ABSTRACT
Background
Methods
Results
Conclusions
Graphical abstract

Key words
INTRODUCTION
- Zheng Y
- Tan AW
- Yong TH
- Chai SC
PATIENTS AND METHODS
Patients
- Umehara H
- Okazaki K
- Masaki Y
- Kawano M
- Yamamoto M
- Saeki T
- Matsui S
- Yoshino T
- Nakamura S
- Kawa S
- Hamano H
- Kamisawa T
- Shimosegawa T
- Shimatsu A
- Nakamura S
- Ito T
- Notohara K
- Sumida T
- Tanaka Y
- Mimori T
- Chiba T
- Mishima M
- Hibi T
- Tsubouchi H
- Inui K
- Ohara H
- Wallace ZS
- Naden RP
- Chari S
- Choi HK
- Della-Torre E
- Dicaire JF
- Hart PA
- Inoue D
- Kawano M
- Khosroshahi A
- Lanzillotta M
- Okazaki K
- Perugino CA
- Sharma A
- Saeki T
- Schleinitz N
- Takahashi N
- Umehara H
- Zen Y
- Stone JH
- Abla O
- Jacobsen E
- Picarsic J
- Krenova Z
- Jaffe R
- Emile JF
- Durham BH
- Braier J
- Charlotte F
- Donadieu J
- Cohen-Aubart F
- Rodriguez-Galindo C
- Allen C
- Whitlock JA
- Weitzman S
- McClain KL
- Haroche J
- Diamond EL
Image evaluation
- Mizushima I
- Kasashima S
- Fujinaga Y
- Notohara K
- Saeki T
- Zen Y
- Inoue D
- Yamamoto M
- Kasashima F
- Matsumoto Y
- Amiya E
- Sato Y
- Yamada K
- Domoto Y
- Kawa S
- Kawano M
- Ishizaka N
Statistical analysis
RESULTS
Patient characteristics in this study
- Umehara H
- Okazaki K
- Masaki Y
- Kawano M
- Yamamoto M
- Saeki T
- Matsui S
- Yoshino T
- Nakamura S
- Kawa S
- Hamano H
- Kamisawa T
- Shimosegawa T
- Shimatsu A
- Nakamura S
- Ito T
- Notohara K
- Sumida T
- Tanaka Y
- Mimori T
- Chiba T
- Mishima M
- Hibi T
- Tsubouchi H
- Inui K
- Ohara H
Age (years) | 68.1 ± 7.7 |
---|---|
Sex (male), n (%) | 15 (78.9) |
Laboratory examinations | |
IgG4, mg/dL [11-121] | 661.0 (235.0–1290.0) |
IgG4 ≥ 135 mg/dL, n (%) | 17 (89.5) |
CRP, mg/dL [0-0.3] | 0.59 ± 0.81 |
Eosinophil, /μL [0-8.5%] | 200.0 (150.0–800.0) |
Diagnosis | |
Definite/probable/possible, n (%) | 7 (36.8) / 2 (10.5) / 10 (52.6) |
Duration of disease (years) | 8.2 ± 5.4 |
Treatment | |
Steroid therapy, n (%) | 15 (78.9) |
Azathioprine, n (%) | 4 (21.1) |
Methotrexate, n (%) | 1 (5.3) |
Involvement organs | |
Single organ, n (%) | 2 (10.5) |
Multiorgan (≥2 organs) , n (%) | 17 (89.5) |
Cardiovascular | |
Aortic periarterial lesion, n (%) | 12 (63.2) |
Coronary periarterial lesion, n (%) | 9 (47.4) |
Pericardial thickening, n (%) | 5 (26.3) |
Non-cardiovascular | |
Retroperitoneal fibrosis, n (%) | 7 (36.8) |
Eye, n (%) | 7 (36.8) |
Salivary, parotid, and submandibular gland, n (%) | 5 (26.3) |
Pancreas, n (%) | 5 (26.3) |
Bone, n (%) | 5 (26.3) |
Lymph node, n (%) | 4 (21.1) |
Lung, n (%) | 4 (21.1) |
Liver and biliary, n (%) | 2 (10.5) |
Kidney, n (%) | 2 (10.5) |
Medical history | |
Hypertension, n (%) | 16 (84.2) |
Diabetes, n (%) | 9 (47.4) |
Dyslipidemia, n (%) | 13 (68.4) |
Smoking history, n (%) | 11 (57.9) |
CKD, n (%) | 8 (42.1) |
CAEs, n (%) | 9 (47.3) |




Differences in clinical features and the frequency of CAEs between patients with and without CP
Coronary periarteritis (−), (n = 10) | Coronary periarteritis (+), (n = 9) | P-value | |
---|---|---|---|
Age (years) | 70.7 ± 5.6 | 65.2 ± 8.9 | 0.123 |
Sex (male), n (%) | 7 (70.0) | 8 (88.9) | 0.582 |
Medical history | |||
Hypertension, n (%) | 9 (90.0) | 7 (77.8) | 0.582 |
Diabetes, n (%) | 4 (40.0) | 5 (55.6) | 0.656 |
Dyslipidemia, n (%) | 6 (60.0) | 7 (77.8) | 0.628 |
Smoking history, n (%) | 5 (50.0) | 6 (66.7) | 0.645 |
CKD, n (%) | 4 (40.0) | 4 (44.4) | >0.999 |
Laboratory examinations | |||
IgG4, mg/dL [11-121] | 705.0 (206.5–1057.0) | 383.0 (230.0–1745.0) | 0.661 |
IgG4 ≥ 135 mg/dL, n (%) | 8 (80.0) | 9 (100.0) | 0.474 |
Diagnosis | |||
Definite/probable/possible, n (%) | 5 (50.0) / 2 (20.0) / 3 (30.0) | 2 (22.2) / 0 (0) / 7 (77.8) |

Differences in clinical features and the frequency of CAEs between patients with and without AP
Aortic periarteritis (−), (n = 7) | Aortic periarteritis (+), (n = 12) | P-value | |
---|---|---|---|
Age (years) | 69.1 ± 7.1 | 67.5 ± 8.2 | 0.666 |
Sex (male), n (%) | 6 (85.7) | 9 (75.0) | >0.999 |
Medical history | |||
Hypertension, n (%) | 5 (71.4) | 11 (91.7) | 0.523 |
Diabetes, n (%) | 4 (57.1) | 5 (41.6) | 0.650 |
Dyslipidemia, n (%) | 3 (42.9) | 10 (83.3) | 0.129 |
Smoking history, n (%) | 5 (71.4) | 6 (50.0) | 0.633 |
CKD, n (%) | 1 (14.3) | 7 (58.2) | 0.147 |
Laboratory examinations | |||
IgG4, mg/dL [11-121] | 979.0 (205.0–1950.0) | 341.0 (240.0–816.0) | 0.340 |
IgG4 ≥ 135 mg/dL, n (%) | 6 (85.7) | 11 (91.7) | >0.999 |
Diagnosis | |||
Definite/probable/possible, n (%) | 4 (57.1) / 1 (14.3) / 2 (28.6) | 3 (25.0) / 1 (8.3) / 8 (66.7) | |
Retroperitoneal fibrosis, n (%) | 1 (14.3) | 6 (50.0) | 0.173 |

Pericardial thickening (−), (n = 14) | Pericardial thickening (+), (n = 5) | P-value | |
---|---|---|---|
Age (years) | 69.7 ± 7.8 | 63.6 ± 5.6 | 0.130 |
Sex (male), n (%) | 11 (78.6) | 4 (80.0) | >0.999 |
Medical history | |||
Hypertension, n (% | 13 (92.9) | 3 (60.0) | 0.155 |
Diabetes, n (%) | 6 (42.9) | 3 (60.0) | 0.629 |
Dyslipidemia, n (%) | 9 (64.3) | 4 (80.0) | >0.999 |
Smoking history, n (%) | 7 (50.0) | 4 (80.0) | 0.338 |
CKD, n (%) | 4 (28.6) | 4 (80.0) | 0.110 |
Laboratory examinations | |||
IgG4, mg/dL [11-121] | 705.0 (193.0–1057.0) | 383.0 (264.5–1790.0) | 0.500 |
IgG4 ≥ 135 mg/dL, n (%) | 12 (85.7) | 5 (100.0) | >0.999 |
Diagnosis | |||
Definite/probable/possible, n (%) | 5 (35.7) / 2 (14.2) / 7 (50.0) | 2 (40.0) / 0 (0) / 3 (60.0) |

Summaries of the IgG4-RD patients with pericardial thickening
DISCUSSION
- Sakamoto A
- Nagai R
- Saito K
- Imai Y
- Takahashi M
- Hosoya Y
- Takeda N
- Hirano K
- Koike K
- Enomoto Y
- Kume H
- Homma Y
- Maeda D
- Yamada H
- Fukayama M
- Hirata Y
- Ishizaka N
- Paratz ED
- Ross L
- Zentner D
- Morgan N
- Bouwer H
- Lynch M
- Parsons S
- La Gerche A
- Akiyama M
- Kaneko Y
- Takeuchi T
CONCLUSION
Acknowledgments
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Conflict of interest
EA belongs to the Department, endowed by NIPRO-Corp., Terumo-Corp., Senko Medical-Instrument-Mfg., Century-Medical, Inc., ONO-pharmaceutical-Co., Ltd. Medtronic-JAPAN Co., Ltd, Nippon-Shinyaku Co., Ltd, Mochida Pharmaceutical Co., Boehringer Ingelheim Pharmaceuticals Inc., Abiomed-Inc, AQuA-Inc, Fukuda-Denshi Co., Ltd, and Sun-Medical-Technology-Research Corp.. EA received research fund from Bristol-Myers Squibb Co..
Data availability
The data that support the findings of this study are available from the corresponding author on reasonable request.
Authors’ contributions
HY, EA, and MU were directly involved in managing the cases. SM, MH, NT, HA, and IK critically revised the manuscript for important intellectual content. All authors approved the content of the manuscript and confirmed the accuracy and integrity of any part of the work.
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