Background: Large vessels could be involved in immunoglobulin (Ig)-G4-related disease (IgG4-RD). This study aimed to clarify the clinical features and evaluate the treatment efficacy for IgG4-RD with aortitis/periaortitis and periarteritis (PAO/PA).Methods: This study prospectively enrolled 587 patients with IgG4-RD with a follow-up time of more than 6 months. The distribution of IgG4-related PAO/PA was classified into four types: type 1, thoracic aorta; type 2a, abdominal aorta; type 2b, abdominal aorta and iliac artery; type 2c, iliac artery; type 3, thoracic and abdominal aorta; type 4, other arteries. Patient’s demographic data, clinical characteristics, laboratory parameters, and treatment efficacy were analyzed.Results: Of 587 IgG4-RD patients, 89 (15.2%) had PAO/PA. The average age was 58.3±11.1 years, with male predominance (85.4%). Vessels affected were as follows: abdominal aorta (83.1%), iliac artery (70.8%), thoracic aorta (13.5%), and other vessels (13.5%). The most prevalent distribution type of IgG4-related PAO/PA was type 2b, with 74 (83.1%) patients, followed by type 2a, type 2c, type 3, and type 1. Fifty-five (61.8%) PAO/PA patients had hydronephrosis, with renal insufficiency occurring in 43 (48.3%), and 31 (34.8%) PAO/PA patients had D-J stent drainage due to severe ureteral obstruction. After treatment with a glucocorticoid and immunosuppressants, 82% patients achieved remission with shrinking of the perivascular mass by more than 30%.Conclusions: IgG4-RD with PAO/PA was distinct from non-PAO/PA in demographic features, organ involvement distribution, inflammatory markers, and serum IgG4 and IgE. The most common affected vessel was the abdominal aorta, and most patients responded well with treatment.
Background Immunoglobulin G4 related disease (IgG4-RD) is a newly recognized systemic, immune-mediated and fibro-inflammatory disease. Hypocomplementemia was found in part of IgG4-RD patients especially in the setting of active disease. Objectives This study aimed to clarify the clinical features, treatment efficacy and outcome in IgG4-RD patients with hypocomplementemia. Methods 312 IgG4-RD patients were recruited in our prospective cohort conducted in Peking union medical college hospital. Patient’s demographic data, clinical characteristics, laboratory parameters, treatment and outcome were analyzed. Results Hypocomplementemia was identified in 65(20.8%) cases of untreated IgG4-RD patients at baseline. The average age of hypocomplementemia group was 55.85±10.89 years, with male predominance (72.3%). Compared with normal complement group, patients with hypocomplementemia were likely to have more involved organs,higher IgG4-RD responder index (IgG4-RD RI), higher laboratory parameters such as counts of eosinophils, inflammatory markers༌immunoglobulin G(IgG), IgG1, IgG3, IgG4 and IgE. In addition, lymph nodes, lacrimal gland༌submandibular gland༌parotid gland༌paranasal sinus༌bile ducts and prostate gland were more commonly affected(p < 0.05). Serum C3 and C4 were negatively correlated with the number of involved organs, IgG4-RD RI, hypersensitive C-reactive protein (hsCRP), IgG, IgG1, IgG3 and IgG4. 64(98.5%) patients responded quickly to initial therapy at 3-month follow-up. Fifteen (23.1%) patients relapsed during follow-up with mean recurrence time of 14.2±13.8 months. Compared with normal complement group, there was no significant difference of relapse rate in two groups (P = 0.7559). Conclusions Clinical characteristics of IgG4-related disease with hypocomplementemia differs from normal complement group.Serum C3 and C4 at baseline before treatment could be biological markers for disease activity. IgG4-RD with hypocomplementemia responded well to treatment and had no significant difference of relapse rate in IgG4-RD with normal complement.
Background: Large vessels could be involved in IgG4-related disease(IgG4-RD).This study aimed to clarify the clinical features and evaluate the treatment efficacy for IgG4-RD with aortitis/periaortitis and periarteritis (PAO/PA). Methods: This study enrolled 587 IgG4-RD patients in a prospective cohort with a follow-up time for more than 6 months. The distribution of IgG4-related PAO/PA was classified into four types: type 1, thoracic aorta; type 2a, abdominal aorta; type 2b, abdominal aorta and iliac artery; type 2c, iliac artery; type 3, thoracic and abdominal aorta; type 4, other arteries. Patient’s demographic data, clinical characteristics, laboratory parameters, and treatment efficacy were analyzed. Results: Of 587 IgG4-RD patients, 89(15.2%) had PAO/PA. The average age was 58.3±11.1 years, with male predominance (85.4%). Vessels affected were as follows: abdominal aorta (83.1%), iliac artery (70.8%), thoracic aorta (13.5%) and other vessels (13.5%). The most prevalent distribution type of IgG4-related PAO/PA was type 2b, with 74 (83.1%) patients, followed by type 2a, type 2c, type 3, and type 1. 55 (61.8%) PAO/PA patients had hydronephrosis, with renal insufficiency occurred in 43 (48.3%), and 31 (34.8%) PAO/PA patients had D-J stent drainage due to severe ureteral obstruction. After treatment with glucocorticoid and immunosuppressants, 82% patients achieved a remission with shrinking of perivascular mass. Conclusions: IgG4-RD with PAO/PA was distinct from non-PAO/PA in demographic features, organs involvement distribution, inflammatory markers, serum IgG4 and IgE. The most common affected vessel was abdominal aorta, and most patients responded well with treatment.
Objective: The aim of this study was to investigate the role of serum IgE level in the clinical features and outcome of IgG-4 related disease (IgG4-RD). Methods: We retrospectively enrolled 459 newly diagnosed IgG4-RD patients with serum IgE examined at baseline from 2012 to 2019, and compared the clinical features between high IgE group (Serum IgE level>60KU/L) and normal IgE group (Serum IgE level≤60KU/L) patients. Then 312 patients who have been followed up for ≥ 1 year were further selected to evaluate the correlation of serum IgE level and disease outcome. Results: At baseline, Serum IgE level was positively correlated with serum IgG4 level (r=0.1779, P=0.0001), eosinophil count (r=0.3004, P<0.0001), serum IgG level (r=0.2189, P<0.0001) in IgG4-RD patients. Compared with normal IgE group patients, high IgE group patients had more patients with allergy disease (P=0.004), more organ involvements (P=0.003), and higher IgG4-RD RI scores (P=0.002). During follow-up, normal IgE group patients had higher remission induction rate than high IgE group patients (88.4% vs. 73.6%, P=0.035), while high IgE group patients had more relapse rate compared with normal IgE group patients (29.0% vs. 16.2%, P=0.039), and relapsed patients had higher serum IgE level at baseline (P=0.046). Cox regression analysis showed that elevation of the eosinophil count was the risk factors of relapse for both normal and high IgE groups patients (HR 8.504 (1.071-42.511), P=0.009; HR 2.078 (1.277-3.380), P=0.003), and the involvement of lacrimal gland (HR 1.756 (1.108-2.782), P=0.017), submandibular gland (HR 1.654 (1.037-2.639), P=0.035), kidney (HR 3.413 (1.076-10.831), P=0.037) were also the risk factors of relapse for high IgE group patients.Conclusion: IgG4-RD patients with high serum IgE level at baseline were more likely to have higher disease activity, and baseline high IgE level were associated with disease relapse.
Objective: The aim of this study was to investigate the role of serum IgE levels in the clinical features and outcomes of IgG4-related disease (IgG4-RD). Methods: We retrospectively enrolled 459 newly diagnosed IgG4-RD patients with serum IgE examined at baseline from 2012 to 2019 and compared the clinical features between group A (serum IgE level ≤ 60 KU/L) and group B (serum IgE level > 60 KU/L). Subsequently, 312 patients who had been followed up for ≥ 1 year were further selected to evaluate the correlation between serum IgE level and disease outcome. Results: At baseline, the serum IgE level was positively correlated with the serum IgG4 level (r=0.1779, P=0.0001), eosinophil count (r=0.3004, P<0.0001), and serum IgG level (r=0.2189, P<0.0001) in IgG4-RD patients. Compared with group A, group B had more patients with allergic diseases (P=0.004), more organ involvement (P=0.003), and higher IgG4-RD responder index scores (P=0.002). During follow-up, group A patients had a higher remission induction rate than group B patients (88.4% vs. 73.6%, P=0.035), while group B patients had a higher relapse rate than group A patients (29.0% vs. 16.2%, P=0.039). Multivariate analysis found that a serum IgE level > 125 KU/L at baseline was a risk factor for disease relapse (hazard ratio [HR], 1.894 [95% confidence interval (CI) 1.022–3.508]; P=0.042). Cox regression analysis showed that elevation of the eosinophil count was a risk factor for relapse in both group A and group B patients (HR, 8.504 [95% CI 1.071–42.511]; P=0.009; and HR, 2.078 [95% CI 1.277–3.380]; P=0.003, respectively), and the involvement of the lacrimal gland (HR, 1.756 [95% CI 1.108–2.782]; P=0.017), submandibular gland (HR, 1.654 [95% CI 1.037–2.639]; P=0.035), and kidney (HR, 3.413 [95% CI 1.076–10.831]; P=0.037) were also risk factors for relapse in group B patients.Conclusion: IgG4-RD patients with high serum IgE levels at baseline were more likely to have higher disease activity, and baseline high IgE levels were associated with disease relapse.
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