Table 1.

Study eligibility criteria of the SLR

CriteriaInclusion criteriaExclusion criteria
Population •Humans with CVID •Studies with ≤10 patients (unless intervention was described) 
•Animal studies 
•In vitro studies 
Interventions/comparators •No restriction, as long as noninfectious, nonmalignant manifestations were reported ​ 
Outcomes •Description of the clinical profile of patients, including: •Studies focused on vaccine response or IRT 
 o Median age and range of symptom onset (including for individual immune dysregulation manifestations when reported) and CVID diagnosis (separating pediatric and adult patients when reported) 
 o Genetic diagnoses 
 o Frequency of patients with: 
  Distinct immune dysregulation manifestations 
  Infections 
  Other reported manifestations 
 o Mortality and causes of death 
•Description of the current treatment landscape for patients with CVID, including treatment agents and responses 
Study design •Randomized controlled trials •SLRsa 
•Nonrandomized clinical trials (e.g., single-arm trials) •Narrative reviews 
•Prospective and retrospective observational studies •Letters to editor, notes, and editorials 
•Case-control studies •Case reports and case series (unless intervention was described) 
Publication type •Full-text publications ​ 
•Conference abstracts/postersb ​ 
Language •Studies published in English only ​ 
Time •No time restriction ​ 
CriteriaInclusion criteriaExclusion criteria
Population •Humans with CVID •Studies with ≤10 patients (unless intervention was described) 
•Animal studies 
•In vitro studies 
Interventions/comparators •No restriction, as long as noninfectious, nonmalignant manifestations were reported ​ 
Outcomes •Description of the clinical profile of patients, including: •Studies focused on vaccine response or IRT 
 o Median age and range of symptom onset (including for individual immune dysregulation manifestations when reported) and CVID diagnosis (separating pediatric and adult patients when reported) 
 o Genetic diagnoses 
 o Frequency of patients with: 
  Distinct immune dysregulation manifestations 
  Infections 
  Other reported manifestations 
 o Mortality and causes of death 
•Description of the current treatment landscape for patients with CVID, including treatment agents and responses 
Study design •Randomized controlled trials •SLRsa 
•Nonrandomized clinical trials (e.g., single-arm trials) •Narrative reviews 
•Prospective and retrospective observational studies •Letters to editor, notes, and editorials 
•Case-control studies •Case reports and case series (unless intervention was described) 
Publication type •Full-text publications ​ 
•Conference abstracts/postersb ​ 
Language •Studies published in English only ​ 
Time •No time restriction ​ 
a

Bibliographies of relevant systematic reviews were reviewed.

b

Conference abstract/poster citations captured through search of main databases were excluded; they were searched separately in the Northern Light database or published proceeding from target conferences.

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