Interstitial Lung Disease - Slocum

Question 1

For Usual interstitial pneumonia (UIP) state:
A. clinical presentaiton
B. relevant Histopath
C. Radiographic patterns
D. Treatment

Answer 1

A. age 55-60, insiduous dry cough and dyspnea lasting months to years. clubbing, fine late inspratory crackles,
B. Patchy, temporally and geographically nonuniform fibrosis, honeycomb change, and normal lung. Type I pneumo are lost and prolif of type II; fibroblastic foci
C. diminished lung volume, increased linear or reticular bibasilar and subpleural opacities; ground-glass and varible honeycomb changes
D. Median surivival is 3 yrs. Nintedanib and pirfenidone reduce rate of decline in lung function

Question 2

For Respiratory bronchiolitis-associated interstitial lung disease state:
A. clinical presentaiton
B. relevant Histopath
C. Radiographic patterns
D. Treatment

Answer 2

A. Similar to UIP but younger. usually are heavy smoker
B. increased macrophage within alveolar space. Alveolar architecture is preserved
C. Indistinguishable from UIP. nodular or reticulonodular pattern. Honeycombing is rare. ground glass opacities
D. Spontaneous remission in 20% of cases. Stop smoking. better prognosis than UIP with median survival of 10 yrs

Question 3

For acute interstitial pneumonia state:
A. clinical presentaiton
B. relevant Histopath
C. Radiographic patterns
D. Treatment

Answer 3

A. wide age range, many youngers. Acute onset of dyspnea followed by rapid respiratory failure; half of pts report viral syndrome preceding lung disease. Clinical course indistinguishable from idiopathic ARDS
B. resembles ARDS with DAD
C. Diffuse b/l airspace consolidation w/ areas of ground-glass attenuation on CT
D. supportive care. 90% die within 2 months. Not progressive if pt survive

Question 4

For nonspecific interstitial pneumonia (NSIP) state:
A. clinical presentaiton
B. relevant Histopath
C. Radiographic patterns
D. Treatment

Answer 4

A. 45-55, more females. similar to UIP but onset of cough and dyspnea over months, not years
B. varying degree of inflammation and fibrosis, patchy in distribution but uniform in time, suggesting response to single injury
C. similar to UIP. b/l areas of ground glass, fibrosis,
D. Treatment is effective but no evidence. Prognosis is good but depends on extent of fibrosis. med survival greater than 10yrs

Question 5

For Cryptogenic organizing pneumonia (COP)state:
A. clinical presentaiton
B. relevant Histopath
C. Radiographic patterns
D. Treatment

Answer 5

A. Typically 50-60. Abrupt onset of frequently weeks to few months following flu like illness. Dyspnea and dry cough prominent, but constitutional symptoms are common; fatigue, fever, wt loss
B. Masson bodies and inflammatory cells fil aveoli and distal bronchiles
C. Lung vol normal. chest radiograph shows interstitial and parenchymal disease with discrete, peripheral alveolar and groundglass infiltrates. CT shows subpleural consolidation and bronchial wall thickness

Question 6

General signs and symptoms of ILD

Answer 6

• shallow, rapid breathign
• SOB
• DOE
• chronic, nonproductive cough

Question 7

Physical findings of ILD

Answer 7

• Cyanosis
• crackles/rales
• inspiratory wheezes
• digital clubbing
• RHF

Question 8

What diagnostic studies can be performed

Answer 8

• Bronchoalveolar lavage (useful in infection, malignancy, eosinophilic pneumonia, langerhan cell histiocytosis, alveolar proeinosis)
• Bransbronchial biopsy (useful in sarcoidosis, phymphagitis spread of carcinoma, PAP, miliary TB, langerhan cell histiocytosis,
• Surgical lung biopsy using VATS: standard form of diagnosis for diffuse interstitial disease
• CXR: gold standard; reticular nodular, ground glass
• PFTs: decreased TLC, FVC, DLCO; normal FEV1/FVC
• Labs: serum markers for connective tissue disease

Question 9

Treatment of ILD

Answer 9

• Supportive
• RB-ILD, NSIP, COP responds to corticosteroids
• IPF: immunodoulatory immunosuppressant agent (nintedanib and pirfenidone. lung transplant is the only difinitive tx for IPF)