대한흉부영상의학회 Weekly Chest Cases

Case No : 1250 Date 2021-10-05

Courtesy of Bo Da Nam, Jung Hwa Hwang / Department of Radiology, Soonchunhyang University Hospital, Seoul, Korea
Age/Sex 62 / M
Chief ComplaintIncidental finding with no subjective symptom

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Diagnosis With Brief Discussion

Diagnosis: IgG4-related disease
Radiologic Findings: Fig. 1. Initial chest radiography showing multiple patchy opacities in both the upper and middle lungs. No evidence of bilateral pleural effusion is identified.
Fig. 2. Follow-up chest radiograph after 5 months showing persistent and increased extent of multiple patchy or mass-like consolidations in the absence of treatment.
Fig. 3, 4. Contrast-enhanced chest CT images demonstrating multifocal areas of airspace consolidations with mild interstitial thickening and lung parenchymal distortion in both upper lobes.
Fig. 5. Mild subpleural reticulations are suspected in the peripheral lower lobes.
Fig. 6, 7. Multiple homogenously enhancing enlarged lymph nodes are noted in almost all nodal stations of both the mediastinum and hilum.
Brief Review: Open biopsy of the right lung and excision of the right mediastinal lymph nodes (#4) were performed, and the final diagnosis was confirmed.
Histopathologic examination showed chronic inflammation with diffuse lymphoplasmacytic infiltration and fibrosis. Immunoglobulin (Ig)G4 immunostaining revealed abundant IgG4-positive plasma cells in the specimen (> 60 per high-power field and IgG4/IgG ratio > 80%). The serum IgG level was 2,322 mg/dL (normal range: 700–1,600 mg/dL), and the IgG4 subclass level was 293.7 mg/L (normal range: 15–157 mg/L).
IgG4-related disease (also called IgG4-related sclerosing disease, IgG4-related systemic disease, or hyper-IgG4 disease) is a systemic fibroinflammatory disease associated with elevated circulating levels of IgG4.
Intrathoracic involvement in IgG4-related disease has been described in the lung parenchyma, airways, pleura, and mediastinum. Inoue et al. described four subtypes of lung parenchymal involvement: solid nodular type, round-shaped ground-glass opacity (GGO) type, alveolar interstitial type, and bronchovascular type. Solid nodular and round GGO lesions can mimic primary lung cancer. The alveolar interstitial type is characterized by diffuse GGO, bronchiectasis, and honeycombing, which are suggestive of nonspecific interstitial pneumonia. The bronchovascular type is characterized by thickening of the bronchovascular bundles and interlobular septa, often associated with lymph node swelling in the hilum and mediastinum. Sarcoidosis or lymphoproliferative disorder is suspected on radiologic examination.
The most common intrathoracic manifestations may be mediastinal and/or hilar lymphadenopathies. Fibrosing mediastinitis is an unusual mediastinal manifestation of IgG4-related diseases. Airway manifestations include tracheobronchial stenosis or extrinsic compression of the central airways due to fibrosing mediastinitis and bronchiectasis. Pleural disease has been reported as nodular lesions in the visceral or parietal pleura, and pleural effusion is an uncommon feature in patients with IgG4-related disease.
In our case, the patient showed typical CT features of IgG4-related disease, including lung parenchymal involvement (nodular and alveolar interstitial types) and mediastinal involvement.
Serum IgG4 levels are elevated in most patients with IgG4-related lung disease. Pathologically, it is characterized by diffuse lymphoplasmacytic infiltration, fibrosis, and IgG4 immunostaining positivity. Intrathoracic IgG4-related disease generally responds well to steroid treatment; however, azathioprine, methotrexate, or rituximab therapy could be applied to steroid-resistant IgG4-related lung disease.
Please refer to: KSTR imaging conference 2018 Summer Case 13 ,
KSTR Imaging Conference 2016 Spring Case 9 ,
KSTR Imaging Conference 2014 Summer Case 5,
References: 1. D. Inoue, Y. Zen, H. Abo,T. Gabata, H. Demachi, T. Kobayashi, et al. Immunoglobulin G4 related lung disease: CT findings with pathologic correlations. Radiology 2009; 251: 260-270.
2. J.H. Ryu, H. Sekiguchi and E.S. Yi Pulmonary manifestations of immunoglobulin G4-related sclerosing disease. EurRespir J 2012; 39: 180-186
3. S.Matsui, A. Hebisawa, F. Sakai, H. Yamamoto, Y. Terasaki, Y. Kurihara, et al. Immunoglobulin G4-related lung disease: Clinicoradiological and pathological features. Respirology 2013; 18, 480-487
3. Steven C.W., John KF. IgG4-Related Disease, Arch Pathol Lab Med 2017; 141(11):1476-1483
4. Chiu-Fan, Kuo-An, Yen-Chiang, Chang-Che, Ruay-Sheng. IgG4-related lung disease presenting as interstitial lung disease with bronchiolitis. Medicine 2017; 96(49):e9140
Keywords

No. of Applicants : 89

▶ Correct Answer : 5/89, 5.6%

- ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
- Other , Korea (South) HYEJIN YANG
- Shimada General Medical Center , Japan HAYATO NOZAWA
- Seoul National University Bundang Hospital , Korea (South) SUNG HYUN YOON
- the first affiliatited hospital of nanjing medical univercity , China HAI XU

▶ Correct Answer as Differential Diagnosis : 30/89, 33.7%

- Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
- Mayo Clinic , United States AKITOSHI INOUE
- Other , Korea (South) MINGI SHIN
- Osaka Red Cross Hospital , Japan YUSUKE UTSUNOMIYA
- National Center of Neurology and Psychiatry , Japan MOTO NAKAYA
- , Italy PAOLO BALDASSARI
- Tiger Gate Hospital , Japan SHIN-ICHI CHO
- Ichinomiya Nishi Hospital , Japan CHIHIRO KIGUCHI
- Vita Hospital , Brazil DIOGO LAGO PINHEIRO
- Other , Korea (South) SEONGSU KANG
- Chonbuk National University Hospital , Korea (South) JUNGHWAN KIM
- Osaka University , Japan AKINORI HATA
- National Center for Global Health and Medicine , Japan MASATOSHI HOTTA
- The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
- Inje University Pusan Paik Hospital , Korea (South) JIYEON HAN
- Yeungnam University Medical Center , Korea (South) JONGSOO PARK
- Gifu University Hospital , Japan Yo Kaneko
- Mallinckrodt Institute of Radiology , United States NAGANATHAN MANI
- Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
- Seoul Veterans Hospital , Korea (South) SEONG WON JANG
- Dokkyo Medical University , Japan HIROAKI ARAKAWA
- Other , Korea (South) KYU-CHONG LEE
- Kyoto University Hospital , Japan SATOSHI IKEDA
- Toranomon Hospital , Japan KAORU SUMIDA
- Ichinomiya Nishi Hospital , Japan Takao Kiguchi
- The University of Tokyo Hospital , Japan HIROSHI TAKUMIDA
- , Japan KAZUMA TERAUCHI
- Private sector , Greece VASILIOS TZILAS
- , Japan YUKI HAYASHI
- , Korea (South) WONJU HONG

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 1250 Date 2021-10-05

Discussion

No. of Applicants : 89