Table 2 Examples of studies examining QOL in PIDD patients

Quality of life in PIDD Patients

Al-Herz et al. [69]

IVIG

Pediatric

98

LPPS

Patients with PIDD had poor performance status. Variables shown to have a positive effect on LPPS score included antimicrobial prophylaxis and IVIG therapy

Tcheurekdijian et al. [70]

IVIG

Adults

58

SF-36

Compared with age and sex matched diabetic and congestive heart failure patients, CVID patients on IVIG had significantly lower scores in general health and mental health. Compared with diabetic patients, CVID patients scored lower across all subscales with statistical significance in 6 of the 8 domains

Zebracki et al. [20]

IVIG

Pediatric

72

CHQ-PF50

Compared with age matched normal controls, PIDD patients on IVIG reported greater limitations in physical functioning, school related and social activities, general health, parental time emotional distress and limitations in family activities

Soresina et al. [18]

IVIG

Pediatric

25

PedsQL 4.0

X-linked agammaglobulinemia children had a significantly lower total score of HRQOL, psychosocial health, emotional functioning, social functioning and school functioning compared to healthy controls

Aghamohammadi et al. [21]

IVIG

Adults

36

SF-36

PIDD patients scored lower in physical and mental components compared to healthy controls. A reverse association was also noted between SF-36 scores and number of infectious episodes and long delays in diagnosis

Tabolli et al. [19]

Both

Adult and pediatric

96

SF-36;

GHQ-12

CVID patients scored lower on the GHQ-12 than patients with other chronic diseases. Over 6 years of longitudinal assessment, a decline in the score on SF-36 scales was observed between the first and the last assessment for the Physical Functioning, Body Pain, General Health, Social Functioning, and Role-Emotional scales. The relative risk (RR) of death associated with PF and SF scales were noted to be 0.98 and 0.97 respectively, independent of patient age

IVIG in Home versus Hospital/Doctor’s Office

Daly et al. [9]

IVIG- home based, self-infused

Adult and Pediatric

66 total

 37 home

 29 clinic

LQI (Daly);

HOS;

MHLC

Home based IVIG group revealed improved independence, convenience, comfort, decreased disruption of activities, travel time and costs

Espanol et al. [61]

IVIG, SCIG

Adult and Pediatric

300 total

 160 IVIG

 134 SCIG

 6 other IG

 150 home

SF-12;

EQ-5D

Patients and caregivers prefer home treatment and less frequent self-administration of shorter duration with fewer needle sticks. Respondents receiving IVIG and those receiving SCIG had similar results though respondents receiving SCIG had a relatively higher preference for self-administration

IVIG versus SCIG

Berger et al. [71]

SCIG previously on IVIG

Adult and pediatric

51

SF-36;

CHQ-PF50

Adult patients had improvement in general health perception, vitality and mental health at 6 months and general health perception at 12 months. Children had improvements in general health at 6 and 12 months

Fasth et al. [72]

SCIG previously on IVIG

Pediatric

12

CHQ (Swedish version)

Patients and the parents of patients receiving SCIG had improvements in child-rated global health and social limitations subscale scores and parent-rated mental health, child’s general health and emotional impact and had significantly fewer days off school and workdays

Fasth et al. [73]

SCIG previously on IVIG

Pediatric

12

CHQ (Swedish version)

Parents noted significant improvements at 6 months in the child’s mental health, a change in health from 1 year ago and family activities. From the children’s perspective at 6 months there were significant improvements in global health and in role limitations and emotional social limitations

Hoffman et al. [39]

SCIG

Previously on IVIG

Adult and pediatric

30

SF 36;

CHQ-PF50

There was a significant improvement in general health perceptions, parental impact-emotional, parental impact-time, and family activities. At follow-up, 92 % of adults stated a preference for SCIG over IVIG therapy and 83 % preferred home therapy over therapy in clinic setting

Nicolay et al. [10]

SCIG vs IVIG

Adult and pediatric

58 (47 on IVIG prior to study)

LQI (Daly);

CHQ-PF50;

SF-36

In adults there was significantly increased satisfaction in scales of treatment interference and therapy setting in patients switching from IVIG to SCIG

Vultaggio et al. [52]

SCIG previously on IVIG and alternate SCIG

Adult and pediatric

40 (44 on IVIG prior to study, 6 on alternate SCIG prior to study)

LQI;

CHQ-PF50;

SF-36

In patients changing from IVIG to SCIG, no significant difference was seen in the SF-36 for patients over 14 years old or in the CHQ-PF50 answered by the caregivers of patients less than 14 years old but in both groups total mean LQI significantly improved at 6 months and the improvement was sustained over the 24 month follow-up period