Sinopulmonary Complications in Subjects With Primary Immunodeficiency

Abstract

BACKGROUND: The aim of this work was to describe the frequency and spectrum of sinopulmonary complications among subjects with primary immunodeficiency disorders. METHODS: The subjects included all patients with primary immunodeficiency who were registered prospectively between January 2004 and December 2013 in the Kuwait National Primary Immunodeficiency Disorders Registry. RESULTS: A total of 202 subjects were registered during the study period. Subjects with combined immunodeficiencies were the most prevalent (65 subjects, 32.1%), followed by well-defined syndromes with immunodeficiency (45 subjects, 22.2%) and predominantly antibody deficiencies (35 subjects, 17.3%). A total of 295 sinopulmonary manifestations were observed in 127 subjects (63%); 157 manifestations (53.2%) were observed among the presenting symptoms, and 138 manifestations (46.8%) occurred after establishment of the primary immunodeficiency disorder diagnosis. Sinopulmonary manifestations were more common in subjects with predominantly antibody deficiencies (2.3 manifestations/subject), followed by subjects with combined immunodeficiencies (1.75 manifestations/subject). Pneumonia was the most common manifestation (108 episodes affecting 80 subjects), followed by otitis media (81 episodes affecting 59 subjects), bronchiectasis in 28 subjects (13.8%), and asthma in 22 subjects (11%). Microbial organisms were isolated during 46 episodes of pneumonia (42.5%) (cytomegalovirus and Pneumocystis jirovecii were the most common). There were 57 deaths (28%) during the study period. Twenty-four deaths (42%) were due to pulmonary complications as follows: pneumonia (16 subjects, 8%), pulmonary hemorrhage (6 subjects, 3%), and aspiration pneumonia (2 subjects, 1%). CONCLUSIONS: Sinopulmonary complications are common in subjects with primary immunodeficiency. They can be serious and continue to occur even after proper treatment is initiated. The pulmonologist should play an important role in the management of subjects with primary immunodeficiency disorder.

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

“…Other studies have also reported a high rate of otitis media in XLA patients [31][32][33][34]. In one study, we demonstrated that 81.1% of XLA patients manifested acute otitis media.…”

“…Yarmohammadi et al [15] reported a high rate of otitis media in patients with PID (81 of 113 patients, 71.6%), which was significantly higher than in patients without immune defects. Owayed and Al-Herz [31] found the rate of otitis media to be lower (59 of 202 patients, 29.2%). In the third report of the Iranian registry, we reported 46 cases in 731 patients (6.3%) in whom the first presentation of PID was otitis media [3].…”

Update on invasive fungal infections in the Middle Eastern and North African region

“…In Turkey, the prevalence of PJP among AIDS/HIV patients ranged from 10 to 46.7% [104][105][106], while in Iran, PJP ranged from 4.5 to 39.3% [107][108][109]. Regarding the Arabian Peninsula region, PJP occurring in 9 episodes among primary immunodeficiency disorder patients in Kuwait [110] was found in 5.1% of PJP Pneumocystis jirovecii pneumonia, HIV human immunodeficiency virus, AIDS acquired immunodeficiency syndrome, PCR polymerase chain reaction, BAL bronchoalveolar lavage, CMV cytomegalovirus, CT computerized tomography, y years total HIV-infected patients in Bahrain [111], while accounted for 25% of opportunistic infections in HIV/AIDS patients in Oman with no mortality reported due to this infection [97]. Concerning North Africa, a retrospective analysis in 2013 conducted on HIV-related hospitalizations showed that PJP was responsible for 8.8% respiratory diseases in Libya [112] and accounted for 11.1% death in HIV-infected patients in Tunisia [100].…”

Monogenic diseases in respiratory medicine: Clinical perspectives

“…Pediatric pulmonologists should consider PID as an important component for differential diagnosis in order to enable early recognition and improve outcomes. Pulmonary manifestations of PID can be divided into two main groups―infections and noninfectious complications―which differ from recurrent infections affecting various locations and opportunistic or unusual pathogenic infections to malignancy and lymphoproliferative disorders . Findings of PID on imaging range from consolidation, abscess, and empyema to bronchiectasis and interstitial lung disease .…”

“…Patients with pulmonary arteriovenous malformation can present with hemoptysis and cyanosis. 17 20 Findings of PID on imaging range from consolidation, abscess, and empyema to bronchiectasis and interstitial lung disease. 21 The organisms in pulmonary infection differ from defects in immunity-encapsulated organisms are typical pathogens in patients with deficient humoral immunity, and infections by fungal organisms are more typical of cellular immune deficiencies.…”