Abstract

Primary immunodeficiency diseases (PIDs) include over 250 diverse disorders. The current study assessed management of PID by family practice physicians. The American Academy of Allergy, Asthma, and Immunology Primary Immunodeficiency Committee and the Immune Deficiency Foundation conducted an incentivized mail survey of family practice physician members of the American Medical Association and the American Osteopathic Association in direct patient care. Responses were compared with subspecialist immunologist responses from a similar survey. Surveys were returned by 528 (of 4500 surveys mailed) family practice physicians, of whom 44% reported following ≥1 patient with PID. Selective immunoglobulin A deficiency (21%) and chronic granulomatous disease (11%) were most common and were followed by significantly more subspecialist immunologists (P < 0.05). Use of intravenously administered immunoglobulin and live viral vaccinations across PID was significantly different (P < 0.05). Few family practice physicians were aware of professional guidelines for diagnosis and management of PID (4 vs. 79% of subspecialist immunologists, P < 0.05). Family practice physicians will likely encounter patients with PID diagnoses during their career. Differences in how family practice physicians and subspecialist immunologists manage patients with PID underscore areas where improved educational and training initiatives may benefit patient care.

Highlights

  • Primary immunodeficiency diseases (PIDs) comprise more than 250 heterogeneous disorders [1]

  • A recent analysis of registry data and epidemiologic surveys estimated that as many as six million people worldwide are living with PID, with the majority undiagnosed (27,000 patients identified from national registries; 60,000 patients identified from the Jeffrey Modell Centers Network) [3]

  • Of the 4500 surveys mailed, 528 completed surveys were returned [14 respondents were excluded from the analysis (5 saw “0” patients on an outpatient basis; 9 failed to answer the question asking how many patients they saw on an outpatient basis)]

Read more

Summary

Introduction

Primary immunodeficiency diseases (PIDs) comprise more than 250 heterogeneous disorders [1]. These diseases are caused by intrinsic defects in the immune system that lead to aberrant immune responses [2]. The field of PID has evolved, and the number of recognized disorders has rapidly increased [3,4,5], creating challenges for maintaining up-to-date disease classifications and for disseminating practical patient management guidelines. A recent analysis of registry data and epidemiologic surveys estimated that as many as six million people worldwide are living with PID, with the majority undiagnosed (27,000 patients identified from national registries; 60,000 patients identified from the Jeffrey Modell Centers Network) [3]. A prior household random-digit dialing telephone survey indicated that as many as 150,000–360,000 persons in the United States have been diagnosed with a PID [6].

Objectives
Methods
Results
Conclusion
Full Text
Published version (Free)

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call