Primary Antibody Deficiencies With Pulmonary Complications: A Single-Center Experience.

Güzin Özden,Pelin Pınar Deniz,Pelin Duru Cetinkaya

doi:10.7759/cureus.21140

Abstract

BackgroundPrimary immunodeficiencies are a heterogeneous group of genetic diseases caused by one or more abnormalities in the immune system. Although pulmonary complications are common in patients with primary immunodeficiency diseases, these complications contribute significantly to morbidity and mortality.AimThe aim of our study was to evaluate the distribution of the features of pulmonary radiological involvement and demographic findings in this patient group.Materials and methodsThe files of patients who were treated and followed up with the diagnosis of primary immunodeficiency between 2014 and 2021 were analyzed retrospectively. Demographic data, symptoms, additional diseases, and computed tomography findings of the patients were recorded.ResultsThe mean age of 32 cases was 37.34±13.54 (20-69) and the age of diagnosis was 28.90±15.75 (1-62). Twenty of the cases were male and 10 were female. The most common symptom was diarrhea with 53.1% and cough with 34.4%. The most common radiological finding is bronchiectasis in 75% of cases. Twenty-one (65.6%) of the cases had recurrent pneumonia before diagnosis and no pneumonia was observed after intravenous ımmunoglobulin replacement therapy. Three of the cases (9.4%) died during the follow-up.ConclusionsPrimary immunodeficiency should be considered in patients with bronchiectasis and a history of recurrent pneumonia, and further investigations should be performed. Early diagnosis of patients with primary immunodeficiency is very important for the early detection and treatment of malignancy and the interstitial lung disease that may develop.

Highlights

Primary immunodeficiency diseases (PIDs) are rare, and more than 330 heterogeneous diseases with 320 different genetic defects have been described [1]
Primary immunodeficiency should be considered in patients with bronchiectasis and a history of recurrent pneumonia, and further investigations should be performed
Diagnosis of patients with primary immunodeficiency is very important for the early detection and treatment of malignancy and the interstitial lung disease that may develop

Summary

Introduction

Primary immunodeficiency diseases (PIDs) are rare, and more than 330 heterogeneous diseases with 320 different genetic defects have been described [1]. Common variable immunodeficiency (CVID) is the most common primary antibody deficiencies (PADs) subgroup with severe and recurrent sinopulmonary infections, poor vaccination response, low total serum immunoglobulin G (IgG) and IgA, and/or IgM levels [2]. Primary immunodeficiencies are a heterogeneous group of genetic diseases caused by one or more abnormalities in the immune system. Pulmonary complications are common in patients with primary immunodeficiency diseases, these complications contribute significantly to morbidity and mortality

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