Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Abstract

Primary immunodeficiency disorders (PIDs) are caused by 1 or more defects of the immune system. Patients are more likely to experience recurrent and/or severe infections and tend to develop a wide range of complications. Respiratory diseases are the main and initial manifestation in most cases and the most common complication. Pulmonary complications cause significant morbidity and mortality in patients with PIDs. Early diagnosis and appropriate treatment can prevent or at least slow the development of respiratory complications. Since the spectrum of pulmonary complications in PIDs is broad, we divided pulmonary complications into upper respiratory complications (eg, sinusitis, otitis media, and laryngeal angioedema) and lower respiratory complications (eg, pneumonia, bronchitis, bronchiectasis, interstitial lung diseases, organizing pneumonia, pulmonary adenopathies and malignancies, hyperreactive airway diseases, pulmonary dysgenesis, and adverse reactions to treatment). This review covers the main respiratory manifestations in patients with PIDs. Key words: Primary immunodeficiency disorders. Pulmonary complications. Upper respiratory tract. Lower respiratory tract. ResumenLas inmunodeficiencias primarias (PIDs) son enfermedades causadas por uno o más defectos del sistema inmunológico. Estos pacientes presentan con frecuencia infecciones recidivantes y/o severas así como otro tipo de complicaciones. Las patologías respiratorias son la principal y más frecuente manifestación y complicación de las PIDs. Las complicaciones de estas patologías pulmonares constituyen una de las principales causas de morbimortalidad entre los pacientes que sufren PIDs. El diagnóstico temprano y el tratamiento adecuado pueden prevenir, o al menos retrasar, la aparición de las complicaciones respiratorias en estos pacientes. Dado que el espectro de las enfermedades pulmonares es muy amplio, hemos dividido estas complicaciones entre aquellas que afectan a las vías aéreas superiores (sinusitis, otitis media y angioedema laríngeo, etc.) y las que afectan a las vías aéreas bajas (neumonía, bronquitis, bronquiectasias, enfermedades pulmonares intersticiales, neumonía organizada, adenopatías pulmonares y neoplasias, hiperreactividad bronquial, disgenesia pulmonar y las debidas a los efectos secundarios del tratamiento instaurado). Este artículo revisa las manifestaciones respiratorias que se observan más frecuentemente en los pacientes con PIDs. Palabras clave: Inmunodeficiencias primarias. Complicaciones pulmonares. Vías respiratorias altas. Vías respiratorias bajas.

Respiratory and allergic disorders in children with severe and partial immunoglobulin A immunodeficiency

“…Indeed, our data analysis confirmed that children with IgAD had higher number of respiratory infections compared to controls, with the highest number of those suffering from bronchitis and pneumonia. Also, there was a higher prevalence of allergic diseases, specifically asthma, allergic rhinitis and atopic dermatitis in children with low IgA, which is in accordance to previous studies . Because of the fact that diagnosis of sIgAD depends on serum IgA concentration levels with monomeric IgA1 being dominant, whereas secretory IgA with dominant IgA2 form is not determined, we are not sure if both subclasses are being affected and why some immunocompromised children can be asymptomatic, despite the important protective role of IgA2.…”

“…Recurrent infections of the respiratory tract are the most common clinical manifestation in immunodeficiencies, especially in sIgAD . Since children with PIDs have recurrent infections that may have a significant role in morbidity and death (30%‐65%), early detection and management of pulmonary disorders related with PID is essential for optimal therapy and management of the disease . Earlier studies have demonstrated that early diagnosis could reduce the number of infections in patients with antibody deficiencies and is the reason why we wanted to compare control group of children with patients with sIgAD and to investigate the connection between pulmonary function and infections with clinical features of PID.…”

“…Primary immunodeficiency disorders (PIDs) are identified as a heterogeneous group of inherited disorders and are characterized by impairment of one or more components of the immune system. It is estimated that PIDs affect between 1:10000 and 1:600 individuals in over 40 countries, but the real number of incidence and prevalence is still unknown . Nowadays, 330 disorders with 320 different gene defects have been identified, which include different disorders and immune dysregulation with defects in innate and intrinsic immunity …”

“…It is estimated that PIDs affect between 1:10000 and 1:600 individuals in over 40 countries, but the real number of incidence and prevalence is still unknown. 1,2 Nowadays, 330 disorders with 320 different gene defects have been identified, which include different disorders and immune dysregulation with defects in innate and intrinsic immunity. 1,3 The most frequent type of PIDs is primary antibody deficiency.…”

Two Faces of LRBA Deficiency in Siblings: Hypogammaglobulinemia and Normal Immunoglobulin Levels

“…Similar to common variable immune deficiency, immune dysregulation as a consequence of LRBA deficiency leads to recurrent infections, autoimmunity, and enteropathy [1][2][3][4][5][6][7]. Affected individuals mainly show reduced levels of at least 2 Ig isotypes.…”