Interstitial Lung Diseases Flashcards
Who gets Respiratory Distress syndrome (RDS)?
preterm infants (<37 wks)
Which syndrome is due to deficiency of surfactant in immature lung?
RDS
What is important to give a newborn w/ RDS?
-Give surfactant
How is RDS diagnosed?
_*Clinically_
- CXR: reticulogranular ground glass appearance (interstitial pattern) w/ air bronchograms
- must r/o other causes of resp. distress
How do you prevent RDS?
-prevent preterm birth
-give antenatal (before birth) steroids
How is RDS managed
CPAP
or
intubation + surfactant therapy + mechanical ventilation
The following are hallmarks of which dz?
Hypoxemia & bilateral radiographic opacities
Acute Respiratory Distress syndrome (ARDS)
(note- this is NOT the same as respiratory distress syndrome in newborns)
Which dz looks like TB but is less severe
(sxs include cough, chest discomfort, +/- hemoptysis, dyspnea, fatigue, malaise, weakness)
MAC pulmonary dz
(a non-TB mycobacteria infection AKA- NTM)
What is the name of each of the 2 major clinical presentations of MAC pulmonary dz?
- _______: pts w/ underlying lung dz
- ______: dz in pt w/o underlying lung dz
cavitary disease: pts w/ underlying lung dz
nodular bronchiectatic disease: dz in pt w/o underlying lung dz
primarily in white, middle-aged to elderly men (often alcoholics) w/ underlying COPD
MAC pulmonary dz in pt w/ underlying dz
Primarily nonsmoking women >50 who have underlying bronchiectasis
MAC pulmonary dz in pt w/o underlying lung dz
What are the 3 ways the lung is affected in interstitial lung disease?
_1. *lung parenchyma is damaged_
_2. *nflammation of the alveolar walls_
_3. *Scarring (fibrosis) begins_
- 2 most common sxs of ILD
- 2 uncommon sxs of ILD
- Progressive dyspnea on exertion
- nonproductive cough
Uncommon: wheezing and CP
What is the most common CXR finding in interstitial lung dz
Reticular “netlike” opacities
**this will be on exam**

What is the prognosis of Interstitial lung dz (ILD) if honeycombing is seen on CXR?
poor prognosis

What do PFTs show in interstitial lung disease (ILD)
decreased TLC (<80%)
normal FEV1/FVC ratio (nml= 70-80%)
= restrictive
(also decreased DLCO and hypoxemia seen)
What is the gold standard for interstitial lung disease (ILD)?
Lung biopsy
(may be required for definitive diagnosis or to stage dz)
not always indicated
T/F: Interstitial Lung Disease (ILD) can lead to Cor pulmonale–> R heart failure?
True
What are the 4 known causes of interstitial lung diseases (ILDs)
- Occupational and environmental exposures (ex: asbestos)
- Drugs and poisons (ex: Chemo, radiation)
- Infections
- Idiopathic of assoc. w/ idiopathic diseases (ex: sarcoidosis)
“IDIO”
_______= any disease of the respiratory tract due to inhalation of dust particles
(ex: asbestosis or silicosis)
Pneumoconiosis
What condition?
- presents 10-15 yrs after exposure
- 40-75 y/o males
- construction, pipefitter or shipyard worker
- smoker
Asbestosis
What disease?
-Cancer assoc. w/ short- term (1-2 yrs) asbestos exposre
-not caused by smoking
-prognosis= 6-12 mo after presentation
Mesothelioma
What is the definitive dx of asbestosis but is not usually indicated
open-lung biopsy
Which dz?
CXR- opacities in lower lungs, _pleural plaques*_
Asbestosis
What 4 things suggest diagnosis of asbestosis?
consistent history, physical findings, sxs and CXR
Asbestosis- what do PFTs look like?
decreased TLC
FEV1/FVC ratio normal
= restrictive
(PFTs to follow dz progress)
T/F: there is no effective therapy for Asbestosis?
True. The goal is to stop progression
T/F: lung damage in Asbestosis is reversible
FALSE
damage is IRREVERSIBLE
_______ + smoking= 59x more likely to get lung CA
What dz gives you an elevated risk for mesothelioma
Asbestosis
(b/c of asbestos exposure)
Which dz has noncaseating granulomas?
Sarcoidosis
Which disease?
- multisystem inflammatory dz (unknown etiology)
- 30-60 y/o African American female
- noncaseating granulomas
- Hilar adenopathy
- elevated ACE
Sarcoidosis

Which disease?
- asymptomatic to multisystem disease
- (Fever, anorexia, arthralgias, DOE, cranial nerve palsies, visual disturbances, erythema nodusum, etc)
Sarcoidosis
Which disease shows the following on CXR depending on the stage?
stage 0=normal
1- hilar adenopathy
2- hilar adeopathy+ diffuse infiltrates
3- only diffuse infiltrates
4- pulmonary fibrosis
Sarcoidosis
What do PFTs show in sarcoidosis?
Isolated decreased DLCO
Restrictive pattern w/ advanced dz
MC pattern of what in which disease?
Bilateral symmetric hilar and right paratracheal mediastinal adenopathy
MC pattern of lymphadenopathy in sarcoidosis

Labs for which dz show the following:
- hypercalcemia
- elevated ESR
- Elevated serum protein (excess immunoglobulins)
- elevated ACE
Diagnosis of Sarcoidosis usually requires what in most cases?
Biopsy:
Fiberoptic bronchoscopy w/ transbronchial biopsy
How do you dx sarcoidosis (3 things)
- compatible clinical and radiographic maifestations
- exclusion of other diseases
- Histopathologic- noncaseating granulomas
How is Sarcoidosis managed?
consult pulmonologist
>75% only need NSAIDS
Corticosteroids if severe
Monitor CXRs and sxs
Which dz?
-immune mediated, systemic vasculitis
-Necrotizing granulomas of the upper and lower resp tracts
- renal involvement
- 40-50y/o white male or female
Granulomatosis w/ Polyangiitis

which disease can result in ESRD
Granulomatosis w/ Polyangiitis
Sxs include:
-upper airway sxs MC
- purulent/bloody nasal discharge
- oral/nasal ulcers
Granulomatosis w/ polyangiitis
What do PFTs in Granulomatosis w/ Polyangiitis look like?
- Restrictive & obstructive patterns found
- decreased DLCO
Are you more likely to see hilar adenopathy on CXR in Sarcoidosis or Granulomatosis w/ Polyangiitis?
Sarcoidosis
(uncommon in Granulomatosis with Polyangiitis)
Which dz would you see irregular and stellate-shaped peripheral pulmonary arteries on CT?
Granulomatosis with Polyangiitis

Which dz has the following lab results?
- elevated ESR
- elevated BUN/Cr, proteinuria (if renal involvement)
- +ANCA (Antineutrophil Cytoplasmic Antibodies), usually C-ANCA
Granulomatosis w/ Polyangiitis
Which dz has histopathologic evidence of vasculitis and granulomatous inflammation?
Granulomatosis w/ Polyangiitis
Management of which dz:
- Refer to Rheumatologist
- may tx w/ immunosuppressant (cyclophosphamide) and glucocorticoid
what is prognosis?
Granulomatosis w/ Polyangiitis
(prognosis is 80% w/ cyclophosphamide)
Which is MC of the 7 idiopathic interstitial pneumonias?
Idiopathic Pulmonary Fibrosis
Which dz?
- inflammation & fibrosis of lung parenchyma
- no specific findings- dx after excluding other causes of ILD
- 60 y/o male
- smoker
Idiopathic Pulmonary Fibrosis
which dz?
CXR: Bilateral diffuse reticular or reticulonodular infiltrates, periphery & bases
Idiopathic pulmonary fibrosis
Which dz has insidious onset: exertional dyspnea and nonproductive cough
Idiopathic Pulmonary Fibrosis
What does idopathic pulmonary fibrosis show on HRCT?
reticular opacities
Histopathology of which dz?
predominantly fibrosis and scant numbers of inflammatory cells
Idiopathic Pulmonary Fibrosis
(this is how you distinguish from interstitial pneumonia)
What is the management for Idiopathic Pulmonary Fibrosis?
- Consult pulmonologist (no tx)
- Eval for lung transplant (MC indication for lung transplant)
Possible tx of which dz?
Azithromycin (macrolide) + Ethambutol + Rifampin
usually x15-18mo (until sputum cultures consecutively neg for at least 12 months)
MAC pulmonary disease
(not all patients need tx at time of dx- hard to tolerate, may decide to monitor closely instead)