Radiological Manifestations of Common Variable Immuodeficiency Sydrome (CVID) and associated complications

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1 Radiological Manifestations of Common Variable Immuodeficiency Sydrome (CVID) and associated complications Poster No.: P-0034 Congress: ESTI 2014 Type: Educational Poster Authors: A. Wallis, C. Ball, K. Jayawardhana, P. McParland, R Dickens ; Portsmouth/UK, Brighton/UK, Ha/UK Keywords: Infection, Decision analysis, CT, Lung DOI: /esti2014/P-0034 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 12

2 Learning objectives Review the typical imaging features of CVID. Highlight features that should alert the radiologist to complications of CVID including Granulomatous and Lymphocytic Interstitial Lung Disease (GLILD). Background Common Variable Immunodeficiency Syndrome (CVID): The most common immunodeficiency syndrome requiring medical treatment with an incidence of 1 in 10,000. Commonly diagnosed between 3rd and 4th decades of life. Characterized by hypogammaglobulinaemia and impaired antigen response predisposing patients to recurrent infections and bronchiectasis. Extrapulmonary features may include infections, autoimmune disease (10%), arthritis, neoplasia (16%, commonly lymphoma and gastric cancer), granulomatous disease of the abdominal solid organs and lymphoid hyperplasia of the liver and GIT 2, 4,5,7,8. Granulomatous-Lymphocytic Interstial Lung disease (GL-ILD): A distinct histopathological complication of CVID with a prevalance reported 3,5,6,9 to range from 5-10%. Reported to reduce the median survival of those suffering CVID by more than 50%. Characterized by follicular bronchiolitis, LIP and granulomata formation and is associated with a poorer prognosis. Other causes of granulomatous and lymphoproliferative lung disorders must be excluded to diagnose GL-ILD. Imaging findings OR Procedure details Fig 1 summarises the main radiological features that differentiate CVID from GL-ILD. CVID: Page 2 of 12

3 Patients with CVID are predisposed to recurrent infections. Therefore the most typical thoracic imaging manifestations are of pneumonia and with repeated infections bronchiectasis and associated findings of bronchial wall thickening, atelectasis and air trapping (Fig 2-4). Other possible complications include organizing pneumonia, hypersensitivity pneumonitis and obliterative bronchiolitis. Patients are also at increased risk of lymphoproliferative disease and mortality in patients with CVID is most commonly due to malignancy including B cell lymphoma. GL-ILD: GL-ILD displays distinct radiological appearance from those of uncomplicated CVID. GL-ILD is a sarcoid-like, non-necrotising infiltration of the lung, with a prevalence of between 5% and 10% in patients with CVID. Typical imaging features of GL-ILD include soft tissue and ground glass micronodules and thoracoabdominal lymphadenopathy (Fig 5). A mid and lower lung predominance helps differentiate patients with GL-ILD from those with sarcoid as does the common finding of associated bilateral smooth interlobular septal thickening (Fig 6,7). Bronchiectasis is typically less common in GL-ILD vs CVID. Patients typically have splenomegaly (Fig 8). Images for this section: Fig. 1: Radiological differentiation of CVID Versus GL-ILD. Page 3 of 12

4 Fig. 2: HRCT Thorax of an immunologically confirmed CVID patient displaying bronchiectasis, air trapping and tree in bud modularity. Page 4 of 12

5 Fig. 3: HRCT Thorax of an immunologically confirmed CVID patient displaying right upper lobe bronchiectasis. Page 5 of 12

6 Fig. 4: HRCT Thorax of the same immunologically confirmed CVID patient as Figure 3 displaying left lower lobe bronchiectasis. Page 6 of 12

7 Fig. 5: Case of pathologically proven GL-ILD displaying mediastinal lymphadenopathy. Page 7 of 12

8 Fig. 6: HRCT Thorax of a case of GL-ILD displaying septal thickening and diffuse lung nodularity. Bronchiectasis is not a dominant feature, allowing differentation from typical CVID. Page 8 of 12

9 Fig. 7: Same case of pathologically proven GL-ILD as Figures 6 and 8 displaying diffuse modularity and mild septal thickening. Page 9 of 12

10 Fig. 8: Same case of pathologically proven GL-ILD as Figures 6 and 7 displaying splenomegaly. Page 10 of 12

11 Conclusion CVID is a common immune deficiency syndrome and as such radiologists must be aware of the usual imaging manifestations. GL-ILD is a rare complication of CVID and requires a multidisciplinary approach for confident diagnosis. The radiologist should be alert to the imaging manifestations of GL-ILD due to its impact on both prognosis and treatment. References 1. Torigian DA, LaRosa DF, Levinson AI, Litzky LA and Miller WT. Granulomatous-Lympocytic Interstial Lung Disease Associated With Common Variable Immunodeficiency CT Findings. Thorac Imaging 2008; 23: Quinti I, Soresina A, Spadaro G, et al. Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency. J Clin Immunol. 2007;27: Bates CA, Ellison MC, Lynch DA, et al. Granulomatouslymphocytic lung disease shortens survival in common variable immunodeficiency. J Allergy Clin Immunol. 2004;114: Cunningham-Rundles C, Bodian C. Common variable immunodeficiency: clinical and immunological features of 248 patients. Clin Immunol. 1999;92: Mechanic LJ, Dikman S, Cunningham-Rundles C. Granulomatous disease in common variable immunodeficiency. Ann Intern Med. 1997;127(8 Pt 1): Fasano MB, Sullivan KE, Sarpong SB, et al. Sarcoidosis and common variable immunodeficiency. Report of 8 cases and review of the literature. Medicine (Baltimore). 1996;75: Kinlen LJ, Webster AD, Bird AG, et al. Prospective study of cancer in patients with hypogammaglobulinaemia. Lancet. 1985;1: Page 11 of 12

12 8. Spector BD, Perry GS III, Kersey JH. Genetically determined immunodeficiency diseases (GDID) and malignancy: report from the immunodeficiency-cancer registry. Clin Immunol Immunopathol. 1978;11: Hermaszewski RA, Webster AD. Primary hypogammaglobulinaemia: a survey of clinical manifestations and complications. Q J Med. 1993;86: Routes J. Granulomatous and Lymphocytic Interstitial Lung Disease (GLILD) in CVID. Clevelandclinicmeded.com. Personal Information Page 12 of 12