Lingual Tonsillectomy and Adenoidectomy: A Treatment Option In Adult Onset PFAPA Syndrome

Abstract

There has been an association between post solid organ transplant (SOT) hypogammaglobulinemia and infections, but the benefit of immunoglobulin (Ig) replacement has been less clear. We hypothesize that (1) only a subset of patients with post-SOT hypogammaglobulinemia have impaired antibody responses; (2) empiric Ig replacement before checking antibody responses may commit some patients to unnecessary therapy; and (3) patients with impaired antibody responses have fewer infections after replacement. METHODS: Epic Slicer Dicer was used to retrospectively identify patients with lung or kidney transplants evaluated by the Immunodeficiency Clinic at Johns Hopkins (2010-2019) for hypogammaglobulinemia (n5 59). Pneumococcal conjugate vaccine challenge titers to assess antibody responses were obtained. If titers revealed protection to fewer than 6/14 serotypes (cutoff 1.0 ug/ml) then patients were started on replacement. Data on severity, type, frequency of infections was collected. RESULTS: 27/36 SOT patients with hypogammaglobulinemia (median Ig level: 445 mg/dl, range: 199-634 mg/dl) had impaired antibody responses, and started replacement. They had a mean of 2.18 infections/year and median of 1 infection/year before replacement. After replacement they had a mean of 1.08 infections/year and median of 1 infection/year. 9/36 SOT patients with hypogammaglobulinemia (median Ig level: 458 mg/dl, range 354-511 mg/dl) had normal antibody responses and did not start replacement. They had a mean of 1.22 infections/year and median of 1 infection/year. CONCLUSIONS: Only a subset of patients with post-SOT hypogammaglobulinemia have evidence of impaired humoral response necessitating Ig replacement. This parameter can help identify patients who may benefit most from Ig replacement to reduce infectious complications.