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INTRODUCTION

4/15/2025 - Medical Grand Rounds: Alpha 1 antitrypsin deficiency: a clue into what causes COPD?

QUIZ

EVALUATION

CERTIFICATE

INTRODUCTION

Credit Hours: CME 1.00

Target Audience:

Faculty, residents, fellows, and community physicians in General Internal Medicine and subspecialties.

Educational Objectives:

Upon completion of this activity, participants should be able to:

    • Explain AATD.
    • Analyze the connection between COPD and AATD.
    • Develop connections and collaborations with RCSI and the University of Pittsburgh.

Suggested Additional Reading & Joint Accreditation Statement - Note: This Accreditation Statement Supersedes All Other Statements:

    Suggested Additional Reading:
    1. Hubbard RC, McElvaney NG, Sellers SE, et al. Recombinant DNA-produced alpha 1-antitrypsin administered by aerosol augments lower respiratory tract antineutrophil elastase defenses in individuals with alpha 1-antitrypsin deficiency. J Clin Invest. 1989 Oct;84(4):1349-54.
    2. McElvaney NG, Nakamura H, Birrer P, et al. Modulation of airway inflammation in cystic fibrosis. In vivo suppression of interleukin-8 levels on the respiratory epithelial surface by aerosolization of recombinant secretory leukoprotease inhibitor. J Clin Invest. 1992 Oct;90(4):1296-301.
    3. McElvaney NG, Stoller JK, Buist AS, et al. Baseline characteristics of enrollees in the National Heart, Lung and Blood Institute Registry of alpha 1-antitrypsin deficiency. Alpha 1-Antitrypsin Deficiency Registry Study Group. Chest. 1997 Feb;111(2):394-403
    4. Taggart C, Cervantes-Laurean D, Kim G, et al. Oxidation of either methionine 351 or methionine 358 in alpha 1-antitrypsin causes loss of anti-neutrophil elastase activity. J Biol Chem. 2000 Sep 1;275(35):27258-65.
    5. Molloy K, Hersh CP, Morris VB, Carroll TP, et al. Clarification of the risk of chronic obstructive pulmonary disease in α1-antitrypsin deficiency PiMZ heterozygotes. Am J Respir Crit Care Med. 2014 Feb 15;189(4):419-27.
    6. Bergin DA, Reeves EP, Hurley K, et al. The circulating proteinase inhibitor α-1 antitrypsin regulates neutrophil degranulation and autoimmunity. Sci Transl Med. 2014 Jan 1;6(217):217ra1
    7. Chapman KR, Burdon JG, Piitulainen E, et al; RAPID Trial Study Group. Intravenous augmentation treatment and lung density in severe α1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trial. Lancet. 2015 Jul 25;386(9991):360-8.
    8. McElvaney NG, Burdon J, Holmes M, et al; RAPID Extension Trial Group. Long-term efficacy and safety of α1 proteinase inhibitor treatment for emphysema caused by severe α1 antitrypsin deficiency: an open-label extension trial (RAPID-OLE). Lancet Respir Med. 2017 Jan;5(1):51-60.
    9. Strnad P, McElvaney NG, Lomas DA. Alpha1-Antitrypsin Deficiency. N Engl J Med. 2020 Apr 9;382(15):1443-1455
    10. Franciosi AN, Hobbs BD, McElvaney OJ, et al. Clarifying the Risk of Lung Disease in SZ Alpha-1 Antitrypsin Deficiency. Am J Respir Crit Care Med. 2020 Jul 1;202(1):73-82.
    11. Fraughen DD, Ghosh AJ, Hobbs BD, et al. Augmentation Therapy for Severe Alpha-1 Antitrypsin Deficiency Improves Survival and Is Decoupled from Spirometric Decline-A Multinational Registry Analysis. Am J Respir Crit Care Med. 2023 Nov 1;208(9):964-974.

Joint Accreditation Statement
In support of improving patient care, the University of Pittsburgh is jointly accredited by the Accreditation Council for Continuing Medical Education (ACCME), the Accreditation Council for Pharmacy Education (ACPE), and the American Nurses Credentialing Center (ANCC), to provide continuing education for the healthcare team.


Physician (CME)
The University of Pittsburgh School of Medicine designates this enduring material activity for a maximum of 1.0 AMA PRA Category 1 Credits™.Each physician should only claim credit commensurate with the extent of their participation in the activity.


Other health care professionals will receive a certificate of attendance confirming the number of contact hours commensurate with the extent of participation in this activity.

Authors:
Noel G. McElvaney, MD — Professor of Medicine, Head of School of Medicine, Royal College of Surgeons in Ireland (RCSI)
No relationships with industry relevant to the content of this educational activity have been disclosed.
No other members of the planning committee, speakers, presenters, authors, content reviewers and/or anyone else in a position to control the content of this education activity have relevant financial relationships with any companies whose primary business is producing, marketing, selling, re-selling, or distributing healthcare products used by or on patients.

This activity is approved for AMA PRA Category 1 Credit™

The University of Pittsburgh is an affirmative action, equal opportunity institution.