Multiple nodules under the pericardium in a patient with IgG4-related disease
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Multiple nodules under the pericardium in a patient with IgG4-related disease Hui Lu 1 & Yaping Luo 2 & Jie Shi 3 & Shan Wang 3 & Changyan Liu 4 & Wen Zhang 1 Received: 18 May 2020 / Revised: 17 August 2020 / Accepted: 1 September 2020 # International League of Associations for Rheumatology (ILAR) 2020
Presentation A 73-year-old male was found with lung nodule during a regular checkup. He had a history of asthma and hypertension for many years. Besides, he had undergone a nasal polypectomy before. Physical examinations showed that the right submandibular gland was swollen and hard. The laboratory tests indicated increased erythrocyte sedimentation rate (33 mm/h, 0–15 mm/h), IgG (22.49 g/L, 7–17 g/L), IgG4 (15600 mg/L, 80–1400 mg/L), T-IgE (6040 KU/L, 0–60 KU/L), and normal high-sensitive C-reactive protein (1.51 mg/L, 0–8 mg/L) level. Antinuclear antibody was positive (1:160, membrane pattern), while other autoantibodies were negative. A whole-body 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/CT (PET/CT) was performed (Fig. 1a) and revealed enlargement and diffusely elevated uptake of bilateral submandibular glands (especially right side), patchy 18F-FDG-avid lesions in the prostate gland, a hypermetabolic nodule in the left lower lobe of the lung, and
multiple nodular lesions in the pericardium and mediastinum, as well as thickness and elevated uptake of bilateral iliac arteries and circumferential soft tissue (Fig. 1c, d, e, f, g, and h). Histopathological findings of the right submandibular gland biopsy revealed dense lymphoplasmacytic infiltration and fibrosis (Fig. 1i). Immunohistochemical staining demonstrated that IgG(+) (Fig. 1j) and IgG4+ cells were 30/HPF (Fig. 1k), IgG4+/IgG+ < 10%, CD3(+), CD35(+), CD21(+), and CD20(+). The patient was diagnosed with IgG4-related disease (IgG4-RD) according to the 2019 ACR/EULAR classification criteria [1]. He was treated with prednisolone 40 mg/day and cyclophosphamide 50 mg every other day. Two weeks later, prednisolone was tapered gradually until maintained at 7.5 mg/day. After treatment, serum IgG4 level declined to 4240 mg/L and he repeated 18F-FDG PET/CT, which showed complete remission of all affected lesions (Fig. 1b).
Discussion * Wen Zhang [email protected]
IgG4-RD is a multi-organ involved systemic disease and pericardial involvement mostly presents as pericarditis and pericardial thickening [2–4]. To our knowledge, pericardial nodules are unusual and there is no such report before. The same as commonly affected organs in IgG4-RD, pericardial nodules respond well to glucocorticoids.
1
Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), No. 1, Shuaifuyuan, Dongdan, Beijing 100730, China
2
Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, No. 1, Shuaifuyuan, Dongdan
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