interstitial lung diseases Flashcards
most common ILDs of UNKOWN etiology. name the 3
idiopathic pulmonary fibrosis
sarcoidosis
pulmo fibrosis assoc with CTDs
most common ILDs with KNOWN etiology
occupational and environmental exposures, inorganic and organic dusts, fumes or gases
immunopathogenic response that leads to pulmo fibrosis
aberrant wound healing
2 major histopathologic patterns in ILD
- granulomatous lung disease
differentials: HP and sarcoidosis - inflammation and fibrosis
most common presentation of ILD based on duration of illness
chronic
unusual to have acute or episodic form
true or false. ILDs most common age occur >60 years old
false.
most common are 20-40 years old
but IPF mostly seen in >60years old
true or false. ILD in hermann pudlak syndrome and those assoc with CTDs are common in women
true
most common PE seen in ILDs
tachypnea and bibasilar end inspiratory rales
most common initial finding on cxr in ILD
bibasilar reticular pattern
diagnostic value of BAL in ILD for sarcoidosis
lymphocytosis
CD4:CD8 ratio >3.5
most common form of ILD is IPF, it has poor response to. chemotherapy.
what is the histologic finding seen in IPF?
usual interstitial pneumonia UIP
alternating areas of normal lung interstitial inflammation and fibrosis
“honeycombing appearance”
ILD seen in younger, nonsmoking women with similar presentation of ILD.
Seen in CTD, drug induced ILD, chronic hypersensitivity pneumonitis
nonspecific interstitial pneumonia
rare, fulminant, diffuse alveolar damage seen in patients >40years old
acute interstitial pneumonia (Hamman Rich Syndrome)
clinicopathologic syndrome of unknown etiology
cryptogenic organizing pneumonia
Rare ILD found almost exclusively in cigarette smokers
desquamative interstitial pneumonia (DIP)
histo: extensive accumulation of macrophages in intraalveolar spaces
DIP characterized by accumulation of macrophages in the PERIBRONCHIAL ALVEOLI
RB ILD respiratory bronchiolitis associated ILD
remember DIP usually have macrophages in the INTRAALVEOLAR SPACES
virtually diagnostic for Pulmonary Langerhans Cell Histiocytosis
combination of nodules and thin walled cysts
pneumothorax occurs in ~25%
majority of CTDs are treated with oral glucocorticoids and immunosuppressive agents except?
progressive systemic sclerosis
classic PFT finding for progressive systemic sclerosis
restrictive pattern and impaired diffusing capacity - BEFORE. and clinical disease appears
dominant histo finding in PSS
NSIP
autoimmune disease with a neutralizing IgG antibody against GM CSF
Pulmonary alveolar proteinosis PAP
common in 30-50 years old male with progressive exertional dyspnea
highly specific and sensitive for diagnosis of acquired PAP
elevated serum anti GM CSF titers
true or false. BAL fluid compared to serum Anti GM CSF better correlates with severity of PAP
true
rare condition in premenopausal women. suspected in young women with “emphysema” and recurrent pneumothorax
pulmonary lymphangioleiomyomatosis LAM
Syndromes with ILD with diffuse alveolar hemorrhage presetns with massive hemoptysis. true or false?
false.
NO hemoptysis but with new alveolar opacities, falling hemoglobin levels, and hemorrhage
mainstay therapy: IV methylprednisolone
