Restrictive Lung Disease

Question 1

Causes of restrictive lung disease ?

Answer 1

_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)

Question 2

Interstitial Lung Disease?

Answer 2

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”).

Question 3

Causes of Interstitial Lung Disease?

Answer 3

Causes include:

• idiopathic interstitial pneumonias,
• collagen vascular disease,
• granulomatous disorders,
• drugs,
• hypersensitivity disorders,
• pneumoconiosis, and
• eosinophilic pulmonary syndromes.

Question 4

HISTORY/PE of Interstitial Lung Disease?

Answer 4

• 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.

Question 5

DIAGNOSIS of Interstitial Lung Disease?

Answer 5

• 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.

Question 6

TREATMENT of Interstitial Lung Disease?

Answer 6

Supportive. Avoid exposure to causative agents. Some inflammatory diseases respond to corticosteroids or other anti-inflammatory/immunosuppressive agents.

Question 7

Systemic Sarcoidosis ?

Answer 7

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.

Question 8

HISTORY/PE of Systemic Sarcoidosis ?

Answer 8

• 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).

Question 9

Features of Sarcoidosis ?

Answer 9

_GRUELING:

• Granulomas
• aRthritis
• Uveitis
• Erythema nodosum
• Lymphadenopathy
• Interstitial fibrosis
• Negative TB test
• Gammaglobulinemia

Question 10

DIAGNOSIS of Sarcoidosis ?

Answer 10

• 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.

Question 11

TTT of of Sarcoidosis ?

Answer 11

Systemic corticosteroids are indicated for constitutional symptoms, hypercal- cemia, or extrathoracic organ involvement.

Question 12

Hypersensitivity Pneumonitis ?

Answer 12

Risk factors include environmental exposure to antigens leading to alveolar thickening and granulomas.

Question 13

HISTORY/PE of Hypersensitivity Pneumonitis ?

Answer 13

• 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.

Question 14

DIAGNOSIS of Hypersensitivity Pneumonitis ?

Answer 14

CXR is normal or shows miliary nodular infiltrate (acute); fibrosis is seen in the upper lobes (chronic).

Question 15

TREATMENT of Hypersensitivity Pneumonitis ?

Answer 15

Avoid ongoing exposure to inciting agents; give corticosteroids to ↓ chronic inflammation.

Question 16

Pneumoconiosis is?

Answer 16

Risk factors include prolonged occupational exposure and inhalation of small inorganic dust particles.

Question 17

Types of Pneumoconiosis ?

Answer 17

• Asbestosis.
• Coal miner’s disease.
• Silicosis.
• Berylliosis.

Question 18

Asbestosis History and diagnosis ?

Answer 18

• 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.

Question 19

Coal miner’s disease History and diagnosis ?

Answer 19

• Work in underground coal mines.
• CXR: Small nodular opacities (< 1 cm) in upper lung zones.
• Spirometry: Consistent with restrictive disease.

Question 20

Silicosis History and diagnosis ?

Answer 20

• 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.

Question 21

Berylliosis History and diagnosis ?

Answer 21

• 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.

Question 22

Usual Interstitial Pneumonia (Idiopathic Pulmonary Fibrosis) ?

Answer 22

The most common form of idiopathic interstitial pneumonia. Has an unrelenting progression, with death usually occurring within 5–10 years.

Question 23

History/PE of Usual Interstitial Pneumonia (Idiopathic Pulmonary Fibrosis) ?

Answer 23

• Exertional dyspnea and a nonproductive cough.

- Inspiratory crackles and/or clubbing on exam.

Question 24

Diagnosis of Usual Interstitial Pneumonia (Idiopathic Pulmonary Fibrosis) ?

Answer 24

• 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.

Question 25

TREATMENT of Usual Interstitial Pneumonia (Idiopathic Pulmonary Fibrosis) ?

Answer 25

Options include corticosteroids, cytotoxic agents (azathioprine, cyclophospha- mide), antifibrotic agents (have not been shown to improve survival), and lung transplantation.

Question 26

Eosinophilic Pulmonary Syndromes?

Answer 26

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.

Question 27

HISTORY/PE of Eosinophilic Pulmonary Syndromes?

Answer 27

Presents with dyspnea, cough, and/or fever.

Question 28

diagnosis of Eosinophilic Pulmonary Syndromes?

Answer 28

CBC reveals peripheral eosinophilia; CXR shows pulmonary infiltrates.

Question 29

TTT of Eosinophilic Pulmonary Syndromes?

Answer 29

Removal of the extrinsic cause or treatment of underlying infection if identified. Corticosteroid treatment may be used if no cause is identified.