Table 3.
Clinical characteristics of included studies.
| Reference | Country | Type of PID | Ig treatment and place of administration | Brand and dose | Sample | n | Mean age and range |
|---|---|---|---|---|---|---|---|
| Chapel et al. (23) | UK and Sweden | CVID 18; IgG subclass deficiency 10; specific antibody deficiency 2 | SCIg (setting unclear); IVIg (clinic-based) | SCIg: Gammabulin; IVIg: Endobulin (doses 400 mg/kg/month in UK, 600 mg/kg/month in Sweden) | Adults (>13 years), previously untreated or previously on prophylactic Ig therapy | 30 | 44; range 18–67 |
| Daly et al. (17) | USA | Not reported | IVIg (home- or hospital-based) | Sandoglobulin (dose not reported) | Participants in a trial of home-based IVIg and patients treated in a clinic setting at the same hospitals | 37 home-based; 29 clinic-based | 35.5 (home-based); 32.5 (clinic-based) |
| Dash et al. (24) | UK | CVID, IgGsubclasss deficiency, specific antibody deficiency, IGG heavy chain deficiency | SC (home) | Subgam (manufactured by BLP), initial weekly dose 100 mg/kg bodyweight—dose then individually adjusted | Adults and children enrolled in a trial of efficacy and safety of Subgam for home-infusion | 28 adults | 45.5 (21.3–75.2) |
| Gardulf et al. (26) | Sweden | Hypogammaglobulinaemia (further details not reported) | SCIg (home-based) | Gammaglobulin Kabi (100 mg/kg/week) | Consecutive patients receiving SCIg by rapid infusion at home | 25 | 43; range 18–73 |
| Gardulf et al. (20) | Sweden | CVID 23; XLA 1; other 1 | SCIg (home-based) | Brand not reported (100 mg/kg/week) | Adults (≥18) with hypogammaglobulinaemia | 25 | 43 (SD 16); range 18–66 |
| Gardulf et al. (14) | Sweden; Denmark; and Norway | Not reported | SCIg (home-based) | Gammaglobulin Kabi (165 mg/ml); Gammabulin (160 mg/ml); and Nordimmun (150 mg/ml) | Adults receiving ongoing treatment with SCIg | 152 | 44; range 18–76 |
| Gardulf et al. (25) | Sweden | CVID or XLA (numbers not reported) | SCIg (home-based) | Brand not reported (100 mg/kg/week) | Adults who switched from hospital to home therapy | 30 | 43; range 18–66 |
| Gardulf et al. (30) | Sweden | cVID 6; IgG subclass deficiency 2; IgA and IgG2 subclass deficiency 1 | SCIg (home-based) | Gammaglobulin or gammanorm (100 mg/kg/week) | Women who became pregnant while receiving SCIg at home | 9 | Mean not reported; range 25–43 |
| Hansen et al. (27) | Sweden | IgG subclass deficiency 29; selective IgA deficiency 9; CVID 3; XLA 1; others 8 | SCIg (home-based) | Gammanorm or immunoglobulin Baxter (100 mg/kg/week) | Patients who had been on rapid self-infusions at home for at least 6 months | 50 | Median 48; range 23–74 |
| Howard et al. (15) | USA | XLA | IVIg (home- or hospital-based) | Not reported | Patients under care of the authors or who had participated in previous research studies | 41 | 33; range 21–63 |
| Jones et al. (13) | USA | CVID | SCIg (home-based) | Hizentra (dose not reported) | Patients from a previous study who agreed to enroll in an extension study | 21 enrolled, 16 analyzed | 47.2 (SD 14.5); range 22–69 |
| Kittner et al. (28) | Germany | CVID 48; IgG subclass deficiency 1; hyper IgM syndrome 1; XLA 2; not stated 9 | SCIg (home-based); IVIg (hospital-based) | Not reported | Patients who had switched to SCIg or opted to continue on IVIg | 61 (33 SCIg and 28 IVIg) | SCIg 37 (SD 9.1); IVIg 51.2 (SD 14.5) |
| Mohamed et al. (19) | USA | CVID 219; XLA 8; other 25 | SCIg or IVIg; home or doctor’s office, hospital, or clinic | Not reported | Patients recruited from the Immune Deficiency Foundation member panel | 252 | 50.2 (SD 13.8); range 19–80 |
| Nicolay et al. (18) | USA and Canada | CVID 34; XLA 10 | SCIg (home-based) | Vivaglobin (160 mg/ml) | Participants in a longitudinal study who agreed to participate in a HRQoL sub-study. Patients had switched to home SCIg from hospital (group A) or home (group B) IVIg | 44 | Group A 36.1 (SD 13.6); group B 35.5 (SD 13.2) |
| Rider et al. (29) | USA | CVID, IgG subclass deficiency, agammaglobulinaemia, “other” PIDs | IV IG, SCIg, Im Ig (at home, infusion suite, hospital, or other) | Not reported | Adult patients registered on the US IDF patient contact database | 945 | Median: 52.9 (range 18–82) |
| Routes et al. (16) | UK; USA; and Brazil | CVID, agammaglobulinaemia, hypogammaglobulinaemia, specific antibody deficiency, and other | IVIg at home or hospital | Not reported | Adult and child patients receiving care via Jeffrey Modell Foundation Treatment Centres in the United States, Brazil, and the United Kingdom | 21 adults | 47.1 (24–67) |
| Tcheurekdjian et al. (22) | USA | CVID | IVIg (home-based and hospital-based) | Not reported | Adult (≥18) patients receiving IVIg therapy via University Hospitals of Cleveland | 58 | 49.9 (SD 15.7); range 19.3–87.4 |
CVID, common variable immune deficiency; IVIg; PID, primary immunodeficiency; SCIg; XLA, X-linked agammaglobulinaemi; HRQoL, health-related quality of life.