To explore the spectrum of the thoracic imaging manifestations demonstrated in patients with primary humoral immunodeficiency.
The imaging findings in primary immunodeficiency vary across the various primary immunodeficiencies.
Using a series of interesting cases,
this educational exhibit aims to:
Describe the early and late thoracic imaging findings.
Present the infectious presentations with the commonest pathogens (Streptococcus pneumoniae,
Illustrate the spectrum of the non-infectious airway’s disorders,
chronic lung changes and chronic inflammatory conditions (Granulomatosis,
Demonstrate the benign...
Primary humoral immunodeficiency constitutes the vast majority of all immunodeficiencies,
characterised by insufficient or impaired antibody production which,
can lead to increased susceptibility to infections.
The clinical presentation,
complications and the radiological appearances depend on the type of defects with a great variability between genotypes and phenotypes (Table 1 and 2).
Common variable immunodeficiency (CVID) and X-linked agammaglobulinaemia (XLA) are the commonest types of humoral immunodeficiency presenting with respiratory symptoms and a variety of pulmonary imaging findings.
Imaging findings OR procedure details
The imaging findings in primary immunodeficiency vary depending on the type of primary immunodeficiency.
The thoracic imaging findings can be broadly characterised into early and late findings.
The early findings encompass respiratory tract infections and small airways disorders whilst late manifestations include chronic lung changes as well as malignancy.
Patients with humoral immunodeficiencies often present with recurrent infections before a diagnosis of immunodeficiency is made.
The imaging patterns of infections in immunodeficiency are non-specific,
however the CT findings can narrow the differential diagnosis (typical...
There are various thoracic manifestations in primary immunodeficiency.
Imaging plays a crucial role in management for this patient group.
Awareness of the imaging findings found in the range of primary immunodeficiencies is crucial for early diagnosis and prompt management to improve prognosis and reduce morbidity and mortality.
Clinical and imaging considerations in primary immunodeficiency disorders: an update.Pediatr Radiol.
Primary immunodeficiencies underlying fungal infections.Curr Opin Pediatr.
Primary immunodeficiency diseases: an update on the classification from the international union of immunological societies expert committee for primary immunodeficiency.
Front Immunol 2014;5;460.
We hope you enjoy this educational exhibit.
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