Three cases of immunoglobulin G4-related respiratory disease with uncommon imaging findings IgG4-related respiratory disease with uncommon imaging findings

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Hakan Abdullah Özgül
Naciye Sinem Gezer
Sermin Özkal
Emine Burçin Tuna
Nezih Özdemir
Pınar Balcı

Keywords

IgG4, Lung, Computed tomography

Abstract

Background: Immunoglobulin G4-related disease (IgG4-RD) is a rare multisystemic idiopathic fibroinflammatory disorder. The rare form of IgG4-RD with isolated thorax involvement is called immunoglobulin G4-related respiratory disease (IgG4-RRD). IgG4-RRD, which is reported in a limited number of cases in the literature, can be categorized into four types on the prevalent chest computed tomography (CCT) findings: solid nodular, round-shaped ground-glass opacity, alveolar interstitial, and bronchovascular. Solid nodular form of IgG4-RRD with mass-like lesions is sporadic and described in the literature with a small number of case reports.  Objectives/Methods: We aim to present the radiologic, pathologic, and clinical findings of three cases of IgG4-RRD mimicking lung cancer. Results: In all three patients, IgG4-RRD occurred with mass-like lesions in the thorax. In case-1 and 2, CCT showed multiple, nodular lesions and multiple mediastinal lymph nodes. On positron emission tomography with 2-deoxy-2-[fluorine-18] fluoro- D-glucose integrated with computed tomography (18F-FDG PET/CT), the masses showed increased 18F-FDG uptake in case-2 and 3. The gold standard histopathological verification for IgG4-RRD was provided for all cases. Conclusions: IgG4-RD is an immune-mediated condition comprised of a collection of disorders that share particular pathologic, radiologic, serologic, and clinical features. Isolated IgG4-RRD is rarely seen and is available in the literature as case reports. IgG4-RRD, which can make lung involvement in different patterns, rarely appears with mass-like lesions. Still, IgG4-RRD must be considered in the differential diagnosis of mass lesions detected in CCT. Laboratory, radiological, and histopathological findings of the disease should be evaluated together for an accurate diagnosis.

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References

1-Katabathina VS, Khalil S, Shin S, Lath N, Menias CO and Prasad S. Immunoglobulin G4–Related Disease: Recent Advances in Pathogenesis and Imaging Findings. Radiol Clin N Am 2016; 54: 535–551.
2-Umehara H, Okazaki K, Masaki Y, et al. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatology 2012; 22(1):1–14.
3-Saeki T, Saito A, Hiura T, Yamazaki H, Emura I, Ueno M, et al. Lymphoplasmacytic infiltration of multiple organs with immunoreactivity for IgG4: IgG4-related systemic disease. Intern Med 2006; 45: 163-7.
4- Ramponi S, Gnetti L, Marvisi M, Bertorelli G, and Chetti A. Lung Manifestations of IgG4-Related Disease. A Multifaceted Disorder. Sarcoidosis Vasc Diffuse Lung Dis 2018; 35:74–80.
5-Ryu JH, Sekiguchi H and Yi ES. Pulmonary manifestations of immunoglobulin G4-related sclerosing disease. Eur Respir J 2012; 39: 180–186.
6-Matsui S. IgG4-related respiratory disease. Modern Rheumatology 2019; 29:2, 251-256.
7-Khosroshahi A and Stone JH. A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol 2011; 23:57-66.
8-Kubo K and Yamamoto K. IgG4-related disease. International Journal of Rheumatic Diseases 2016; 19: 747–762.
9-Dai Inoue, Yoh Zen, Hitoshi Abo et al. Immunoglobulin G4 –related Lung Disease: CT Findings with Pathologic Correlations. Radiology 2009; 251(1):260-270.
10- Stone JH. IgG4-related disease: nomenclature, clinical features, and treatment. Seminars in Diagnostic Pathology 2012; 29:177-190.
11- Zen Y, Inoue D, Kitao A, et al. IgG4-related lung and pleural disease: a clinicopathologic study of 21 cases. Am J Surg Pathol 2009; 33:1886–1893.
12- Takato H, Yasui M, Ichikawa Y, et al. Nonspecific interstitial pneumonia with abundant IgG4-positive cells infiltration, which was thought as pulmonary involvement of IgG4-related autoimmune disease. Intern Med 2008; 47:291–294.
13- Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012; 366(6):539–51.
14- Ito M, Yasuo M, Yamamoto H, et al. Central airway stenosis in a patient with autoimmune pancreatitis. Eur Respir J 2009; 33:680–683.
15- Kobayashi H, Shimokawaji T, Kanoh S, Motoyoshi K, Aida S. IgG4-positive pulmonary
disease. J Thorac Imaging 2007; 22:360–362.
16-Wand O, Fox BD, Shtraichman O, Moreh-Rahav O, Kramer RM. Non-tuberculous, adenosine deaminase-positive lymphocytic pleural effusion: Consider immunoglobulin G4-related disease. Sarcoidosis Vasc Diffuse Lung Dis 2020; 37(2):225-230.
17- Matsui S, Hebisawa A, Sakai F, et al. Immunoglobulin G4-related lung disease: clinicoradiological and pathological features. Respirology 2013; 18(3):480–7.
18- Khosroshahi A, Wallace ZS, Crowe JL et al. International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Arthritis Rheumatol 2015; 67(7):1688–99.
19- Khosroshahi A, Bloch DB, Deshpande V, et al. Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related systemic disease. Arthritis Rheum 2010; 62(6):1755–62.