Thoracic Manifestations of Primary Immunodeficiency Disorders
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CLINICAL BRIEF
Thoracic Manifestations of Primary Immunodeficiency Disorders Poonam Sherwani 1 & Ashu Seith Bhalla 1 & Manisha Jana 1 & Priyanka Naranje 1 & Sushil K. Kabra 2 & Arun Kumar Gupta 1 & Devasenathipathy Kandasamy 1 Received: 23 December 2019 / Accepted: 1 April 2020 # Dr. K C Chaudhuri Foundation 2020
Abstract Primary immunodeficiency disorders (PIDD) are a group of disorders presenting with recurrent infections. The authors retrospectively reviewed the imaging records of 24 proven cases of PIDD and correlated the imaging findings with the type of defect. Final diagnoses were categorized in four groups; Group I (humoral immunodeficiency), Group 2 (cell mediated immunodeficiency), Group 3 (phagocytic disorders) and Group 4 (others). Group 3 was the commonest, followed by 1 and 2. Three most common disorders encountered were chronic granulomatous disease (CGD) (7/24), hyper IgE syndrome (5/24) and common variable immunodeficiency (CVID) (4/24). Consolidation (12/24), nodules (10/24), bronchiectasis (9/24) and lymphadenopathy (8/24) were the commonest imaging findings. Although not statistically significant, some definite imaging trends could be established. Presence of consolidation and nodules; with absence of bronchiectasis was the striking finding in Group 3. Group 2 disorders predominantly showed bronchiectasis, whereas presence of consolidation, nodules, bronchiectasis all were very common in Group 1. Keywords PIDD . Primary immunodeficiency . Imaging . HRCT
Introduction Primary immunodeficiency disorders (PIDDs) are inherited diseases which develop due to defect in the development of specific component of the immune system. Making a clinical diagnosis is challenging because of its variable presentation and diverse nature [1]. Imaging is frequently performed in the acute phase or with symptomatic sequelae. However, literature is not available for addressing the imaging pointers for specific type of defect [1, 2]. In this retrospective study, the authors attempted to analyse the frequency of morphological
Electronic supplementary material The online version of this article (https://doi.org/10.1007/s12098-020-03289-w) contains supplementary material, which is available to authorized users. * Manisha Jana [email protected] 1
Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi 110029, India
2
Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India
changes on CT in different types of defect, and to correlate imaging features with the type of defect.
Material and Methods After obtaining institute’s ethical committee approval, the authors retrospectively reviewed imaging records of 24 proven cases of PIDD from June 2016 through April 2018. Contrast enhanced CT (CECT) examinations were performed using multislice CT scanner (MDCT) (Supplementary Tables). Appropriate image reconstructions were done and imaging findings were recorded in a predesigned proforma. The radiologists were not aware of the final diagnosis or the type of defect. The f
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