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Longitudinal Follow-up of Patients With ₁-Protease Inhibitor Deficiency Before and During Therapy With IV ₁-Protease Inhibitor^*

Marion Wencker, MD; Bernward Fuhrmann, MD; Norbert Banik, MD, PhD; Nikolaus Konietzko, MD, PhD, and for the Wissenschaftliche Arbeitsgemeinschaft zur Therapie von Lungenerkrankungen

^* 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{at}aol.com

Background: The efficacy of IV augmentation therapy with human ₁-protease inhibitor (₁-Pi) in patients with severe ₁-Pi deficiency is still under debate.

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

Design: Multicenter, retrospective cohort study.

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

Patients: Ninety-six patients with severe ₁-Pi deficiency receiving weekly augmentation therapy with human ₁-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%, IV 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 ₁-Pi deficiency and well-preserved lung function show a rapid decline in FEV₁. These patients profit from weekly IV therapy with human ₁-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.

Key Words: ₁-antitrypsin • ₁-antitrypsin deficiency • augmentation therapy • emphysema • lung function • smoking

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