Restrictive Diseases

Question 1

Restrictive disease

Answer 1

TLC decreases

decrease in FVC and FEV1 but more so in FVC so the FEV1:FVC ratio stays the same or is increased (greater than 80%)

Question 2

what is the most common cuases of restrictive disease

Answer 2

interstitial disease - interstitial fibrosis

may also arise with chest wall abnormality (obesity)

Question 3

ideopathic pulmonary fibrosis

Answer 3

fibrosis of lung interstitum

• etiology is releated to cyclical lung injury (and healing)
  
  • TGF-b from injured pneumocytes induce the fibrosis
  • secondary cuases such as drugs (bleomycin and amiodarone) and radiation therapy must be excluded before making the diagnosis

Question 4

clinical featuers of ideopathic pulm fibrosis

Answer 4

progressive dyspnea and cough

fibrosis on lung CT - usually just starts in the sub pleura region but can progress to include the entire lung

treatment is lung transplantation

cobble stone pleural surface

intralobular - sub pleural

Question 5

pneumoconioses

Answer 5

• interstitial fibrosis due to ocupational exposure (cuasing the macrophages to lay down fibrosis)
• requires chronic exposure to small particals that are fibrogenic
  
  • must be small enough to get to the bottom of the lung
  • small particals get taken up by macrophages to induce the fibrosis

Question 6

things that can cuase pneumoconioses

Answer 6

• coal workers pneumoconiosis - carbond dust
  
  • leads to diffuse fibrosis (black lung) lung shrinks becuase of fibrosis, associated with rheumatoid arthritis (caplan syndrome)
  • mild exposure = anthracosis (dark macrophages in lung and LNs = not clinically significant
• Silicosis - seen in sand blasters and silica miners
  
  • fibrotic nodules in upper lobes of the lung
  • increased risk for TB (only one that does this), silica impares phagolysosome formation by macrophages
• berylliosis - seen in beryllium miners and workers in aerospace industry
  
  • noncaseating granulomas in the lung, hilar LNs and systemic organs (sounds like sarcoidosis but it is driven by beryllium)
  • it also increases risk of lung cancer
• asbestosis - construction workers, plumbers and shipyard workers
  
  • can cuase fibrosis of lung or pleura(plaques) and cancer of the lung or pleura (mesothelioma)
    
    • these patients are much more likely to get lung cancer than mesothelioma
  • can see asbestos bodies histologically
  • greeks and people from turky are more likely

Question 7

sarcoidosis

Answer 7

• systemic disease characterized by noncaseating granulomas in multiple organs (usually lung and hilar LNs)
  
  • leads to restrictive lung disease
  • other commonly involved tissues
    
    • uvea (uveitis) - eye
    • skin (cutaneous nodules or erythema nodosum)
    • salivary and lacriminal glands
    • almost any tissue can be involved
• classically seen in AA females
• etiology is unknown but probably due to CD4 helper T cell response to unknown antigen
• can see epitheiliodi histeocytes with giant cells, astroid bodies can also be seen sometimes also showmans bodies
• high ACE and high calcium

Question 8

clincal features of sarcoidosis

Answer 8

• dyspnea or cough
• elevated serum ACE
• hypercalcemia - occurs becuase the granulomas can activate Vit D - this will happen with any disease that has noncaseating granulomas
• treatment is steriods; often resolves spontaneously without treatment

Question 9

hypersensitivity pneumonitis

Answer 9

• granulomatous reaction to inhaled organic antigens (pideon breeders lung) - but there are eosinophils associated with the granuloma (because this is a hypersensitive reaction)
  
  • farmers lung - moldy hay
  • bagassosis - moldy sugar cain
  • humidifie lung - thermophilic bacteria in heated water resevours
• presents with fever, cough, and dyspnea hours after exposure; resolves with removal of the exposure
• chronic exposure leads to interstitial fibrosis
  
  • if you take away stimuli before this then it resolves
• type 3/ type 4 immune response

Question 10

Cryptogenic organizing pneumonia

Answer 10

unknown etiology

polyploid plugs of loose organizing CT within small airways

treat with steriods - may also resolve on its own

Question 11

Langerhans cell histiocytosis

Answer 11

birbeck granules (tenssis racket shaped) cells on electron microscopy

seen in adults who smoke

pneumothorax is a classic complication

Question 12

pulmonary alveolar proteinosis

Answer 12

autoimmune (acquired) APA - most common type

• anti GM-CSF antibodies - blocks macrophages ability to digest surfactant - you get accumulation of acellular surfactant in intra alveolar and bronchiolar spaces
• when you do a CXR you will have bilateral opacities (in both lung)

Question 13

Respiratory bronchiolysis

Answer 13

• presents in 30s and 40s males and females who has excessive cigarette smoking
• macrophage infiltrates whith rare eosinophils, mucostasis and atelectasis
• CXR = bilateral reticulnodular infiltrates; ground galss alveolar inflitrates focally
• positive response to steriods; stop smoking - very good survival rate 100%

Question 14

usual interstitial pneumonia

Answer 14

same as ideopathic pulm fibrosis