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.
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 3A and figure 3B represented onset symptoms and organs affected of IgG4-RD with PAO/PA. * represented P<0.05, *** represented P<0.001.
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 December 1, 2020.« Back to 2019 ACR/ARP Annual Meeting
ACR Meeting Abstracts - 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/