Restrictive lung disease + neoplasms Flashcards
What is the overarching umbrella of restrictive disease in lungs that is exposure, autoimmune, and idiopathic related w/ insidious onset of progressive dyspnea + nonproductive cough, tachypnea, small lung volumes, bibasilar dry rales, digital clubbing, RHF w/ advanced disease?
interstitial lung disease
What does clubbing in the hands indicate?
years of hypoxia
What would you expect in a restrictive lung disease’s PFT?
REDUCED lung volumes, low DLCO, high FEV1/FVC ratio
What would you see on a CXR of interstitial lung disease?
low lung volumes + patchy distribution of ground glass, reticular, nodular, reticulonodular, or cystic opacities
What’s the standard for diagnosis of diffuse lung disease?
surgical biopsy w/ 2-3 biopsies from multiple sites, usually in patients <60
and can consider bronchoalveolar lavage in cases of infection or malignancy
What should you consider diagnosis in patients >65 w/:
- Idiopathic disease by history and who have inspiratory crackles
- PFT = restrictive
- Characteristic radiographic evidence of progressive fibrosis over several years
- AND diffuse patchy fibrosis w/ pleural-based honeycombing on high resolution CT scan
with symptoms of: DOE, cough, fatigue (gradual onset)?
idiopathic pulmonary fibrosis
In who is idiopathic pulmonary fibrosis common?
men>50, smoking
T/F: idiopathic pulm fibrosis patients need a lung biopsy to confirm diagnosis
F
What would these diagnostic studies indicate?
CT = predominantly basilar, peripheral (bronchiectasis = upper) pattern of dilated bronchiectasis, reticulation, and early honeycombing
PFT: restrictive pattern w/ increased FEV1/FVC + decreased lung volumes
idiopathic pulmonary fibrosis
How do you manage idiopathic pulmonary fibrosis?
pulmonologist
Nintedanib + pirfenidone (reduce lung function decline but do not improve survival, $$)
Oxygen, pulmonary rehab, monitoring pulm HTN
Only definitive treatment = lung transplant
What causes sarcoidosis?
systemic granulomatous inflammation forming in multiple organs (we don’t know why) – lungs, lymph nodes, skin, eyes, nerves, liver, kidney, heart
What could a patient with sarcoidosis come in complaining about?
malaise, night sweats, fever, dyspnea, cough, CP
What is stage 1 of sarcoidosis?
bilateral hilar adenopathy alone
What is stage 2 of sarcoidosis?
hilar adenopathy AND parenchymal involvement (diffuse reticular infiltrates, focal infiltrates, bronchiole changes, nodules, pleural effusion)
What is stage 3 of sarcoidosis?
parenchymal involement alone
What is stage 4 of sarcoidosis?
advanced fibrotic changes in upper lobes
In who is sarcoidosis prominent?
30s or 40s onset, african americans
What could this PE indicate:
- atypical
-crackles uncommon but skin involved w/ erythema nodosum, lupus pernio, iritis, peripheral neuropathy, arthritis, cardiomyopathy
- parotid gland enlargement, hepatosplenomegaly, lymphadenopathy
sarcoidosis
How can you confirm diagnosis of sarcoidosis?
clinical findings + consistent XR (bilateral hilar + right paratracheal lymphadenopathy) and biopsy evidence of noncaseating granuloma from safest organ
BAL fluid = increase in lymphocytes + high CD4/CD8 ratio - monitoring and ophthalmologic evaluation
What could this indicate:
Lymphopenia, elevated ESR, hypercalcemia, hypercalciuria, elevated LFTs
PFTS = restrictive changes w/ decreased lung volumes + diffusing capacity (may show airflow obstruction)
ECG = conduction disturbances & dysrhythmias
sarcoidosis
What are these indications for:
Disabling constitutional symptoms
Hypercalcemia
Iritis
Uveitis
Arthritis
CNS involvement
Cardiac involvement
Granulomatous hepatitis
Cutaneous lesions other than erythema nodosum
Progressive pulmonary lesions
sarcoidosis = oral steroids - prednisone w/ taper
If someone who has sarcoidosis is intolerant of steroids what do you give instead?
methotrexate, azathioprine, infliximab
What does long-term follow up look like for sarcoidosis?
yearly physical exam and PFTs, chemistry panel, ophthalmologic evaluation, CXR, ECG
What should you think if a patient presents asymptomatic w/ slightly affected pulmonary function or progressive, massive fibrosis w/ conglomeration + contraction in upper lungs and is characteristic of Caplan syndrome and could be from the inhalation of inorganic dusts, smoking does not?
coal worker’s pneumoconiosis
What would you expect on CXR and PFT for coal worker’s pneumoconiosis?
CXR: formation of coal macules as diffuse small opacities in upper lungs w/ hyperinflation of lower lobes in an obstructive pattern (resembling emphysema)
PFT: obstructive pattern