30-Year Review of Pediatric- and Adult-Onset CVID: Clinical Correlates and Prognostic Indicators

• Published in: Journal of clinical immunology Vol. 39; no. 7; pp. 678 - 687
• Main Authors: Baloh, Carolyn, Reddy, Anupama, Henson, Michele, Prince, Katherine, Buckley, Rebecca, Lugar, Patricia
• Format: Journal Article
• Language: English
• Published: New York Springer US 01.10.2019; Springer Nature B.V
• Subjects: Adolescent; Adult; Age; Age of Onset; Biomedical and Life Sciences; Biomedicine; Child; Common variable immunodeficiency; Common Variable Immunodeficiency - diagnosis; Common Variable Immunodeficiency - epidemiology; Common Variable Immunodeficiency - etiology; Common Variable Immunodeficiency - mortality; Comorbidity; Delayed Diagnosis; Diagnosis; Disease Susceptibility; Female; Gastroesophageal reflux; Humans; Immunology; Infectious Diseases; Internal Medicine; Kaplan-Meier Estimate; Laboratories; Lung diseases; Male; Medical Microbiology; Mental depression; Mortality; Odds Ratio; Original Article; Patients; Pediatrics; Phenotype; Prognosis; Risk factors; Young Adult; mortality; immunodeficiency; depression; granulomatous lymphocytic interstitial lung disease (GLILD); Common variable immunodeficiency disorders (CVID); pediatric
• ISSN: 0271-9142; 1573-2592; 1573-2592
• DOI: 10.1007/s10875-019-00674-9

Purpose To evaluate mortality risk factors in pediatric-onset common variable immunodeficiency disorders (CVID), we evaluated the largest single-institution cohort of pediatric-onset CVID patients. Previous publications on CVID have provided valuable descriptive data, but lack risk stratification to guide physicians in management of these patients. Methods Retrospective chart review of 198 subjects with CVID at a single institution, of whom 91 had disease onset at a pediatric age. Clinical and laboratory data were collected at diagnosis and in follow-up. Odds ratios and Fisher tests were utilized to examine trends. This study was approved by an institutional review board. Results Clinical features and laboratory results for subjects diagnosed with CVID at a pediatric age are similar to those who had adult-onset CVID. However, majority of the deceased subjects (13/18) were at a pediatric age at CVID symptom onset. These subjects had a lower age at mortality, multiple comorbidities, and often depression. The most common cause of death was infection. Lung disease (OR 5, p  < 0.05) and infection with severe/opportunistic organisms (OR 9, p  < 0.05) are directly related to increased mortality. Delay in diagnosis of CVID is also correlated with mortality. Intermediary markers correlating with mortality include anemia, GERD, and depression. Conclusions There are many similarities between patients with pediatric- and adult-onset CVID; however, the mortality of pediatric CVID in our cohort is striking. This is the first study to identify specific factors correlated with mortality in pediatric-onset CVID to guide pediatricians and subspecialists in managing these immunodeficient patients.