Interstitial lung disease

Question 1

usual interstitial pneumona: etiology and clinical presentation

Answer 1

idopathic ( IPF- idiopathic pulmonary fibrosis) or secondary to asbestos, CT tissue disease, drug toxicities. cough and dyspnea with low DLCO, clubbing.

Question 2

changes on chest x ray for usual interstitial pneumonia

Answer 2

reticular lines, honeycomb changes, no ground glass, nodules, or consolidation

Question 3

Pathologic features of usual interstitial pneumonia

Answer 3

FIBROBLASTIC FOCI, temporal and spacial heterogeneity of scar deposition

Question 4

usual inteststial pneumonia summary

Answer 4

IPF has a very bad prognosis
peaks in 50s and 60s.  
lacks traditional inflammation but has fibroblastic foci
lower lobe predominant
poor prognosis
no response to steroids

Question 5

Aciute interstitial pneumonia: etitology

Answer 5

usually associated with severe infection or shock
may be secondary to toxic exposure or an exacerbation of UIP/IFP. may be idiopathic. causes acute respiratory distress syndrome

Question 6

acute interstitial pneumonia clinical features

Answer 6

viral like prodrome, acute onset with bilateral alveolar infiltrates, fever in 50%, hypoxia, high mortality

Question 7

Pathology of acute interstitial pneumonia

Answer 7

uniform temporal appearance, edema, hyaline membranes, epithelial cell necrosis, NO COLLAGEN OR HONEYCOMB change

Question 8

Summary of acute interstitial pneumonia. diseases, pathology, treatment

Answer 8

it is the pathology associated with acute respiratory distress syndrome but may be associated with exacerbation of IPF.
diffuse, bilateral disease
pathology included epithelial damage and hyaline membranes without collagen deposition or honeycomb change.
no response to steroids and high mortality.

Question 9

cryptogenic organizing pneumonia: summary

Answer 9

multiple etiologies
often post-infectious
mixed cellular infiltrate
diffuse, bilateral, and patchy
responds to steroids but often relapses.

Question 10

crytpogenic organizing pneumonia etiology

Answer 10

idiopathic.
this pathology is called BOOP if it is related to a known cause like bleomycin, CT tissue diseases (rheumatoid arthritis, SLE), or post-infection

Question 11

Pathology of crytpogenic organizing pneumonia

Answer 11

granulaiton tissue in small airways and alveolar ducts. chronic inflammation with mixed cellular infiltrate in surrounding alveoli. patchy distribution but lung architecture is preserved

Question 12

silicosis summary

Answer 12

from inhalation of silica dust. associated with nodules and adenopathy that can calcify. usually upper airlobe disease. avoid exposures and be aware of greater risk of TB. most prevalent occupational lung disease worldwide.

Question 13

silicosis clinical features

Answer 13

long latency, but acute form does exist. may be progressive, increased risk of cancer and TB.

Question 14

Silicosis pathology

Answer 14

concenctrically layered hyalinized fibrous tissue. see crystals in the tissue under right microscope.

Question 15

What is prgressive massive fibrosis. what is alveolar proteinosis

Answer 15

enlargement of masses in silicosis. associated with higher mortality.
alveolar proteinosis: from large dose silica exposure: see abnormal macrophage activity, accumulation of proteinaceous debris

Question 16

coal worker’s pnemoconiosis summary

Answer 16

major factor that induces the development of coal worker’s pnemoconiosis is the amt of silica in the coal. increased prevalence of chronic bronchitis, treat by avoidance. no response to steroids.

Question 17

Clinical features of coal workers pneumoconiosis

Answer 17

long latency. no acute form of disease. many are asymptomatic or show chronic bronchitis.

Question 18

Radiology and pathology for coal worker’s pnemoconiosis

Answer 18

radiology: upper loabe predominant, reticulo-nodular infiltrates with mediastinal and hila adenopathy. may have mass like infiltrates.
path: black pigment; maybe cavitation

Question 19

Asbestosis summary

Answer 19

long latency with a slowly progressive disease
lower lobe fibrosis with pathology indistinguishable form usual interstitial pneumonia except for the presecne of ferruginous bodies.
pleural plaques may be present
no response to steroids but petter prognosis than IPF

Question 20

pathology of asbestosis

Answer 20

ferruginous bodies. biopsy usually unnecessary

Question 21

radiology of asbestosis

Answer 21

basialr predominance with reticular changes, honeycombing. may have ground glass or pleural plaques.

Question 22

radiology AIP.

Answer 22

Patchy bilateral infiltrates, consolidation, GGO, bilateral, no honeycombing, no collagen deposition

Question 23

Clinical features of COP

Answer 23

No wheezing or clubbing in most cases, mimics community acquired pneumonia, inspiratory rales and rhonchi