular disease,
- granulomatous disorders,
- drugs,
- hypersensitivity disorders,
- pneumoconiosis, and
- eosinophilic pulmonary syndromes.
HISTORY/PE of Interstitial Lung Disease?
- Presents with shallow, rapid breathing.
- dyspnea with exercise; and a nonpro- ductive cough. Patients may have cyanosis, inspiratory squeaks, fine or “Vel- cro-like” crackles, finger clubbing, or right heart failure.
DIAGNOSIS of Interstitial Lung Disease?
- CXR: Reticular, nodular, or ground-glass pattern; “honeycomb” pattern (severe disease).
- ↓ TLC, ↓ FVC, ↓ DLCO (may be normal if the cause is extrapulmonary), normal FEV1/FVC. Serum markers of connective tissue diseases should be obtained if clinically indicated.
TREATMENT of Interstitial Lung Disease?
Supportive. Avoid exposure to causative agents. Some inflammatory diseases respond to corticosteroids or other anti-inflammatory/immunosuppressive agents.
Systemic Sarcoidosis ?
A multisystem disease of unknown etiology characterized by noncaseating “non-necrotizing” granulomas. Most commonly found in African-American females and northern European Caucasians; most often arises in the third or fourth decade of life.
HISTORY/PE of Systemic Sarcoidosis ?
- Presents with fever, cough, malaise and arthritis.
- weight loss & dyspnea.
The lungs, liver, eyes, skin (erythema nodosum, violaceous skin plaques), nervous system, heart, and kidney may be affected. Symptoms may be GRUELING (see mnemonic).
Features of Sarcoidosis ?
_GRUELING:
- Granulomas
- aRthritis
- Uveitis
- Erythema nodosum
- Lymphadenopathy
- Interstitial fibrosis
- Negative TB test
- Gammaglobulinemia
DIAGNOSIS of Sarcoidosis ?
- CXR: Radiographic findings are used to stage the disease.
- Biopsy: Lymph node biopsy or transbronchial/video-assisted thoraco-
scopic lung biopsy reveals noncaseating granulomas. - PFTs: Restrictive or obstructive pattern and ↓ diffusion capacity.
- Other findings: ↑ serum ACE levels (neither sensitive nor specific), hy-
percalcemia, hypercalciuria, ↑ alkaline phosphatase (with liver involve- ment), lymphopenia, cranial nerve defects, arrhythmias.
TTT of of Sarcoidosis ?
Systemic corticosteroids are indicated for constitutional symptoms, hypercal- cemia, or extrathoracic organ involvement.
Hypersensitivity Pneumonitis ?
Risk factors include environmental exposure to antigens leading to alveolar thickening and granulomas.
HISTORY/PE of Hypersensitivity Pneumonitis ?
- Acute: Dyspnea, fever, malaise, shivering, and cough starting 4–6 hours after exposure.
- Chronic: Patients present with progressive dyspnea; exam reveals fine bi-lateral rales.
DIAGNOSIS of Hypersensitivity Pneumonitis ?
CXR is normal or shows miliary nodular infiltrate (acute); fibrosis is seen in the upper lobes (chronic).
TREATMENT of Hypersensitivity Pneumonitis ?
Avoid ongoing exposure to inciting agents; give corticosteroids to ↓ chronic inflammation.
Pneumoconiosis is?
Risk factors include prolonged occupational exposure and inhalation of small inorganic dust particles.
Types of Pneumoconiosis ?
- Asbestosis.
- Coal miner’s disease.
- Silicosis.
- Berylliosis.
Asbestosis History and diagnosis ?
- Work involving manufacture of tile or brake linings, insulation, construction, demolition, or shipbuilding. Presents 15–20 years after initial exposure.
- CXR: Linear opacities at lung bases and interstitial fibrosis; calcified pleural plaques are indicative of benign pleural disease.
Coal miner’s disease History and diagnosis ?
- Work in underground coal mines.
- CXR: Small nodular opacities (< 1 cm) in upper lung zones.
- Spirometry: Consistent with restrictive disease.
Silicosis History and diagnosis ?
- Work in mines or quarries or with glass, pottery, or silica.
- CXR: Small (< 1-cm) nodular opacities in upper lung zones.
- Eggshell calcifications.
- Spirometry: Consistent with restrictive disease.
Berylliosis History and diagnosis ?
- Work in high-technology fields such as aerospace, nuclear, and electronics plants; ceramics industries; foundries; plating facilities; dental material sites; and dye manufacturing.
- CXR: Diffuse infiltrates; hilar adenopathy.
Usual Interstitial Pneumonia (Idiopathic Pulmonary Fibrosis) ?
The most common form of idiopathic interstitial pneumonia. Has an unrelenting progression, with death usually occurring within 5–10 years.
History/PE of Usual Interstitial Pneumonia (Idiopathic Pulmonary Fibrosis) ?
- Exertional dyspnea and a nonproductive cough.
- Inspiratory crackles and/or clubbing on exam.
Diagnosis of Usual Interstitial Pneumonia (Idiopathic Pulmonary Fibrosis) ?
- High-resolution CT: Patchy opacities at the lung bases, often with honey- combing.
- PFTs: Restrictive pattern.
- Surgical biopsy (usually required to confirm the diagnosis): Interstitial in-
flammation, fibrosis, and honeycombing.
TREATMENT of Usual Interstitial Pneumonia (Idiopathic Pulmonary Fibrosis) ?
Options include corticosteroids, cytotoxic agents (azathioprine, cyclophospha- mide), antifibrotic agents (have not been shown to improve survival), and lung transplantation.
Eosinophilic Pulmonary Syndromes?
A diverse group of disorders characterized by eosinophilic pulmonary infil- trates and peripheral blood eosinophilia. Includes allergic bronchopulmo- nary aspergillosis, Löffler’s syndrome, and acute eosinophilic pneumonia.
HISTORY/PE of Eosinophilic Pulmonary Syndromes?
Presents with dyspnea, cough, and/or fever.
diagnosis of Eosinophilic Pulmonary Syndromes?
CBC reveals peripheral eosinophilia; CXR shows pulmonary infiltrates.
TTT of Eosinophilic Pulmonary Syndromes?
Removal of the extrinsic cause or treatment of underlying infection if identified. Corticosteroid treatment may be used if no cause is identified.
