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Natural history of AATD in adults
– not only COPD
Paweł Kuca
Warsaw, 17-10-2015
Introduction (1)
Clinical presentations that may be consistent
with AATD
Emphysema
- early onset, no smoking, basilar predominance, family history
Other lung disease
- bronchiectasis of unclear etiology
Liver disease
Other
- necrotizing panniculitis
- anti-proteinase 3-positive vasculitis (C-ANCA)
Introduction (2)
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•
•
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Asthma
Bronchiectasis
Lung cancer
COPD-associated cardiovascular risk
Asthma
• Asthma in patients with AATD
• Reversibility/ BHR in patients with AATD
• AAT in patients with asthma
Bronchiectasis
• Bronchiectasis in patients
with AATD
• AATD in patients with
bronchiectasis
Figure 3.
Top: Lower zone image (at the level of the inferior pulmonary veins) from a
subject with severe cystic bronchiectasis and mild emphysema.
Bottom: Quantitative analysis of emphysema, with the low-density voxels
highlighted in pink (Pulmo-CMS; Medis Medical Imaging Systems).
Lung cancer
• AATD status and lung cancer risk
• Emphysema as risk factor for lung cancer
• AATD in advanced lung cancer
COPD-associated cardiovascular risk
• AAT and cardiovascular risk in population
• AATD and aortic stiffness
• AATD and systemic inflammation
General conclusions (1)
• Q1: Does suboptimal AAT status predispose
to other lung diseases?
• Yes,
- asthma,
- bronchiectasis,
- lung cancer etc.
General conclusions (2)
• Q 2: Is the list of AATD-related lung
diseases now closed?
• No !
- TB, NTM-infections,
- idiopathic pulmonary fibrosis,
- pneumothorax,
- pulmonary vasculitis etc.
Natural history of AATD in adults
- AATD related lung disease
(airways remodeling, parenchyma destruction
- radiological and functional evidence)
end stage phase
chronic systemic inflammation
exacerbations
colonisation (Pseudomonas, NTM)
RF (blue blotter), RHF, RF (pink puffer), lung cancer
cardiovascular risk (MI, stroke)
late phase
asthma (no radiological signs, functional reversibility)
smoking/ infections
chronic bronchitis, bronchiectasis, emphysema
early phase
AATD status