Retrospective evaluation of adults with primary immunodeficiency disease.

Abstract

Primary immunodeficiency diseases (PIDs) are a group of heterogeneous disorders that result from one or more immune system abnormalities and have a wide range of clinical manifestations. To evaluate the demographic, clinical, and radiological features of adult patients with PID. We retrospectively reviewed the data of adult patients with PID who had been receiving immunoglobulin therapy at the adult allergy and immunology outpatient clinic between November 2017 and April 2022. Of the 23 patients included, 4 (17.4%) were females and 19 (82.6%) were males. The mean age was 38.7 ±16.2 (range: 18-75) years. Time of delay in the diagnosis of immunodeficiency was 14.9 ±15.7 (range: 0.5-49) years. The most common complaint on admission was sinopulmonary infection, and 65% of the patients had bronchiectasis. A total of 6 patients had a history of lymphoma, and 3 of them were diagnosed during the study period. Recurrence of lymphoma was observed in 1 patient. Patients with PID frequently have findings related to more than one organ system, and the diagnosis is often delayed in adults. Recognition and increased awareness of these manifestations is essential for early diagnosis and reducing morbidity and mortality.