Overall survival among patients with activated phosphoinositide 3-kinase delta syndrome (APDS)

Table 1 PICO criteria

Category	Inclusion criteria	Exclusion criteria
Population	• Patients with APDS OR patients with ≥ 1 clinical sign consistent with the clinical spectrum of APDS^aAND a first-degree relative who has a genetically confirmed diagnosis of APDS	• Studies not reporting individual patient data for outcome of interest
Interventions or comparators	• Any	• Not applicable
Outcomes	• Age at last observation • Alive status • Age at death	• No reported outcome of interest
Publication type	• Articles, letters, clinical communications, and case series	• Any other publications
Language	• English language • Study publication date: 2013^b to March 2023	• Studies published in languages other than English or prior to 2013^b

^aClinical signs included documented severe recurrent sinopulmonary infections (> 2 events within 3 years of each other); bronchiectasis; lymphadenopathy for greater than 1 month; any nodular lymphoid hyperplasia; chronic hepatomegaly or chronic splenomegaly; severe, persistent, or recurrent Herpesviridae infections (e.g., Epstein-Barr virus, cytomegalovirus); autoimmune cytopenia; enteropathy; lymphoma; hypogammaglobulinemia; elevated levels of immunoglobulin M; reduced number of CD3⁺CD4⁺ T cells; increased number of follicular helper T cells; reduced number of naïve T cells; clinical diagnosis of CVID or a primary immunodeficiency; evidence of PI3K pathway activation; and additional clinical features within the clinical spectrum of APDS, with a consensus. ^bStudies included were published in 2013 or later, as APDS was characterized in 2013. APDS, activated phosphoinositide 3-kinase delta syndrome; CVID, common variable immunodeficiency; PI3K, phosphoinositide 3-kinase; PICO, population, intervention, comparison, and outcome

ISSN: 1750-1172