Family Physician Perspectives on Primary Immunodeficiency Diseases

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.

Keywords: diagnosis; family practice physicians; primary immunodeficiency disease; survey; treatment.

Figure 1

Percentage of family practice physicians, compared with subspecialist immunologists, who have ever followed patients with a PID. *P < 0.0001. AT, ataxia telangiectasia; CGD, chronic granulomatous disease; CMC, chronic mucocutaneous candidiasis; CVID, common variable immunodeficiency; IgA, immunoglobulin A; IgG, immunoglobulin G; IgM, immunoglobulin M; PID, primary immunodeficiency disease; SAD, selective antibody deficiency; SCID, severe combined immunodeficiency; WAS, Wiskott–Aldrich syndrome; XLA, X-linked agammaglobulinemia (Bruton’s agammaglobulinemia).

Figure 2

Percentage of family practice physicians ordering specific tests for the diagnosis of PID. Anti-Pneumo titer, anti-Pneumococcal titer; CBC, complete blood count; CH50, hemolytic complement; CT, computed tomography; DTH, delayed-type hypersensitivity; IgG, immunoglobulin G; PID, primary immunodeficiency disease; QIgs, quantitative serum; SPEP, serum immunoelectrophoresis.

Figure 3

Percentage of family practice physicians, compared with subspecialist immunologists, recommending IVIG therapy for all or most patients with a diagnosis of a specific PID (in at least some patients within the listed diagnosis). *P < 0.0001; ^†P = 0.006. CGD, chronic granulomatous disease; CVID, common variable immunodeficiency; IgA, immunoglobulin A; IgG Sub, immunoglobulin G subclass; IgM, immunoglobulin M; IVIG, intravenously administered immunoglobulin; PID, primary immunodeficiency disease; SCID, severe combined immunodeficiency; XLA, X-linked agammaglobulinemia (Bruton’s agammaglobulinemia).

Figure 4

Percentage of family practice physicians, compared with subspecialist immunologists, avoiding live vaccines for patients with specific PID. *P < 0.0001. Ab, antibody; CGD, chronic granulomatous disease; CVID, common variable immunodeficiency; IgA, immunoglobulin A; IgG, immunoglobulin G; IgM, immunoglobulin M; PID, primary immunodeficiency disease; SAD, selective antibody deficiency; SCID, severe combined immunodeficiency; WAS, Wiskott– Aldrich syndrome; XLA, X-linked agammaglobulinemia (Bruton’s agammaglobulinemia).