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Abstract Number: 945

Comparison of Clinical Manifestations in IgG4 Related Disease Patients with/without Aortitis/Periaortitis and Periarteritis: A Prospective Cohort Study of 587 Patients with IgG4-RD Disease

Linyi Peng 1, Wen Zhang 2 and Panpan Zhang1, 1Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China (People's Republic), 2Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China (People's Republic)

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: aortitis, IgG4 Related Disease, periaortitis/periarteritis and treatment

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Session Information

Date: Sunday, November 10, 2019

Session Title: 3S112: Vasculitis – Non-ANCA-Associated & Related Disorders I: Miscellaneous Disorders (945–950)

Session Type: ACR Abstract Session

Session Time: 4:30PM-6:00PM

Background/Purpose: We aimed to clarify the clinical features of IgG4-related disease (IgG4-RD) with/without aortitis/periaortitis and periarteritis (PAO/PA), and evaluate the treatment efficacy of patients with PAO/PA.

Methods: A total of 587 IgG4-RD patients with a follow up time of more than 6 months were enrolled in this study. The distribution of IgG4 related PAO/PA was classified into four types: type 1, thoracic aorta affected; type 2a, abdominal aorta affected, type 2b, abdominal aorta and iliac artery affected, type 2c, iliac artery affected; type 3, thoracic and abdominal aorta affected; type 4, other artery affected. The demographic data, clinical characteristics, laboratory parameters and treatment efficacy of enrolled patients were analyzed.

Results: Of 587 IgG4-RD patients, 89 (15.2%) had PAO/PA. The age of PAO/PA patients was 58.3 ± 11.1 years, and there was a male dominance(85.4%, 76 male patients). The percentage of vessels affected in IgG4 related PAO/PA was as follows: the abdominal aorta affected was 83.1%, the iliac artery affected was 70.8%, the thoracic aorta affected was 13.5% and the other vessels were affected. The most prevalent type of PAO/PA was type 2B, with 74 (83.1%) patients, followed by type 2A, type 2C, type 3 and type 1. Compared with patients without PAO/PA, patients with PAO/PA had higher percentage of back pain, and abdominal pain, the percentage of dacryoadenitis/ sialadenitis and paranasal sinus affected was lower. ESR and hsCRP was higher in patients with PAO/PA, whereas serum IgG4 and T-IgE levels were lower compared with patients without PAO/PA. After treatment with glucocorticoid and immunosuppressants, patients IgG4-RD RI, ESR, hsCRP, serum IgG4 and T-IgE  levels decreased significantly. 44 (38.2%) patients achieved a remission with the reduction of abnormal soft tissues more than 70%, 39 (43.8%) with a reduction of 31%-70%, and 16 (18.0%) patients with a reduction of less than 30%.

Conclusion: The most common affected vessel in patients with PAO/PA was abdominal aorta, and type 2b was the predominant distribution of PAO/PA. Patients with PAO/PA had lower percentage of allergy history, dacryoadenitis/sialadenitis and paranasal sinus affected, lower serum IgG4 levels, T-IgE, higher IgG4-RD RI, ESR and hsCRP than patients without PAO/PA.

Vessels affected of IgG4-RD patients with PAO/PA
Figure 1A represented vessels affected of patients with PAO/PA. Figure 1B represented the distribution of IgG4 related PAO/PA. figure 1C and 1D represented the comparison of vessel distribution between male and female patients, PAO/PA merely and PAO/PA respectively

Figure 2A represented the reduction of soft tissues around PAO/PA. figure 2B represented the comparison of soft tissues reduction of PAO/PA between male and female patients. Figure 2C was the recovery of nephrosis, renal function and D-J tube implacement of patients before and after treatment. Figure 2D represented the remission rate of patients with PAO/PA. figure 2E to 2I represented IgG4-RD RI, ESR, hsCRP, serum IgG4 and T-IgE respectively before and after treatment.

Figure 3 Onset symptoms and organs affected of patients with/without PAO/PA
Figure 3A and figure 3B represented onset symptoms and organs affected of IgG4-RD with PAO/PA. * represented P<0.05, *** represented P<0.001.


Disclosure: L. Peng, None; W. Zhang, None; P. Zhang, None.

To cite this abstract in AMA style:

Peng L, Zhang W, Zhang P. Comparison of Clinical Manifestations in IgG4 Related Disease Patients with/without Aortitis/Periaortitis and Periarteritis: A Prospective Cohort Study of 587 Patients with IgG4-RD Disease [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/comparison-of-clinical-manifestations-in-igg4-related-disease-patients-with-without-aortitis-periaortitis-and-periarteritis-a-prospective-cohort-study-of-587-patients-with-igg4-rd-disease/. Accessed June 1, 2023.
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