Primary immunodeficiency diseases treated with immunoglobulin and associated comorbidities

Abstract

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.

Bronchiectasis and obstructive lung diseases in primary antibody deficiencies and beyond: update on management and pathomechanisms

“…Data on the estimated prevalence of asthma and COPD in patients with primary immunodeficiency vary significantly. In a retrospective US insurance claims database analysis from 2021, Dilley et al [49] report a prevalence as high as 51.5% for asthma and/ or COPD. However, the relevance of data collection is exemplified by a recent single-center study by de Wit et al [50] In this cohort of 253 mostly adult primary antibody deficient patients, the prevalence of obstructive lung disease was 56% when using patient questionnaires as the data source, and dropped to 16% when only including patients with diagnosis confirmed by pulmonary function testing.…”

A Single Center Study Investigating Clinical Outcomes of Testing for Multiple Myeloma and Immune Deficiency at Low Globulin Levels

“…The clinical presentation of PID, and one of the most common symptomatic primary immune deficiency disorders, common variable immune deficiency (CVID) is broad and may present as predominantly recurrent infections or may present with autoimmune, inflammatory conditions or malignancy. 3 , 4 Secondary immune deficiency is more common than PID, and may present within a wider range of specialties and is associated with an increased risk of infection. 5 Calculated globulin may be low in hypogammaglobulinemia.…”

Efficacy And Safety of Blood Derivatives Therapy in Alzheimer’s Disease: A Systematic Review And Meta-Analysis

“…IVIg, containing the full range of antibody spectrum, is derived from the plasma of thousands of healthy donors. It has been used to treat patients with autoimmune diseases for decades [15][16][17][18]. Moreover, it has been demonstrated that in addition to anti-amyloid-β (Aβ), IVIG also has anti-in ammatory and immunomodulatory functions [19][20][21].…”