- Genetic d/o that leads to lung destruction and liver dysfxn
- Episodic exacerbations
- Similar to COPD but no/limited smoking hx
- Chronic progressive liver dz
- Alpha-1 anti-trypsin level low, genetic testing
- Lung Disease: CXR, PFTs
- Elevated alt/ast, other liver tests
- Rule out other liver disease with labs, imaging: NAFLD/NASH, ETOH, hep B, Hep C, Wilson's Dz, hemochromatosis, auto-immune hepatitis, DILI, other