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Education, Teaching, and Quality Improvement |

The Pulmonologist Approach to Diagnosis and Management of Patients With Primary Immunodeficiency Diseases

Jordan Orange; Javeed Akhter; Filiz Seeborg; Marcia Boyle, BA; Chris Scalchunes; Vivian Hernandez-Trujillo
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Texas Children’s Hospital, Houston, TX


Chest. 2014;146(4_MeetingAbstracts):486A. doi:10.1378/chest.1994436
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Abstract

SESSION TITLE: Education and Teaching in Critical Care Posters

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Wednesday, October 29, 2014 at 01:30 PM - 02:30 PM

PURPOSE: As pulmonary symptoms are common in patients with primary immunodeficiency diseases (PID), pulmonologists are often the first referred specialists. In a recent Immune Deficiency Foundation (IDF) survey, patients reported ≥1 case of pneumonia (40%) and permanent lung damage before diagnosis (24%). We evaluated the practice of pulmonologists in regards to PID diagnosis and treatment.

METHODS: The American Academy of Allergy, Asthma, and Immunology (AAAAI) Primary Immunodeficiency Committee and the IDF conducted an anonymous, incentivized mail survey of American Medical Association and American Osteopathic Association members specializing in pulmonology. Responses were compared to a historical survey of 71 sub-specialist immunologists (AAAAI members devoting >10% of their practice to PID management).

RESULTS: Surveys were returned by 485 pulmonologists actively practicing in the United States; 39% reported a specialty of adult pulmonology and 52% reported they expect <3 new patients per year with PID. Forty-nine percent have diagnosed a patient with a PID. The most commonly followed diagnoses were common variable immunodeficiency (57%) and immunoglobulin G (IgG) subclass deficiency (51%), which were significantly more prevalent in sub-specialist immunologists practices (99% and 89%, respectively; p<.001). Pulmonologists most commonly ordered quantitative serum Igs (96%) and IgG subclasses (83%) for PID diagnosis; 48% requested vaccine antibody titers (no comparative sub-specialist immunologist data). Significant differences between pulmonologists and sub-specialist immunologists were evident in the utilization of intravenously-administered IgG therapy and prophylactic antibiotics across PIDdiagnoses and in live viral vaccine avoidance for patients with several diagnoses (p<.001). Significantly fewer pulmonologists (11% vs 79%, p<.001) were aware of published professional guidelines regarding management of PI.

CONCLUSIONS: Pulmonologists can have an important role in management of patients with PID, especially given the incidence of pneumonia in these patients. The current study reveals several significant differences in how pulmonologists diagnose and treat patients with PID compared with sub-specialist immunologists.

CLINICAL IMPLICATIONS: Increased educational and training initiatives aimed at pulmonologists may contribute to improved PID diagnosis and patient management and potentially minimize long-term pulmonary damage.

DISCLOSURE: Jordan Orange: Grant monies (from industry related sources): CSL Behring, Grant monies (from industry related sources): Baxter, Consultant fee, speaker bureau, advisory committee, etc.: ASD, Consultant fee, speaker bureau, advisory committee, etc.: Griffols, Consultant fee, speaker bureau, advisory committee, etc.: Octapharma, Consultant fee, speaker bureau, advisory committee, etc.: IDT/Viracor, Consultant fee, speaker bureau, advisory committee, etc.: BPL, Consultant fee, speaker bureau, advisory committee, etc.: Atlantic Research, Consultant fee, speaker bureau, advisory committee, etc.: Up to date, Consultant fee, speaker bureau, advisory committee, etc.: Immune Deficiency Foundation Marcia Boyle: Employee: Immune Deficiency Foundation Chris Scalchunes: Employee: Immune Deficiency Foundation Vivian Hernandez-Trujillo: Consultant fee, speaker bureau, advisory committee, etc.: CSL Behring, Consultant fee, speaker bureau, advisory committee, etc.: IFIR , Consultant fee, speaker bureau, advisory committee, etc.: Baxter, Consultant fee, speaker bureau, advisory committee, etc.: Sanofi, Consultant fee, speaker bureau, advisory committee, etc.: Merck, Consultant fee, speaker bureau, advisory committee, etc.: Immune Deficiency Foundation The following authors have nothing to disclose: Javeed Akhter, Filiz Seeborg

No Product/Research Disclosure Information


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