Biomaterial Database

Primary immunodeficiency diseases treated with immunoglobulin and associated comorbidities. 2021

Michelle Dilley, and Hannah Wangberg, and Joshua Noone, and Bob Geng

From the Division of Allergy, Immunology and Rheumatology, University of California San Diego and Rady Children's Hospital, San Diego, California.

Background: Primary immunodeficiency diseases (PIDD) consist of a heterogeneous group of disorders characterized by various aspects of immune dysregulation. Although the most universally recognized manifestation of PIDD is an increased susceptibility to infections, there is a growing body of evidence that patients with PIDD often have a higher incidence of lung disease, autoimmunity, autoinflammatory disorders, and malignancy. Objective: The purpose of this study was to better understand the noninfectious complications of PIDD by determining the comorbid disease prevalence across various age groups, genders, and immunoglobulin replacement types compared with the general population. Methods: A large U.S. insurance claims database was retrospectively analyzed for patients who had a diagnosis of PIDD and who had received intravenous immunoglobulin (IVIG) or subcutaneous immunoglobulin (SCIG). The prevalences of 31 different comorbid conditions in the Elixhauser comorbidity index were compared among the 3125 patients in the PIDD population to > 37 million controls separated by gender and by 10-year age cohorts. Results: In the PIDD population, statistically significantly higher comorbid diagnoses included chronic obstructive pulmonary disease-asthma in 51.5%, rheumatoid disease in 14%, deficiency anemia in 11.8%, hypothyroidism in 21.2%, lymphoma in 16.7%, neurologic disorders in 9.7%, arrhythmias in 19.9%, electrolyte disorders in 23.6%, coagulopathies in 16.9%, and weight loss in 8.4%. Conclusion: PIDD that require immunoglobulin replacement are associated with an increased risk of numerous comorbid conditions that affect morbidity and mortality. Recognition and increased awareness of these noninfectious complications can allow for better monitoring, care coordination, targeted treatments, and improved prognosis.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D007231	Infant, Newborn	An infant during the first 28 days after birth.	Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D007345	Insurance Claim Review	Review of claims by insurance companies to determine liability and amount of payment for various services. The review may also include determination of eligibility of the claimant or beneficiary or of the provider of the benefit; determination that the benefit is covered or not payable under another policy; or determination that the service was necessary and of reasonable cost and quality.	Claims Review,Insurance Audit,Insurance Claims Processing,Analysis, Claims,Claim Review, Insurance,Claims Analysis,Insurance Claims Analysis,Review, Claims,Review, Insurance Claim,Analyses, Claims,Analyses, Insurance Claims,Analysis, Insurance Claims,Audit, Insurance,Audits, Insurance,Claim Reviews, Insurance,Claims Analyses,Claims Analyses, Insurance,Claims Analysis, Insurance,Claims Processing, Insurance,Claims Reviews,Insurance Audits,Insurance Claim Reviews,Insurance Claims Analyses,Processing, Insurance Claims,Reviews, Claims,Reviews, Insurance Claim
D008297	Male		Males
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000081207	Primary Immunodeficiency Diseases	Genetic immunologic deficiency diseases and syndromes due to mutations in genes involved in IMMUNITY generally characterized by an increased susceptibility to infectious diseases. They are often associated with AUTOIMMUNE DISEASE manifestations.	Congenital Immunodeficiency Disease,Congenital Immunodeficiency Diseases,Congenital Immunodeficiency Disorder,Congenital Immunodeficiency Disorders,Congenital Immunodeficiency Syndrome,Congenital Immunodeficiency Syndromes,Inherited Immunodeficiency Disease,Inherited Immunodeficiency Diseases,Inherited Immunodeficiency Disorder,Inherited Immunodeficiency Disorders,Inherited Immunodeficiency Syndrome,Inherited Immunodeficiency Syndromes,Primary Antibody Deficiencies,Primary Antibody Deficiency Disorder,Primary Antibody Deficiency Disorders,Primary Antibody Deficiency Syndrome,Primary Antibody Deficiency Syndromes,Primary Immune Deficiency,Primary Immune Deficiency Disease,Primary Immune Deficiency Diseases,Primary Immune Deficiency Disorder,Primary Immune Deficiency Disorders,Primary Immune Deficiency Syndrome,Primary Immune Deficiency Syndromes,Primary Immunodeficiency Disease,Primary Immunodeficiency Disorder,Primary Immunodeficiency Disorders,Primary Immunodeficiency Syndromes,Antibody Deficiency, Primary,Deficiency, Primary Immune,Immune Deficiency, Primary,Immunodeficiency Disease, Congenital,Immunodeficiency Disease, Inherited,Immunodeficiency Disease, Primary,Immunodeficiency Diseases, Congenital,Immunodeficiency Diseases, Inherited,Immunodeficiency Diseases, Primary,Immunodeficiency Disorder, Congenital,Immunodeficiency Disorder, Inherited,Immunodeficiency Disorder, Primary,Immunodeficiency Disorders, Congenital,Immunodeficiency Disorders, Inherited,Immunodeficiency Syndrome, Congenital,Immunodeficiency Syndrome, Inherited,Immunodeficiency Syndrome, Primary,Immunodeficiency Syndromes, Congenital,Immunodeficiency Syndromes, Inherited,Immunodeficiency Syndromes, Primary,Inherited Immunodeficiency Syndrome,Primary Antibody Deficiency,Primary Immune Deficiencies,Primary Immunodeficiency Syndrome