Physical health conditions and quality of life in adults with primary immunodeficiency diagnosed during childhood: A French Reference Center for PIDs (CEREDIH) study

Barlogis, Vincent; Mahlaoui, Nizar; Auquier, Pascal; Pellier, Isabelle; Fouyssac, Fanny; Vercasson, Camille; Allouche, Maya; Azevedo, Carolina Brito De; Suarez, Félipe; Moshous, Despina; Neven, Bénédicte; Pasquet, Marlène; Jeziorski, Éric; Aladjidi, Nathalie; Schleinitz, N.; Thomas, Caroline; Gandemer, Virginie; Mazingue, Françoise; Lutz, Patrick; Hermine, Olivier; Pïcard, Capucine; Blanche, Stéphane; Gautier, Michel; Fischer, Alain

doi:10.1016/j.jaci.2016.08.027

Cited by 27 publications

(13 citation statements)

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“…A lack of definition and quantification of the burden of some CVID comorbidities in the scientific literature, such as unspecified autoimmunity and certain blood disorders, may have led to additional underestimation of the total CVID disability. A structural and uniform collection of the self-perceived health status in patients with CVID would help to better determine the burden for CVID patients with different clinical phenotypes and has been introduced by individual centers [ 46 , 47 ].…”

Section: Discussionmentioning

confidence: 99%

The burden of common variable immunodeficiency disorders: a retrospective analysis of the European Society for Immunodeficiency (ESID) registry data

Odnoletkova

Kindle

Quinti

et al. 2018

Orphanet J Rare Dis

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115

102

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BackgroundCommon variable immunodeficiency disorders (CVID) are a group of rare innate disorders characterized by specific antibody deficiency and increased rates of infections, comorbidities and mortality. The burden of CVID in Europe has not been previously estimated. We performed a retrospective analysis of the European Society for Immunodeficiencies (ESID) registry data on the subset of patients classified by their immunologist as CVID and treated between 2004 and 2014. The registered deaths and comorbidities were used to calculate the annual average age-standardized rates of Years of Life Lost to premature death (YLL), Years Lost to Disability (YLD) and Disability Adjusted Life Years (DALY=YLL + YLD). These outcomes were expressed as a rate per 105 of the CVID cohort (the individual disease burden), and of the general population (the societal disease burden).ResultsData of 2700 patients from 23 countries were analysed. Annual comorbidity rates: bronchiectasis, 21.9%; autoimmunity, 23.2%; digestive disorders, 15.6%; solid cancers, 5.5%; lymphoma, 3.8%, exceeded the prevalence in the general population by a factor of 34.0, 7.6, 8.1, 2.4 and 32.6, respectively. The comorbidities of CVID caused 8722 (6069; 12,363) YLD/105 in this cohort, whereas 44% of disability burden was attributable to infections and bronchiectasis. The total individual burden of CVID was 36,785 (33,078, 41,380) DALY/105. With estimated CVID prevalence of ~ 1/ 25,000, the societal burden of CVID ensued 1.5 (1.3, 1.7) DALY/105 of the general population.In exploratory analysis, increased mortality was associated with solid tumor, HR (95% CI): 2.69 (1.10; 6.57) p = 0.030, lymphoma: 5.48 (2.36; 12.71) p < .0001 and granulomatous-lymphocytic interstitial lung disease: 4.85 (1.63; 14.39) p = 0.005. Diagnostic delay (median: 4 years) was associated with a higher risk of death: 1.04 (1.02; 1.06) p = .0003, bronchiectasis: 1.03 (1.01; 1.04) p = .0001, solid tumor: 1.08 (1.04; 1.11) p < .0001 and enteropathy: 1.02 (1.00; 1.05) p = .0447 and stayed unchanged over four decades (p = .228).ConclusionsWhile the societal burden of CVID may seem moderate, it is severe to the individual patient. Delay in CVID diagnosis may constitute a modifiable risk factor of serious comorbidities and death but showed no improvement. Tools supporting timely CVID diagnosis should be developed with high priority.Electronic supplementary materialThe online version of this article (10.1186/s13023-018-0941-0) contains supplementary material, which is available to authorized users.

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Section: Discussionmentioning

confidence: 99%

The burden of common variable immunodeficiency disorders: a retrospective analysis of the European Society for Immunodeficiency (ESID) registry data

Odnoletkova

Kindle

Quinti

et al. 2018

Orphanet J Rare Dis

Self Cite

115

102

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“…It has been widely documented that patients with PAD have reduced perceived health and poorer QoL than healthy matched controls [13]. This was evident in both adults [14,15] and in children [16][17][18]. In the case of children with primary immune deficiency, their QoL was considered comparable to that of patients with cancer [17].…”

Section: Introductionmentioning

confidence: 99%

“…While earlier diagnosis and commencement of appropriate immunoglobulin replacement therapy has done much to improve physical health and reduce the frequency of infections [20], there remains a significant reduction in QoL that is related to the number of illnessrelated health conditions experienced by the patient [14,16,17]. In addition, the 'life quality index' was better in some patient populations on home therapy as opposed to hospital based immunoglobulin replacement therapy [21].…”

Section: Introductionmentioning

confidence: 99%

The presence of overlapping quality of life symptoms in primary antibody deficiency (PAD) and chronic fatigue syndrome (CFS)

Bansal¹,

Tadros

Bansal

2020

Allergy Asthma Clin Immunol

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Background: Fatigue, sleep disturbance and altered mood are frequently reported in patients with primary antibody deficiency syndrome (PADS) on adequate immunoglobulin replacement therapy. This study aimed to determine the frequency of symptoms compatible with chronic fatigue syndrome (CFS) in patients with PADS. Methods: The study involved the distribution of 682 self-completed postal questionnaires to ascertain the presence and frequency of symptoms compatible with CFS in patients with PADS. The reporting of symptoms for each patient were scored against the CFS diagnostic criteria used within our own South London Chronic Fatigue service. Results: The frequency of symptoms compatible with CFS were evident in 26 of the 188 patients (16.25%) returning adequately completed questionnaires. We considered a bias in the return of questionnaires amongst PADS patients with fatigue to be likely. As such we estimated the minimum frequency of CFS in patients with PADS to be 4% based on the 682 PAD patients to whom the questionnaire was distributed. This was significantly higher than the 0.5% estimate of the prevalence of CFS in the community in western populations. While the presence of significant fatigue correlated with the presence of anxiety and depression, there was no association with self-reported lung damage. Sleep disturbance affected 60% of the PAD patients returning satisfactory questionnaires and as expected the CFS score was higher in those with greater physical limitation. Conclusions: We conclude that patients with PADS have a high frequency of fatigue, low mood and anxiety. We suggest routine questioning for the symptoms of fatigue, disturbed sleep and altered mood in patients with PADS. The use of several treatment strategies in CFS may prove beneficial in improving the quality of life of patients with PAD.

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“…Furthermore, childhood development and growth have an impact on physical performance in midlife13 and may increase the prevalence of falls in later life 14. Poor childhood health status has a long-range negative influence on adult health outcomes in terms of self-reported health status and successful ageing 15–17. These findings indicate that early life malnutrition may increase the prevalence of poor physical function to a certain extent.…”

Section: Introductionmentioning

confidence: 99%

Association between early-life exposure to the Great Chinese Famine and poor physical function later in life: a cross-sectional study

Tao

Dai

et al. 2019

BMJ Open

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ObjectivesThis study aimed to evaluate the association between early-life exposure to the Great Chinese Famine (1959–1961) and the prevalence of poor physical function in midlife.DesignA population-based historical prospective study was performed as part of a wider cross-sectional survey. Exposure to famine was defined by birthdate, and participants were divided into non-exposed group, fetal-exposed group and infant-exposed group.Setting and participantsA total of 3595 subjects were enrolled into the study from the China Health and Retirement Longitudinal Study (CHARLS) 2015 based on random selection of households that had at least one member aged 45 years old and older in 28 provinces of mainland China.Main outcome measuresPhysical function status was assessed by a six-item self-report on the Barthel scale which rated basic activities of daily living (BADL).Results743 (20.7%) out of all participants were exposed to the Great Chinese Famine in their fetal periods, while 1550 (43.1%) participants were exposed at the age of an infant. The prevalence of poor physical function in the non-exposed group, fetal period-exposed group and infant period-exposed group were 12.3%, 15.5% and 17.0%, respectively. Among males, after stratification by gender and severity of famine, the prevalence of poor physical function in the fetal period was significantly higher (OR 2.40, 95% CI 1.18 to 4.89, p=0.015) than the non-exposed group in severely affected areas, even after adjusting for the number of chronic diseases, place of residence, smoking and alcohol drinking habits, marital status, educational level and body mass index. A similar connection between prenatal and early postnatal exposure to the Great Chinese Famine and the prevalence of poor physical function in midlife, however, was not observed from female adults.ConclusionsMales who were exposed to the Great Chinese Famine (1959–1961) present considerably decreased physical function in their later life.

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Physical health conditions and quality of life in adults with primary immunodeficiency diagnosed during childhood: A French Reference Center for PIDs (CEREDIH) study

Abstract: Adults with PIDs diagnosed during childhood experienced a heavy burden of health conditions, which affected their HRQoL. Our results emphasize the need to closely monitor this vulnerable population.

Cited by 27 publications

References 27 publications

The burden of common variable immunodeficiency disorders: a retrospective analysis of the European Society for Immunodeficiency (ESID) registry data

The burden of common variable immunodeficiency disorders: a retrospective analysis of the European Society for Immunodeficiency (ESID) registry data

The presence of overlapping quality of life symptoms in primary antibody deficiency (PAD) and chronic fatigue syndrome (CFS)

Association between early-life exposure to the Great Chinese Famine and poor physical function later in life: a cross-sectional study

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