Treatment of lung disease in alpha-1 antitrypsin deficiency: a systematic review

Figures & data

Figure 1 PICO chart detailing inclusion in systematic review.

Note: PICO assessed are shown.
Abbreviations: AATD, alpha-1 antitrypsin deficiency; AAT, alpha-1 antitrypsin; Pi, protease inhibitor; COPD, chronic obstructive pulmonary disease; PICO, population, interventions, comparators, and outcomes.

Figure 2 PRISMA flow diagram.

Notes: Adapted from Moher et al.²⁵ Articles were excluded where there were insufficient data in the public domain for the study to be assessed against the inclusion criteria including data from ClinicalTrials.gov.
Abbreviation: COPD, chronic obstructive pulmonary disease.

Figure 3 Forest plots of the objective results from meta-analysis of augmentation trials.

Notes: (A) Mean annual change in lung density. (B) Mean FEV₁ % predicted. (C) Standardized mean difference in DLCO. Differences in units used for DLCO (mmol/min/kPa and mL/mmHg/min) and the use of percentage change from baseline in RAPID, but annual change in the other studies required the use of a standardized mean difference plot.

Abbreviations: FEV₁, forced expiratory volume in one second (L); DLCO, diffusing capacity of the lungs for carbon monoxide; mmol/min/kPa, millimole per minute per kilopascal; mL/mmHg/min, milliliter per millimeter of mercury per minute; SD, standard deviation; CI, confidence interval; df, degrees of freedom; Std, standard; IV, inverse variance.

Figure 4 Forest plots for patient-reported outcomes.

Notes: (A) Annual patient-reported exacerbation episodes. (B) Health status: SGRQ, measures recorded as change from baseline.
Abbreviations: SGRQ, St Georges Respiratory Questionnaire; SD, standard deviation; CI, confidence interval; df, degrees of freedom; IV, inverse variance.

Figure 5 Risk of bias in included studies.

Notes: (A) Risk of bias in augmentation RCTs. (B) In view of the large number of studies and the differing scales for assessment of risk based on the study design, a summary of the relative risk of bias is shown across all included studies, other than the RCTs of augmentation. This represents review authors’ judgments about each risk of bias item presented as the mean percentage across all included studies. Individual bias assessments are available in the Supplementary materials.
Abbreviation: RCT, randomized controlled trial.

MoherDLiberatiATetzlaffJAltmanDGPRISMA GroupPreferred reporting items for systematic reviews and meta-analyses: the PRISMA statementPLoS Med200967e100009719621072

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