Prevalence and Morbidity of Primary Immunodeficiency Diseases, United States 2001–2007

Kobrynski, Lisa; Powell, Rachel; Bowen, Scott

doi:10.1007/s10875-014-0102-8

Cited by 110 publications

(94 citation statements)

References 40 publications

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“…This is supported by data indicating that patients with CVID are diagnosed a mean age of 20–43 years [6, 33, 34] and have several impairments, with chronic lung disease being the most common [4]. Data from the current analysis also indicated that, overall, adult patients with CVID had significantly diminished mental and physical well-being compared with the general US population (Fig.…”

Section: Discussionsupporting

confidence: 79%

Health-Related Quality of Life in Adult Patients with Common Variable Immunodeficiency Disorders and Impact of Treatment

et al. 2017

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PurposeCommon variable immunodeficiency disorder (CVID) is a primary immunodeficiency disease (PIDD) often associated with severe and chronic infections. Patients commonly receive immunoglobulin (Ig) treatment to reduce the cycle of recurrent infection and improve physical functioning. However, how Ig treatment in CVID affects quality of life (QOL) has not been thoroughly evaluated. The purpose of a recent Immune Deficiency Foundation (IDF) mail survey was to assess the factors that are associated with QOL in patients with CVID receiving Ig treatment.MethodsA 75-question survey developed by the IDF and a 12-item Short Form Health Survey (SF-12) to assess QOL were mailed to adults with CVID. Mean SF-12 scores were compared between patients with CVID and the general US adult population normative sample.ResultsOverall, 945 patients with CVID completed the surveys. More than half of the patients (54.9%) received intravenous Ig and 44.9% received subcutaneous Ig treatment. Patients with CVID had significantly lower SF-12 scores compared with the general US population regardless of sex or age (p < 0.05). Route of IgG replacement did not dramatically improve QOL. SF-12 scores were highest in patients with CVID who have well-controlled PIDD, lacked physical impairments, were not bothered by treatment, and received Ig infusions at home.ConclusionThese data provide insight into what factors are most associated with physical and mental health, which can serve to improve QOL in patients in this population. Improvements in QOL can result from early detection of disease, limiting digestive system disease, attention to fatigue, and implementation of an individual treatment plan for the patient.Electronic supplementary materialThe online version of this article (doi:10.1007/s10875-017-0404-8) contains supplementary material, which is available to authorized users.

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Section: Discussionsupporting

confidence: 79%

Health-Related Quality of Life in Adult Patients with Common Variable Immunodeficiency Disorders and Impact of Treatment

et al. 2017

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“…Although primary immune deficiency disorders (PIDDs) are considered ‘rare diseases’, both the incidence and the prevalence of those disorders are increasing . With this increase, it is reasonable to anticipate that the demand for immunoglobulin replacement therapy (IgGRT) would increase.…”

Section: Introductionmentioning

confidence: 99%

Latent therapeutic demand model for the immunoglobulin replacement therapy of primary immune deficiency disorders in the USA

Stonebraker

Hajjar

2018

Vox Sanguinis

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“…In 2013, there were an estimated 6 million people living with PID worldwide [3], with PID affecting 29.1–50.5 per 100,000 [4]. It is likely that prevalence of PID will continue to increase as the extended recognition of these diagnoses grows, significantly affecting both adults and children at the population level [3].…”

Section: Introductionmentioning

confidence: 99%

Increased Incidence of Fatigue in Patients with Primary Immunodeficiency Disorders: Prevalence and Associations Within the US Immunodeficiency Network Registry

2017

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Introduction Patients with primary immunodeficiency (PID) often report fatigue, yet this symptom has not been studied in PID. Fatigue affects 6–7.5% of healthy adults. The goal of this study is to estimate the prevalence of fatigue in patients with PID and investigate its associated factors. Methods We analyzed 2537 PID patients registered in USIDNET to determine responses to the field “fatigue” in the core registry form. Demographics, immune phenotypes, and comorbid conditions were compared between fatigued and non-fatigued patients to identify relevant associations and potential drivers. A focused analysis was performed for patients with predominantly antibody deficiency disorders (PADs). Results Fatigue was reported in 25.9%(95% CI 23.7–28.3) of PAD patients, compared to 6.4% (95% CI 4.9–8.2) of non-PAD. Patients with common variable immunodeficiency (CVID) had the highest prevalence of fatigue (p < 0.001) among all PID diagnoses. Other factors that were associated with a higher rate of fatigue among PAD patients included female sex, higher BMI, depression, bronchiectasis, and autoimmunity. Additionally, fatigued PAD patients had lower absolute lymphocyte, CD3, CD4, and CD8 counts compared to non-fatigued patients. Conclusion Our findings suggest that fatigue is overrepresented in PAD patients. Prospective studies to estimate prevalence, risk factors, and fatigue etiology in PID are warranted, so therapeutic interventions can be considered.

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Prevalence and Morbidity of Primary Immunodeficiency Diseases, United States 2001–2007

Cited by 110 publications

References 40 publications

Health-Related Quality of Life in Adult Patients with Common Variable Immunodeficiency Disorders and Impact of Treatment

Health-Related Quality of Life in Adult Patients with Common Variable Immunodeficiency Disorders and Impact of Treatment

Latent therapeutic demand model for the immunoglobulin replacement therapy of primary immune deficiency disorders in the USA

Increased Incidence of Fatigue in Patients with Primary Immunodeficiency Disorders: Prevalence and Associations Within the US Immunodeficiency Network Registry

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