Pulm Path III

Question 1

Categories of Chronic Interstitial Lung Disease

Answer 1

• Fibrosing (usual/idiopathic pulmonary fibrosis or pneumoconiosis)
• Granulomatous (Sarcoidosis)
• Eosinophilic
• Smoking related

Question 2

Interstitial Lung Disease clinical presentation

Answer 2

• dyspnea
• end inspiratory crackles
• hypoxia

Question 3

Interstitial Lung Disease Lab

Answer 3

Chest radiographs

-Nodules, irregular lines, ground glass shadows

Question 4

Complications of Interstitial Lung Disease

Answer 4

• Resp. failure via pulmonary HTN or cor pulmonale

- Honeycomb lung (end stage pathology)

Question 5

Cause of Usual/Idiopathic Pulmonary Fibrosis

Answer 5

-repeated cycles of lung injury and wound healing with increased collagen

Question 6

Pathology of Idiopathic Pulmonary Fibrosis

Answer 6

-Pleural surface scarring (cobblestoned)
-firm, rubbery white cut surface
-patchy interstitial fibrosis
Early: exuberant fibroblastic proliferation

Question 7

Clinical Presentation of Idiopathic Pulmonary Fibrosis

Answer 7

• 40 to 70 years old more in men
• Dyspnea on exertion (DOE)
• Dry cough
• Velcro crackles
• Deterioration, less than 3 year survival
• Lung transplant only definite Tx

Question 8

What is pneumoconiosis?

Answer 8

The accumulation of dust in the lungs and the tissue reaction to its presence

Question 9

What pneumoconiosis is coal dust?

Answer 9

Anthracosis from coal mining

-Caplan syndrome suggesting rheumatoid arthritis modifies response to coal mine exposure

Question 10

What pneumoconiosis is silica?

Answer 10

Silicosis from sandblasting

• most prevalent chronic world occupation disease
• Nodules: concentric hyalinized, whorled appearance
• inc. susceptibility to TB

Question 11

What pneumoconiosis is asbestos?

Answer 11

Asbestosis from ship building, roofing, or plumbing

• Asbestos bodies and/or pleural plaques
• 5x increase chance for lung cancer (55x if they smoke too)
• malignant mesothelioma is 1000x w/ asbestos but not cig smoke with latency of 25-40 years

Question 12

What pneumoconiosis is beryllium?

Answer 12

Berylliosis from mining, fabrication, or stealth jet building

Question 13

What is the pathogenesis of pneumoconiosis?

Answer 13

• inhaled dust largely entrapped in mucus
• 1 to 5 micrometer particles are most dangerous
• They reach the terminal small airways, air sacs, and settle in lining
• Macrophages endocytose trapped particles and release inflammatory mediators which lead to fibrogenesis and collagen deposition

Question 14

Classification of Sarcoidosis

Answer 14

multi system disease of unknown etiology

• characterized by non-caseating granulomas
• diagnosis of exclusion

Question 15

Sarcoidosis CXR

Answer 15

Abnormalities in 90% of patients

• Bilateral hilar lymphadenopathy
• Lung parenchymal abnormalities

Question 16

Sarcoidosis pathogenesis

Answer 16

• CD4 T cells dominant in sarcoid granulomas
• Tregs down regulated
• INF gamma activates macrophages
• TNF is a mediator of granuloma formation

Question 17

Clinical Presentation of Sarcoidosis

Answer 17

• usually young adults
• African americans, danish, swedish
• usually upper lobe
• SOB, dry cough, non-specific
• 10 - 15% progress to pulmonary fibrosis
• 2/3 of patients are asymptomatic

Question 18

Histology for Sarcoidosis

Answer 18

• Non-caseating granulomas
• coalesce granulomas with a rim of lymphocytes, embedded in eosinophilic collagenous storm
• present along lymphatic routes
• multinucleated giant cells with cytoplasmic inclusions: Schumann bodies, steroid bodies

Question 19

Dx of Sarcoidosis

Answer 19

transbronchial biopsy in 90% if 4 pieces submitted

• routine AFB and GMS
• Dx of exclusion

Question 20

Labs for Sarcoidosis

Answer 20

• elevated ACE level
• hypercalcemia
• markers to systemic immune abnormalities (anergy to common skin test antigens)
• Polyclonal hypergammaglobulinemia