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Pulmonary Disease Burden in Primary Immune Deficiency Disorders: Data from USIDNET Registry

  • Meera Patrawala
  • Ying Cui
  • Limin Peng
  • Ramsay L. Fuleihan
  • Elizabeth K. Garabedian
  • Kiran Patel
  • Lokesh GuglaniEmail author
Original Article
  • 51 Downloads

Abstract

Purpose

Pulmonary manifestations are common in patients with primary immunodeficiency disorders (PIDs) but the prevalence, specific diseases, and their patterns are not well characterized.

Methods

We conducted a retrospective analysis of pulmonary diseases reported in the database of the United States Immunodeficiency Network (USIDNET), a program of the Immune Deficiency Foundation. PIDs were categorized into 10 groups and their demographics, pulmonary diagnoses and procedures, infections, prophylaxis regimens, and laboratory findings were analyzed.

Results

A total of 1937 patients with various PIDs (39.3% of total patients, 49.6% male, average age 37.9 years (SD = 22.4 years)) were noted to have a pulmonary disease comorbidity. Pulmonary diseases were categorized into broad categories: airway (86.8%), parenchymal (18.5%), pleural (4.6%), vascular (4.3%), and other (13.9%) disorders. Common variable immune deficiency (CVID) accounted for almost half of PIDs associated with airway, parenchymal, and other pulmonary disorders. Pulmonary procedures performed in 392 patients were mostly diagnostic (77.3%) or therapeutic (16.3%). These patients were receiving a wide variety of treatments, which included immunoglobulin replacement (82.1%), immunosuppressive (32.2%), anti-inflammatory (12.7%), biologic (9.3%), and cytokine (7.6%)-based therapies. Prophylactic therapy was being given with antibiotics (18.1%), antifungal (3.3%), and antiviral (2.2%) medications, and 7.1% of patients were on long-term oxygen therapy due to advanced lung disease.

Conclusions

Pulmonary manifestations are common in individuals with PID, but long-term pulmonary outcomes are not well known in this group of patients. Further longitudinal follow-up will help to define long-term prognosis of respiratory comorbidities and optimal treatment modalities.

Keywords

Lung disease immune deficiency airway infection bronchiectasis pulmonary immunity 

Notes

Acknowledgments

The authors would like to thank the U.S. Immunodeficiency Network (USIDNET), a program of the Immune Deficiency Foundation (IDF) that is supported by a cooperative agreement, U24AI86837, from the National Institute of Allergy and Infectious Diseases (NIAID).

Authorship Contributions

LG and KP conceived this study. KP reached out to USIDNET for collecting anonymized subject data from the USIDNET patient registry. LG and KP also analyzed the data, wrote and edited the manuscript. MP, YC, and LP were involved in data collection and analysis and were also involved in writing the manuscript. RLF and EKG contributed to the data included in the USIDNET database.

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflicts of interest.

Supplementary material

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2020

Authors and Affiliations

  1. 1.Department of Pediatrics, Division of Pulmonology, Allergy/Immunology, Cystic Fibrosis and SleepEmory UniversityAtlantaUSA
  2. 2.Department of BiostatisticsEmory UniversityAtlantaUSA
  3. 3.Division of Allergy and Immunology, Ann & Robert H. Lurie Children’s Hospital of ChicagoNorthwestern University Feinberg School of MedicineChicagoUSA
  4. 4.National Human Genome Research Institute, National Institutes of HealthBethesdaUSA

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