THREE CASES OF IMMUNOGLOBULIN G(4)-RELATED RESPIRATORY DISEASE WITH UNCOMMON IMAGING FINDINGS


Özgül H. A., Gezer N. S., Özkal S., Tuna E. B., Özdemir N., Balcı P.

SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES, vol.38, no.2, 2021 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 38 Issue: 2
  • Publication Date: 2021
  • Doi Number: 10.36141/svdld.v38i2.10302
  • Journal Name: SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, EMBASE
  • Keywords: IgG4, Lung, Computed tomography, LUNG, MANIFESTATIONS, INFILTRATION, IGG4
  • Dokuz Eylül University Affiliated: Yes

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.