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Background:In rheumatoid arthritis (RA), lung involvement includes interstitial lung disease (ILD) and airway disease (AD). While these have been reported to be associated with mortality, the impact of AD deterioration on prognosis remains unclear.Objectives:To investigate the relationship between radiographic deterioration of AD in RA and mortality.Methods:We retrospectively examined 720 RA patients who visited our department between 2011 and 2021. Among these, we enrolled patients who underwent chest computed tomography (CT) scans at least twice. The baseline was defined as the time of the initial chest CT scan, and the final observation as the final visit or death. CT findings were evaluated by two experienced radiologists. Patients whose RA-related lung involvement could not be fully evaluated from chest CT scans due to other causes were excluded. Radiographic definitions of AD and ILD followed the previous report (Ref. 1). The severity of AD was evaluated semi-quantitatively using radiographic grades ranging from 0 to 4. AD deterioration was defined as an increase in radiographic grade in grade 0–3 or an expansion of lesion areas in grade 4. Among patients complicated AD, we compared patients in the AD deterioration group with those in the non-AD deterioration group. In a multivariate Cox regression analysis, we identified factors associated with mortality by adjusting for age, sex, RA treatment, comorbidities including diabetes, deterioration of AD, and the presence of ILD and AD.Results:Among the 353 patients with repeated scans (mean age, 60.9 years.; female, 73.7%; mean observation period, 186.6 months), 126 had AD (35.7%), 102 had ILD (28.9%) at the baseline.In the subset of RA patients with complicated AD (126 cases), baseline characteristics (age, sex, RA disease activity, RF/ACPA positivity, RA treatment, presence of ILD, AD grade, comorbidities including diabetes, and observation period) were similar between the AD deterioration group (25 cases, 19.8%) and the non-AD deterioration group. However, mortality was significantly higher in the AD deterioration group (16.0% vs. 4.0%, p=0.049). Kaplan-Meier analysis with log-rank tests confirmed significantly higher mortality in the AD deterioration group (p=0.005). Cox regression analysis revealed that AD deterioration was a significant independent risk factor for mortality (HR 6.029, 95% CI 1.400–25.970).Moreover, in all cases with repeated CT scans (353 cases), Cox regression analysis also revealed that AD deterioration was an independent risk factor for mortality (HR 3.596, 95% CI 1.166–11.097).Figure 1.Kaplan-Meier analysis with log-rank tests. It revealed significantly higher mortality in the AD deterioration group (p=0.005).Conclusion:In RA patients, the deterioration of AD is an independent risk factor for mortality. Consequently, longitudinal pulmonary radiographic examinations are essential for those with RA complicated by AD.REFERENCES:[1] Radiology 2004;232:81–91.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:IgG4-related hypophysitis (IgG4-RH) represents central nervous system involvement in IgG4-related diseases (IgG4-RD). However, its diagnosis is challenging in many patients, primarily due to the difficulty in obtaining biopsies. There are few case reports of IgG4-RH, and much remains unknown about its pathology.Objectives:To discuss the clinical characteristics of 17 patients diagnosed with IgG4-RH among five institutions including our hospital.Methods:Using EZR statistical software, we analyzed the clinical characteristics of 17 IgG4-RH patients diagnosed at Kanazawa Medical University Hospital, Kanazawa University Hospital, Toyama University Hospital, Sapporo Medical University Hospital, and Nagaoka Red Cross Hospital.Results:Among 877 patients diagnosed with IgG4-RD at these centers, 17 (1.9%) were diagnosed with IgG4-RH. Of these ten (58.8%, median age 67 yrs.) were men. Pituitary lesions were present in ten patients (58.8%) upon IgG4-RD diagnosis. Diabetes insipidus was present in nine (52.9%), fatigue in six (35.3%) headache in two (11.8%), and ocular symptoms in two patients (11.8%). The median number of affected organs in IgG4-RD was four, with the salivary glands and lymph nodes (11 patients, 64.7%), and lungs (nine patients, 52.9%) being the most commonly involved. Blood tests revealed decreased ACTH, TSH, LH, or GH in eight patients (50.0%) and a decrease in ≧2 hormones in two (12.5%). ADH was decreased in all 14 measured patients. Imaging studies showed pituitary enlargement in 13 patients (81.3%). Pituitary biopsy was performed in only three patients (18.8%). A total of 12 patients (70.6%) met the comprehensive diagnostic criteria for IgG4-RD 2011, and 13 (76.5%) had an ACR/EULAR classification criteria score of ≧20. Prednisolone (PSL) treatment or increased dosage was initiated in 13 patients, with one starting hydrocortisone. The median initial PSL dose was 30 mg/day. Desmopressin treatment for diabetes insipidus was provided to six patients. Among the 16 patients receiving treatment, 14 (87.5%) showed symptom improvement. No relapses occurred during the observation period, and no patients died.Conclusion:IgG4-RH is a relatively rare lesion among IgG4-RD, and symptoms due to hormonal abnormalities are nonspecific and may be overlooked. All patients exhibited decreased ADH levels, suggesting that measuring hormone levels in IgG4-RD patients may prevent potential pituitary lesions from being overlooked. Maintenance therapy with glucocorticoids was administered to most patients, contributing to a low relapse rate.REFERENCES:NIL.Table 1.Figure 1.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:The long-term prognosis of IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis (IgG4-PA/RPF) has not been clarified.Objectives:To elucidate the prognosis of IgG4-PA/RPF, specifically focused on renal dysfunction due to hydronephrosis, and aneurysm formation that may lead to rupture, surgical intervention, or dilatation.Methods:A retrospective study was conducted on 61 patients diagnosed with IgG4-PA/RPF at our hospital between January 1, 2006 and January 1, 2023. All cases were diagnosed by experts, based on serological and imaging findings, clinical course, and exclusion of other diseases. The change in estimated glomerular filtration rate (eGFR) from the best eGFR within 3 months of treatment intervention to that at 36 months was defined as the renal outcome. Rupture, surgical intervention, or ≥5mm aneurysm dilatation caused by IgG4-related PA/RPF were defined as aneurysmal outcomes.Results:Among the patients, 53 (86.8%) were men, and the average age at onset was 76.3 ± 9.65 years. The median observation period was 4.93 years(interquartile range [IQR]: 3.35–9.72). The abdominal aorta and the iliac arteries were affected in 48 patients (78.6%), which were the most common sites of involvement. Fifty cases (81.9%) were treated with prednisolone (PSL) monotherapy at a median dose of 25.0 mg/day (IQR: 20.0–30.0), and the remaining cases were observed without intervention. No deaths were recorded during the observation period. Fifteen cases (24.5%) showed hydronephrosis, with bilateral involvement in two. In six cases (40.0%), ureteral stents were placed at a median 13 days (IQR: 1.75–35.5) after identification. All stents were removable, and the median duration until removal was 232 days (IQR: 174–316). The median change in eGFR at 36 months was -5.38 ml/min/1.73m2 (IQR: -15.85–5.08) and the median rate of change was -10.5 % (IQR: -24.1–8.18). In cases without hydronephrosis, the changes in eGFR were -10.84 ml/min/1.73m2 (IQR: -20.25–[-3.54]) and -12.67% (-24.4–[-6.21]) respectively, and there was no significant difference compared to cases with hydronephrosis. One patient with hydronephrosis progressed to end-stage kidney disease, which was complicated by the IgG4-related kidney disease. Seventeen aneurysms were detected in 10 cases (16.3%), with multiple formations observed in five cases. Twelve aneurysms (70.5%) were already formed at the time of IgG4-PA/RPF diagnosis, while two new aneurysms appeared more than 5 years after IgG4-PA/RPF diagnosis. Aneurysmal outcomes were observed in seven cases, including one case of aneurysm rupture, two cases of surgical intervention, and four cases of aneurysm dilatation. The incidence rate in all cases was 2.14 per 100 patients-years (/100PY), while it was 19.0/100PY among cases with aneurysms. These outcomes were observed after a median of 53.0 months (IQR: 26.6–76.6). The presence of an aneurysm at the time of diagnosis was the risk factor of these outcomes (HR=39.1; 95% CI: 4.51–338.6), whereas the administration or dosage of PSL was not.Conclusion:Patients with IgG4-PA/RPF may have a relatively high risk of aneurysm rupture, surgical intervention, and dilatation, and these events may occur more than 5 years after the onset. Patients with aneurysm formation at the time of diagnosis require particular attention. The impact on renal function due to hydronephrosis may be manageable with appropriate interventions among these patients.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Diagnosing IgG4-related periaortitis/retroperitoneal fibrosis (IgG4-PA/RPF) without biopsy of PA/RPF lesions is often challenging owing to various secondary causes of PA/RPF.Objectives:This study was aimed at exploring clinical findings other than those of PA/RPF biopsy that may be useful for differentiating IgG4-PA/RPF from mimickers.Methods:In this multicenter cross-sectional study, we analyzed 75 Japanese patients diagnosed with IgG4-related disease (IgG4-RD) having cardiovascular and/or retroperitoneal manifestations, along with 20 mimickers identified by experts. We performed an intergroup comparison of clinical characteristics other than biopsy findings of PA/RPF lesions between IgG4-RD and mimickers. In addition, factors related to the final diagnosis of IgG4-RD by experts were assessed by age-, sex-, and serum IgG4 level-adjusted logistic regression analyses.Results:The final diagnoses of mimickers mainly consisted of Takayasu arteritis, giant cell arteritis, infectious aortic aneurysm, lymphoma, plasmacytoma, and urinary tract carcinoma. Compared with mimickers, IgG4-PA/RPF was associated with higher levels of serum IgG4 and IgE; higher eosinophil counts; lower levels of serum C3, C4, CH50, and C-reactive protein; male predominance; allergic predisposition; fewer cases with physical pain and/or fever; fewer instances of thoracic aorta involvement; more iliac artery involvement; prevalence of major extra-PA/RPF lesions of IgG4-RD; and higher inclusion scores of the American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria [1]. Age-, sex-, and serum IgG4 level-adjusted logistic regression analysis indicated that iliac artery involvement [odds ratio (OR) 8.701, 95% confidence interval (CI) 1.006-75.243], presence pf extra-PA/RPF lesions (OR 9.097, 95% CI 1.055-78.419), and inclusion scores of the ACR/EULAR classification criteria for IgG4-RD (OR 1.155, 95% CI 1.025-1.301) were positively related to a final diagnosis of IgG4-RD. However, two cases of follicular lymphoma with periaortic lesions, paravertebral lesions in the thorax, renal pelvic lesions, and/or focal pancreatic lesions could be differentiated only based on biopsy.Conclusion:The present study suggests that in the absence of PA/RPF biopsy findings, iliac artery involvement, presence pf extra-PA/RPF lesions, and high inclusion scores of the ACR/EULAR classification criteria for IgG4-RD are useful for differentiating IgG4-PA/RPF from mimickers. Nevertheless, biopsy is still necessary to differentiate some cases of low-grade lymphoma from IgG4-RD.REFERENCES:[1] Wallace ZS, et al. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis. 2020;79:77-87.Acknowledgements:We sincerely thank all the members of Department of Nephrology and Rheumatology, Kanazawa University Hospital.Disclosure of Interests:None declared.

Background:Diagnosing IgG4-related periaortitis/retroperitoneal fibrosis (IgG4-PA/RPF) is often challenging, especially in PA/RPF-limited cases, due to the difficulty in obtaining periaortic/retroperitoneal specimens. In 2018, diagnostic criteria were introduced for IgG4-PA/RPF in Japan [1], whereas the American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for IgG4-related disease (IgG4-RD) were established in 2019 [2].Objectives:This study aimed to validate the existing Japanese organ-specific diagnostic criteria as well as the ACR/EULAR classification criteria in a Japanese cohort of patients with IgG4-PA/RPF and formulate an improved version.Methods:In this nationwide multicenter study, we retrospectively analyzed 110 Japanese patients diagnosed with IgG4-RD involving cardiovascular and/or retroperitoneal manifestations, along with 73 mimickers identified by experts. Patients were stratified into derivation (n=88) and validation (n=95) groups. Classification as IgG4-RD or non-IgG4-RD was based on the 2018 diagnostic criteria and various revised versions in addition to the ACR/EULAR classification criteria. Sensitivity and specificity were calculated using experts’ diagnosis as the gold standard for the diagnosis of true IgG4-RD and mimickers.Results:The final diagnoses of mimickers mainly consisted of non-IgG4-related idiopathic PA/RPF, lymphoma, Takayasu arteritis, giant cell arteritis, and atherosclerotic or infectious aortic aneurysm. The ACR/EULAR classification criteria had a sensitivity of 52% and a specificity of 95%. In the derivation group, the 2018 Japanese diagnostic criteria showed 59% sensitivity and 100% specificity. The revised version, incorporating “radiologic findings of pericarditis,” “eosinophilic infiltration or lymphoid follicles,” and “probable diagnosis of extra-PA/RPF lesions,” improved sensitivity to 70% while maintaining 100% specificity. In the validation group, the original and revised diagnostic criteria had sensitivities of 68% and 77%, and specificities of 97% and 95%, respectively. When definite, probable, and possible diagnoses were regarded as a diagnosis of IgG4-PA/RPF, the diagnostic criteria showed 100% sensitivity and 92% specificity.Conclusion:The present study suggests that the 2019 ACR/EULAR classification criteria for IgG4-RD and the 2018 Japanese diagnostic criteria for IgG4-PA/RPF have excellent diagnostic specificities but relatively low sensitivities. Proposed 2023 revised IgG4-related cardiovascular/retroperitoneal disease criteria show significantly enhanced sensitivity while preserving high specificity, achieved through the inclusion of new items in radiologic, pathological, and extra-cardiovascular/retroperitoneal organ categories.REFERENCES:[1] Mizushima I, et al. Clinical and pathological characteristics of IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis diagnosed based on experts’ diagnosis. Ann Vasc Dis. 2019;12:460-72.[2] Wallace ZS, et al. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis. 2020;79:77-87.Acknowledgements:We sincerely thank all the members of IgG4-related cardiovascular disease working group.Disclosure of Interests:None declared.

BackgroundDiagnosing IgG4-related periaortitis/retroperitoneal fibrosis (IgG4-PA/RPF) is often challenging, especially in PA/RPF-limited cases, due to the difficulty in obtaining periaortic/retroperitoneal specimens. In addition to the Japanese diagnostic criteria for IgG4-PA/RPF [1], the 2019 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for IgG4-related disease (IgG4-RD) [2] have not been validated for IgG4-PA/RPF diagnosis in daily clinical practice.ObjectivesThis study aimed to validate the ACR/EULAR classification criteria and the Japanese organ-specific diagnostic criteria in a Japanese cohort of patients with IgG4-related PA/RPF.MethodsIn this nationwide multicenter study, we retrospectively reviewed the medical records of 110 patients with IgG4-PA/RPF and 79 non-IgG4-RD patients (mimickers) diagnosed by experts. Using the collected data, we calculated sensitivity and specificity in both the ACR/EULAR classification criteria and the Japanese diagnostic criteria. To clarify the characteristics of false-negative cases of IgG4-PA/RPF, we compared the clinical features between the false-negative and true-positive cases.ResultsPatients with IgG4-PA/RPF and mimickers had a median age of 70 and 71 years, and were 90% and 68% male, respectively. The final diagnoses of mimickers mainly consisted of non-IgG4-related idiopathic PA/RPF, lymphoma, Takayasu arteritis, giant cell arteritis, and atherosclerotic or infectious aortic aneurysm. The ACR/EULAR classification criteria had a sensitivity of 52% and a specificity of 95%. Of the 53 false-negative cases, 13 did not fulfill the entry criteria, 13 fulfilled one exclusion criterion, and 36 did not achieve sufficient inclusion criteria scores. Compared with the true-positive cases, the false-negative cases had significantly lower serum IgG4, IgG, and IgE levels, higher serum C3 and C4 levels, and lower prevalence of extra-vascular/retroperitoneal lesions; frequency of periaortic/retroperitoneal biopsies (36% vs 37%), frequency of storiform fibrosis and obliterative phlebitis in the obtained specimens, and numbers of infiltrating IgG4-positive cells were similar between the false-negative and true-positive cases. The Japanese diagnostic criteria for IgG4-PA/RPF had a sensitivity of 63% and a specificity of 99% when definite and probable diagnoses in the diagnostic criteria were regarded as a diagnosis of IgG4-PA/RPF (a strict version). When definite, probable, and possible diagnoses were regarded as a diagnosis of IgG4-PA/RPF (a lenient version), the diagnostic criteria had a sensitivity of 95% and a specificity of 90%.ConclusionThe present study suggests that the 2019 ACR/EULAR classification criteria for IgG4-RD and a strict version of the Japanese diagnostic criteria for IgG4-PA/RPF have an excellent diagnostic specificity in daily clinical practice. A lenient version of the Japanese diagnostic criteria serves as more sensitive criteria, although care should be taken to avoid misdiagnoses for mimickers due to the relatively low specificity.References[1]Mizushima I, et al. Clinical and pathological characteristics of IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis diagnosed based on experts’ diagnosis. Ann Vasc Dis. 2019;12:460-72.[2]Wallace ZS, et al. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis. 2020;79:77-87.AcknowledgementsWe sincerely thank all the members of IgG4-related cardiovascular disease working group.Disclosure of InterestsNone Declared.

In recent years, IgG4-related disease (IgG4-RD) has become a widely recognized disorder. However, mortality and its related factors in this disease are not well known. This study aimed to clarify mortality and its related factors in patients with IgG4-RD. We retrospectively reviewed the medical records of patients with IgG4-RD diagnosed by experts based on fulfillment of the Japanese comprehensive diagnostic criteria and/or the 2019 ACR/EULAR classification criteria for IgG4-RD at a single center in Japan. Using the collected data, we calculated the crude mortality rate and the standardized mortality ratio (SMR) using national Japan mortality statistics and investigated the cause of death. We performed Cox regression analyses to assess mortality-related factors. A total of 179 patients with IgG4-RD were included: 124 were male (69.3%); the median age was 68 years (interquartile range [IQR] 60-75 years); and the median follow-up from diagnosis was 47 months (IQR 17-84). Ten patients (5.6%) in our cohort died during the follow-up period. Five died of malignancy, one of respiratory failure, two of infectious pneumonia, one of sudden cardiac event, and one of suspected aortic aneurysmal rupture. The crude mortality rate was 11.1 per 1,000 person-years. According to national Japan mortality statistics, 11.6 age- and sex-matched deaths were expected to occur within the follow-up period, resulting in a SMR of 0.86 (95% confidence interval [CI] 0.41-1.59). Univariate Cox regression analyses indicated that the number of affected organs at diagnosis (hazard ratio [HR] 1.45, 95% CI 1.02-2.05), serum creatinine levels at diagnosis (HR 1.82, 95% CI 1.06-3.12), and the presence of malignancy during the clinical course (HR 3.93, 95% CI 1.10-14.02) had a significant impact on the time to death, whereas the other factors including age at diagnosis and serum C-reactive protein and IgG4 levels at diagnosis did not. Our findings suggest that the mortality rate of patients with IgG4-RD does not significantly differ from that of the Japanese general population. Multi-organ involvement and renal dysfunction at diagnosis as well as malignancy during the clinical course may be associated with higher mortality. An appropriate clinical evaluation for the early detection of these risk factors is required at first diagnosis and during long-term follow-up. None declared

BackgroundAfter the introduction of biologic therapy, the frequency of rheumatoid vasculitis (RV) has decreased, but its prognosis remains poor [1-3]. No specific biomarker in RV has been established. In laboratory findings, anti-neutrophil cytoplasmic antibody (ANCA) positivity and hypocomplementemia (HC) were reported in 39% and 14%, respectively [2]. Though depressed C4 level had been reported to predict mortality in peripheral neuropathy with necrotizing vasculitis in RA before the biologic era [4], the significance of HC remains unclear in RV in the biologic era.ObjectivesTo evaluate the clinical significance of HC in RV in the biologic era.MethodsWe enrolled 20 Japanese RV patients (12 women; mean age 68.0 years; mean duration of RA 13.6 years, observation period of RV 4.9 years) diagnosed at 4 hospitals from 2000 to 2013. We analyzed retrospectively their clinical findings, treatment and prognosis. All patients met the Scott and Bacon criteria [5]. HC was defined as low levels of serum CH50, C3 or C4 complement; <30 U/ml, <75 mg/dl, <14 mg/dl, respectively.ResultsIn the present 20 patients, cutaneous (60%) and pulmonary lesions (50%) were common. Histopathological confirmation of vasculitis was available for 50%. None had ANCA positivity. HC was found in 65% at RV diagnosis. Serum CH50, C3 and C4 levels on average were 27.9 U/ml, 82.3 mg/dl and 15.7 mg/dl, respectively. Patients with depressed C3 and/or C4 levels were included in those with depressed CH50 level. Patients with HC (n=13) at diagnosis of RV were older at development of RA (54.0 years vs 47.7 years, p<0.01), and had a lower frequency of neuropathy (7.7% vs 57.1%, p=0.03), and higher rheumatoid factor (RF) level (2179 IU/ml vs 209 IU/ml, p<0.01) than those without HC. Treatment for RA before RV was corticosteroid (PSL) (75%), methotrexate (25%), biologics (20%), and others. After development of RV, PSL (95%) and biologics (65%) were most common. After the treatment, HC improved (CH50 40.8 U/ml, C3 85.1 mg/dl, C4 19.5 mg/dl) as CRP did. CH50 (p=0.02) and C4 levels (p=0.05) had significant elevation after the treatment, but C3 level did not (p=0.53). No patients developed recurrence. Severe infection developed in 35% and was fatal in 3.ConclusionsSixty-five percent of Japanese patients with RV showed HC. Compared with RV patients who were diagnosed in almost the same period in the UK (18 patients) and USA (86 patients) reported in 2014 [1,2], a higher frequency of HC and lower positivity of ANCA were shown. Patients with HC at diagnosis of RV had different characteristics such as older age at development of RA, lower frequency of neuropathy, and higher RF level than those without HC. HC, especially CH50 and C4 levels, improved in parallel with amelioration of the activity of RV. These results suggest that HC can be a useful biomarker for the diagnosis and follow up of RV in the biologic era.ReferencesRheumatology 2014;53:145-52.Rheumatology 2014;53:890-9.Curr Opin Rheumatol 2015;27:63-70.Arthritis Rheum 1995;38:1618-29.Am J Med 1984;76:377-84.Disclosure of InterestNone declared

BackgroundScleroderma renal crisis (SRC) adversely affects renal and patient survival in systemic scleroderma (SSc)[.1, 2 The survival rate of SRC has been improved dramatically by angiotensin-converting enzyme inhibitor therapy, but SRC still has a poor prognosis.3 Factors predictive of SRC include early diffuse skin involvement, rapid skin thickening, anti-RNA polymerase (RNAP) III antibodies, arthralgia/synovitis, and high glucocorticoid dosage.4 Although classical data have implicated pericardial effusion as another predictive factor of SRC,5 its role in SRC has not been well established.ObjectivesTo clarify the clinical impact of pericardial effusion as a predictor of SRC.MethodsNinety-five patients diagnosed with SSc at our hospital between January 2003 and December 2017 were enrolled in the study. They were divided into a pericardial effusion group (n=21) and non-pericardial effusion group (n=74), and their clinical features retrospectively compared. Cox-regression analysis was performed to identify factors predictive of SRC.ResultsThe patients comprised 14 men and 81 women with an average age of 57.4 years (range, 14 to 82) and the mean observation period was 65 months (range, 1 to 125). Pericardial effusion was detected in 21 of 95 (22.1%) cases. In the pericardial effusion group, SRC, modified Rodnan’s total skin thickness score (mRSS), C-reactive protein, maximum glucocorticoid dose, proteinuria, finger apex ulcers, and interstitial pneumonia were significantly more prevalent compared to the non-pericardial effusion group. Cox regression analysis indicated that pericardial effusion (hazard ratio; HR 11.8 [95% CI 1.6–84.6], p=0.014) and anti-RNAP III antibodies (HR 11.1 [95% Cl 2.0–59.6], p=0.005) were independent risk factors for SRC, while mRSS (HR 1.0 [95% CI 0.9–1.1], p=0.12), finger apex ulcers (HR 0.57 [95% CI 0.073–4.2], p=0.57), max glucocorticoid dose (HR 1.0 [95% CI 0.9–1.0, p=0.89]), and interstitial pneumonia (HR 0.9 [95%CI 0.2–3.7], p=0.96) were not. In the Kaplan-Meier method, SRC was significantly increased in the pericardial effusion group compared to non-pericardial effusion group (p<0.0001 by log rank test).ConclusionsPericardial effusion is another independent factor predictive of SRC in addition to anti-RNAP III antibodies.References[1] Steen VD, et al. Scleroderma renal crisis. Rheum Dis Clin North Am2003;29:315–33.[2] Hamaguchi Y, et al. Clinical and immunologic predictors of scleroderma renal crisis in Japanese systemic sclerosis patients with anti-RNA polymerase III antibodies. Arthritis Rheumatol2015;67:1045–52.[3] Penn H, et al. Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM2007;100:485–94.[4] Woodworth TG, et al. Scleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Rheumatol2016;12:678–91.[5] Steen VD, et al. Factors predicting development of renal involvement in progressive systemic sclerosis. Am J Med1984;76:779–86.Disclosure of InterestNone declared

BackgroundIgG4-related disease (IgG4-RD) is a recently recognised systemic fibro-inflammatory disorder that can affect many organs.1 In IgG4-RD, spontaneous, or at least temporary, remissions without treatment have been reported, and watchful waiting may be appropriate in certain patients with asymptomatic and inactive disease.2 However, the outcomes of patients with IgG4-RD who do not undergo treatment are still unclear.ObjectivesThis study aimed to clarify the outcomes of untreated patients with IgG4-RD and the factors related to the outcomes.MethodsWe retrospectively reviewed the medical records of 107 patients with IgG4-RD, who were followed up for more than 6 months, at a single centre in Japan. Among them, 27 patients were followed up without treatment after the initial diagnosis. We compared the clinical features of these 27 patients with those of the 80 patients who underwent treatment. The outcomes of untreated patients were investigated, and logistic regression analysis was performed to assess factors related to the outcomes. Deterioration of IgG4-RD was defined as symptomatic, radiological, or functional exacerbation of the organ involved or new organ involvement.ResultsThe patients comprised 73 men and 34 women (mean age 65.7 years). The follow-up periods were 7–252 (mean, 64.1) months, and the serum IgG4 levels at diagnosis were 10.7–3610 (mean, 706) mg/dL. The 27 untreated patients had significantly fewer affected organs (1.9±1.2 vs 3.0±1.6, p=0.001), lower IgG4-RD responder index (10.8±5.1 vs 13.8±6.8, p=0.048), and lower frequency of ophthalmic and renal parenchymal lesions (25.9% vs 53.8%, p=0.015, and 3.7% vs 26.3%, p=0.012, respectively) than did the 80 patients who underwent treatment. Of these 27 patients, 8 experienced deterioration of IgG4-RD 3–232 months (mean, 62.8) after the diagnosis. New organ involvement was observed in all 8 patients, 2 of whom concurrently suffered exacerbation of the organs involved. In age- and sex-adjusted logistic regression analysis, serum IgG4 elevation (per 100 mg/dL, odds ratio 1.194, 95% confidence interval 1.017–1.402, p=0.030) was the only significant factor related to deterioration of disease in untreated patients with IgG4-RD.ConclusionsThe present study suggests that serum IgG4 levels may be useful to predict the outcomes of untreated patients with IgG4-RD, who tend to have fewer affected organs and lower IgG4-RD responder index.References[1] Stone JH, et al. IgG4-related disease. N Engl J Med2012Feb 9;366(6):539–51.[2] Khosroshahi A, et al. International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease.Arthritis Rheumatol2015Jul;67(7):1688–99.Disclosure of InterestNone declared