Clinical and pathological characteristics of IgG4‑related interstitial lung disease

Abstract

IgG4-related interstitial lung disease (IgG4-RILD), which is characterized by increased IgG4 levels, IgG4+ plasma cell infiltration and irregular whorled fibrosis, is a recently described lung disorder that belongs to the group of systemic fibroinflammatory IgG4-related diseases (IgG4-RD). The aim of the present study was to improve the current knowledge regarding the specific clinical and histopathological characteristics of IgG4-RILD and to investigate its underlying immune mechanism in vivo. A total of 7 patients newly diagnosed with IgG4-RILD were enrolled in the present study (4 men and 3 women; mean age, 57 years; range, 29–71 years). Patients' clinical history was collected and serological indicators, including C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), antinuclear antibodies (ANAs) and tumor markers were measured. Serum immunoglobulin G (IgG), IgE and IgG4 levels were also evaluated. In addition, computed tomographic (CT) images and pathological examinations were used to determine the characteristics of lung lesions in all patients. The majority of patients presented with symptoms of fever, cough and dyspnea, while allergic symptoms were also encountered. The laboratory examination results revealed different degrees of increased CRP, ESR, tumor markers, ANA, serum IgE and IgG4. The CT images revealed diffuse ground glass opacities, bronchiectasis and thickened bronchovascular bundles. Histologically, the lung lesions were characterized by dense IgG4+ lymphoplasmacytic infiltrates intermixed with extensive fibrous tissue hyperplasia and an irregularly storiform pattern of fibrosis. The mean number of IgG4+ plasma cells was >10 cells/high power field. The ratio of IgG/IgG4+ plasma cells was >50% in inflamed lesions and the number of parenchymal cells was markedly reduced. Obliterative phlebitis or obliterative arteritis was observed in all patients. In conclusion, the clinicopathological similarities between IgG4-RILD and other IgG4-RD suggest that IgG4-related immunopathological processes may be associated with the pathogenesis of pulmonary lesions. Future studies based on the findings herein may elucidate the specific pathological process underlying the development of this fibroinflammatory disorder.

Thoracic involvement and imaging patterns in IgG4-related disease

“…In addition, there were no major differences in gender, age, IgG4 level, asbestosis or tobacco exposure between patterns. We confirm as reported from other series that lung involvement in IgG4-RD is heterogeneous [6,7,[11][12][13][14][17][18][19][20]. The discrepancies with other series between the frequencies of lung abnormalities observed suggests either a bias related to retrospective data collection, the lack of central imagery reviewing in some studies or a role of ethnic background.…”

CALCB rs3829222 T/T Genotype and Low Expression of CALCB Are High-Risk Factors for Adenoid Cystic Carcinoma of Salivary Gland

“…Studies have shown that neurotropic metastasis is related to the fusion of multiple genes in the host and gene mutations. There is no mention of the incidence-related genotype [ 18 ]. In lung cancer research, it was found that the CALCB gene polymorphism is associated with the incidence of lung cancer [ 19 ].…”

Inflammatory abdominal aortic aneurysms: place of Ig G4related diseases

“…IgG4-связанное склерозирующее заболевание, так называемая IgG4-связанная склерозирующая болезнь (IgG4-ССБ) впервые описана применительно к аутоиммунному панкреатиту (склерозирующему панкреатиту) [6]. Впоследствии описание этого заболевания включило в себя поражение других органов, в том числе желчного протока, слюнной железы, забрюшинного пространства и легкого [7]. Вне зависимости от пораженного органа, IgG4-ССБ демонстрирует общие клинико- При хирургическом вмешательстве из-за присутствия при воспалительной АБА плотного фиброзного сращения со структурами, прилегающими к брюшной аорте, рассечение затруднено, а непреднамеренное прокалывание аневризмы может привести к летальным осложнениям [4].…”

“…До сих пор интерпретация воспалительной АБА неоднозначна. Некоторые исследователи рассматривают воспалительную АБА как крайний случай атеросклеротической АБА [5,7], тогда как другие предлагают видеть в воспалительной АБА аутоиммунный или инфекционный процесс [8,9]. Мы изучили воспалительную АБА с точки зрения IgG4-ССБ и пришли к выводу, что воспалительные АБА можно разделить на два типа: IgG4-связанные и IgG4-несвязанные случаи [9].…”