Which histopathologic pattern of interstital lung involvement is characterized by heterogenous involvement of the lung with different stages of progression of fibrosis in adjacent areas of the lung?
Usual Interstitial Pneumonitis (UIP)
A pt presenting with progressive dyspnea (over months), reduced exercise tolerance, and a persistent dry cough should raise suspicion for what?
Interstitial lung disease
Which imaging modality is a crucial tool for the diagnostic evaluation of interstitial lung diseases?
Interstital lung disease evaluation must include hx of what 5 things?
- Drugs both Rx and non-Rx
Which lung sound auscultated in a patient with interstitial lung disease is almost pathognomonic?
Crackles often described as "velcro" developing in the bases throughout inspiration
What are the prototypic sign/sx's of interstitial lung disease?
- Dyspnea + cough + crackles
- Digital clubbing (presence does not confirm and absence does not eliminate ILD)
- Exercise induced hypoxemia
What is the FRC, TLC, FEV1/FVC and DLCO like with interstitial lung diseases?
- ↓ FRC and ↓ TLC
- Normal FEV1/FVC
- Low DLCO
Very low pleural glucose is characteristics of pleural disease associated with what?
Shrinking lung syndrome is associated with what systemic disease?
Which 3 autoantibodies may be seen in Scleroderma; which is most common in those with ILD; which is associated with worst prognosis?
- SCL-70 = more common in ILD
- Antinucleolar = predicts worst prognosis
Which autoantibody is seen in >90% of drug induced SLE cases?
Which autoantibody is seen in 90-95% of SLE cases?
Which autoantibody is seen in pt's with SLE induced nephritis?
Which antiarrhythmic and antibiotic used for UTI's are commonly associated with drug induced interstitial lung disease?
When is the early phase vs. late phase of radiation induced lung disease?
- Early phase = 1-3 months after radiation
- Late phase = 6-12 months
What is the rate of decline and cough like in ILD vs. COPD?
- COPD = slower decline w/ more frequent exacerbations + productive cough
- ILD = more rapid decline + non-productive cough
What is the mainstay drug used to tx non-IPF induced ILD?
What are the 2 anti-fibrotic agents which are ONLY used for idiopathic pulmonary fibrosis; describe each drug?
1) Pifenidone = antifibrotic and anti-inflammatory
2) Nintedanib = tyrosine kinase inhibitor targets inflammatory and profibrotic factors
What is the characteristic finding on a CXR of someone with acute interstitial pneumonia (aka Hamman-Rich syndrome)?
Diffuse, symmetrical, and bilateral
What is the onset like with acute interstitial pneumonia (aka Hamman-Rich syndrome) vs. IPF?
- AIP --> abrupt onset w/ flu-like sx's
- IPF --> gradual onset, afebrile
Acute interstitial pneumonia (aka Hamman-Rich syndrome) vs. IPF occurs in which age groups?
- AIP --> occurs in patients from all age groups, mostly adults
- IPF --> occurs almost exclusively in adults
How is the diagnosis of acute interstitial pneumonia (aka Hamman-Rich syndrome) vs. IPF established?
- AIP --> BIOPSY establishes diagnosis
- IPF --> hx/clinical findings, imaging, and PFTs; biopsy optional