0
Clinical Investigations |

Longitudinal Follow-up of Patients With α1-Protease Inhibitor Deficiency Before and During Therapy With IV α1-Protease Inhibitor*

Marion Wencker, MD; Bernward Fuhrmann, MD; Norbert Banik, MD, PhD; Nikolaus Konietzko, MD, PhD,;
Author and Funding Information

*From the Ruhrlandklinik (Drs. Wencker, Fuhrmann, and Konietzko), Department of Pneumology, University Hospital, Essen; and Department of Biostatistics (Dr. Banik), Bayer Vital, Leverkusen, Germany.

A complete list of participants is located in the Appendix.

Correspondence to: Marion Wencker, MD, Department of Pneumology, Ruhrlandklinik, University Clinic, Tueschener Weg 40, 45239 Essen, Germany; e-mail: mwencker@aol.com



Chest. 2001;119(3):737-744. doi:10.1378/chest.119.3.737
Text Size: A A A
Published online

Background: The efficacy of IV augmentation therapy with human α1-protease inhibitor (α1-Pi) in patients with severe α1-Pi deficiency is still under debate.

Study objectives: To evaluate the progression of emphysema in patients with α1-Pi deficiency before and during a period in which they received treatment withα 1-Pi.

Design: Multicenter, retrospective cohort study.

Setting: Outpatient clinics of 26 university clinics and pulmonary hospitals.

Patients: Ninety-six patients with severeα 1-Pi deficiency receiving weekly augmentation therapy with human α1-Pi, 60 mg/kg of body weight, had a minimum of two lung function measurements before and two lung function measurements after augmentation therapy was started. Lung function data were followed up for a minimum of 12 months both before and during treatment (mean, 47.5 months and 50.2 months, respectively).

Measurements and results: Patients were grouped according to the severity of their lung function impairment. The change in FEV1 was compared during nontreatment and treatment periods. In the whole group, the decline in FEV1 was significantly lower during the treatment period (49.2 mL/yr vs 34.2 mL/yr, p = 0.019). In patients with FEV1 > 65%, IVα 1-Pi treatment reduced the decline in FEV1 by 73.6 mL/yr (p = 0.045). Seven individuals had a rapid decline of FEV1 before treatment, and the loss in FEV1 could be reduced from 256 mL/yr to 53 mL/yr (p = 0.001).

Conclusion: Some patients with severe α1-Pi deficiency and well-preserved lung function show a rapid decline in FEV1. These patients profit from weekly IV therapy with human α1-Pi and have less rapid decline if treated. Early detection of patients at risk and early start of augmentation therapy may prevent accelerated loss of lung tissue.

Figures in this Article

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Figures

Tables

References

NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Related Content

Customize your page view by dragging & repositioning the boxes below.

  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543