PMID- 35361631
OWN - NLM
STAT- MEDLINE
DCOM- 20221031
LR  - 20221129
IS  - 1399-3003 (Electronic)
IS  - 0903-1936 (Linking)
VI  - 60
IP  - 4
DP  - 2022 Oct
TI  - Cancer risk in severe alpha-1-antitrypsin deficiency.
LID - 2103200 [pii]
LID - 10.1183/13993003.03200-2021 [doi]
AB  - BACKGROUND: Severe alpha-1-antitrypsin deficiency (AATD), phenotype PiZZ, is a 
      risk factor for pulmonary emphysema and liver disease, but its effect on cancer 
      risk is unknown. Our aim was to evaluate the risk and the risk factors for 
      incident cancer in PiZZ individuals compared with the general population with 
      known smoking habits. METHODS: A longitudinal study of PiZZ individuals (n=1595) 
      from the Swedish National AATD Register, and controls (n=5999) from Swedish 
      population-based cohorts. Data on cancer and mortality were obtained by 
      cross-linkage with national registers. Individuals who had undergone lung 
      transplantation (n=10) and those with a cancer diagnosis within 5 years prior to 
      inclusion (n=63) were excluded. The risk factors for developing cancer were 
      analysed using proportional hazards and Fine-Gray regression models, adjusting 
      for age, sex, smoking habits and the presence of liver disease. RESULTS: The 
      median follow-up time was 17 years (interquartile range 11 years) for the whole 
      study population. The incidence rates of hepatic and non-hepatic cancer per 1000 
      person-years were 1.6 (95% CI 1.1-2.3) and 8.5 (95% CI 7.2-10.0), respectively, 
      for the PiZZ individuals, and 0.1 (95% CI 0.04-0.2) and 6.6 (95% CI 6.0-7.1), 
      respectively, for the controls. The adjusted hazard ratios for hepatic and for 
      non-hepatic cancer were 23.4 (95% CI 9.9-55.4) and 1.3 (95% CI 1.1-1.5), 
      respectively, in the PiZZ individuals compared with the controls. CONCLUSION: 
      These results suggest that individuals with severe AATD may have an increased 
      risk of developing both hepatic and non-hepatic cancer, compared with the general 
      population.
CI  - Copyright (c)The authors 2022. For reproduction rights and permissions contact 
      permissions@ersnet.org.
FAU - Hiller, Adriana-Maria
AU  - Hiller AM
AUID- ORCID: 0000-0002-5452-0314
AD  - Dept of Respiratory Medicine and Allergology, Lund University, Skane University 
      Hospital, Malmo, Sweden adriana-maria.hiller@med.lu.se.
FAU - Ekstrom, Magnus
AU  - Ekstrom M
AUID- ORCID: 0000-0002-7227-5113
AD  - Lund University, Faculty of Medicine, Dept of Clinical Sciences Lund, Respiratory 
      Medicine and Allergology, Lund, Sweden.
FAU - Piitulainen, Eeva
AU  - Piitulainen E
AD  - Dept of Respiratory Medicine and Allergology, Lund University, Skane University 
      Hospital, Malmo, Sweden.
FAU - Lindberg, Anne
AU  - Lindberg A
AUID- ORCID: 0000-0002-3292-7471
AD  - Dept of Public Health and Clinical Medicine, Section of Medicine, the OLIN unit, 
      Umea University, Umea, Sweden.
FAU - Ronmark, Eva
AU  - Ronmark E
AUID- ORCID: 0000-0002-2358-8754
AD  - Dept of Public Health and Clinical Medicine, Section of Sustainable Health, the 
      OLIN unit, Umea University, Umea, Sweden.
FAU - Tanash, Hanan
AU  - Tanash H
AD  - Dept of Respiratory Medicine and Allergology, Lund University, Skane University 
      Hospital, Malmo, Sweden.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20221027
PL  - England
TA  - Eur Respir J
JT  - The European respiratory journal
JID - 8803460
SB  - IM
CIN - Eur Respir J. 2022 Nov 3;60(5):. PMID: 35595316
CIN - Eur Respir J. 2022 Nov 3;60(5):. PMID: 35595322
CIN - Eur Respir J. 2022 Oct 27;60(4):. PMID: 36302522
MH  - Humans
MH  - Longitudinal Studies
MH  - *alpha 1-Antitrypsin Deficiency/complications/epidemiology/diagnosis
MH  - *Pulmonary Emphysema/complications/epidemiology/diagnosis
MH  - Smoking/adverse effects/epidemiology
MH  - Risk Factors
MH  - *Neoplasms/epidemiology
COIS- Conflict of interest: A. Lindberg reports lecture honoraria from Boehringer 
      Ingelheim and Novartis; personal fees for advisory board participation with 
      AstraZeneca, GlaxoSmithKline, Novartis and Boehringer Ingelheim; outside the 
      submitted work. All other authors have nothing to disclose.
EDAT- 2022/04/02 06:00
MHDA- 2022/11/01 06:00
CRDT- 2022/04/01 05:34
PHST- 2021/12/19 00:00 [received]
PHST- 2022/03/12 00:00 [accepted]
PHST- 2022/04/02 06:00 [pubmed]
PHST- 2022/11/01 06:00 [medline]
PHST- 2022/04/01 05:34 [entrez]
AID - 13993003.03200-2021 [pii]
AID - 10.1183/13993003.03200-2021 [doi]
PST - epublish
SO  - Eur Respir J. 2022 Oct 27;60(4):2103200. doi: 10.1183/13993003.03200-2021. Print 
      2022 Oct.