316 The Korean Association of Internal Medicine

The Korean Association of Internal Medicine

316 32nd World Congress of Internal Medicine (October 24-28, 2014)

PS 1261 Pulmonology

A Case of Extensive IgG4-Related Disease Presenting as Mediastinal Mass, Massive Pleural Effusion and Mesen- teric Lymphadenopathy in a 16-Year-Old Male

Su-Young Jung¹, Ji-Eun Park¹, Hye-Sun Park¹, Seung-hee Han¹, Ji-yeon Lee¹, Joo Han Song², Song Yee Kim², Eun Young Kim², Ji Ye Jung², Young Ae Kang², Moo Suk Park², Young Sam Kim², Se Kyu Kim², Joon Chang², Kyoung Soo Chung²

Department of Internal Medicine, Yonsei University College of Medicine, Korea¹, Division of Pulmonology, Department of Internal Medicine, Institute of Chest Disease, Yonsei University College of Medicine, Ko- rea²

IgG4 related disease is a fi bro-infl ammatory disease, characterized by lymphoplasma- cytic infi ltration of various organs and frequently affected middle-aged males. Massive pleural effusion in children is an uncommon feature in IgG4 related pulmonary disease (IPD). We report a case of young male with extensive IPD presenting mediastinal mass, massive pleural effusion and mesenteric lymphadenopathy. A 16-year-old male was admitted for treatment on a recurrent bilateral pleural effusion. He has a history of admission to other hospital because of similar symptoms last year. At that time, Chest CT showed bilateral pleural effusion and anterior mediastinal soft tissue density. Vid- eo-assisted thoracoscopic mediastinal biopsy revealed angiomyolipoma. The follow-up chest CT fi ndings were not specifi c but progressive increase of pleural effusion may suggest diffuse pleural involvement of IgG4-related disease. Abdomen CT showed dif- fusely increased attenuation of the mesentery and enlarged mesenteric lymph nodes, which was also associated with IgG4-related disease. Our histopathological review of prior mediastinal biopsy was consistent with IgG4 related sclerosing disease rather than angiomyolipoma, which included papillary proliferation of reactive mesothelial cells and dense collagenous fi brosis with stromal dense lymphoplasmacytic infi ltration.

[IgG4-/IgG-(+) plasma cell>40%, IgG4: maximal count 62/HPF, HMB-45(-)] In addi- tion, serum IgG level was elevated (626mg/dl, normal range 700~1600mg/dl), as was the serum IgG4 level (1650mg/dl, normal range 110~1570md/dl), thereby confi rming a diagnosis of IgG4 related disease. The characteristic of his pleural fl uid was exudate and chylothorax (triglyceride 551mg/dl). He was treated with high dose steroid. The present case is the fi rst case of extensive IPD in a 16-year-old male in our country.

We recommend that clinicians consider IgG4-related disease as a possible differential diagnosis from the children with massive pleural effusion, elevation of serum IgG4/IgG and lymphoplasmacytic infi ltrated in pathology.

PS 1262 Pulmonology

Immunoglubulin G4-Related Disease Mimicking Meta- static Lung Cancer

Wookyung Sung¹, Min Soo Han¹, Kyoung Min Moon¹, Sun Young Jung¹, Yongseon Cho¹, Joo Heon Kim²

Department of Internal Medicine, Eulji University School of Medicine, Korea¹, Department of Pathology, Eulji University School of Medicine, Korea²

Immunoglobulin G4-related disease (IgG4RD) is characterized by elevated serum IgG4 concentration and tissue infi ltration by IgG4 positive plasma cells. IgG4RD is a mul- ti-organ disorder but rarely involving the lung. We report a case of Immunoglubulin G4-related disease mimicking metastatic lung cancer in a 74-year-old man presenting with pulmonary nodules. He had a history of subtotal gastrectomy for gastric cancer two years ago. The chest CT scan showed two pulmonary nodules in right middle lobe.

We considered these lesions as metastatic lung cancer, so transthoracic needle biopsy was performed. The biopsy revealed a dense lymphoplasmacytic infi ltration with nu- merous IgG4 positive plasma cells. The serum IgG4 level was 52.4 mg/dL. The patient was discharged with prednisolone. After corticosteroid therapy for 3 months, the size of nodules was markedly decreased.

PS 1263 Pulmonology

Immunoglobulin G4-Related Lung Disease Involving Mediastinal Lymph Nodes : A Case Report

Minji Kim¹, Sang Hee Lee¹, Jung Sup Eom¹, Mi Hyun Kim¹, Ki Uk Kim¹, Hye-Kyung Park¹, Eun Jung Cho¹, Jeong Ha Mok¹, Min Ki Lee¹, Kwangha Lee¹

Department of Internal Medicine, Pusan National University Hospital, Korea¹

We report on a rare case of Immunoglubulin (Ig) G4-related lung disease involving mediastinal lymph node, and mimicking a malignancy. A 58-year-old man was ad- mitted to our hospital after several weeks of fever, chilling, myalgia, and body weight loss. Chest computed tomography (CT) revealed multiple enlarged lymph nodes (LN) in neck, paratracheal, subcarinal, hilar, and axillary area. Malignancy (such as lymphoma) was suspected, the patient underwent video-assisted thoracic surgery of mediastinal LN. Biopsy showed reactive hyperplasia with signifi cantly increased plasma cell in- fi ltration, and immunohistochemical staining revealed that the lesion was composed mainly of IgG4 positive plasma cells, characterized by IgG4/IgG ratio >40%. His serum IgG level was increased (3732mg/dL, reference range 700-1600mg). Corticosteroid therapy was considered as effective for his diagnosis. After eleven weeks of steroid therapy, his chest CT showed interval decrease in the size of multiple LN. He was fi - nally diagnosed with IgG4-related lung disease.