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Objective: To determine whether tumor necrosis factor (TNF) blockade improves small airway obstruction in rheumatoid arthritis (RA) patients without apparent respiratory symptoms.Methods: Pulmonary function tests were performed before and one year after TNF blockade therapy in 29 RA patients without apparent respiratory symptoms. As a control, pulmonary function was examined at a one-year interval in 27 RA patients with conventional disease-modifying antirheumatic drugs (DMARDs) alone. Small airway obstruction was diagnosed when one of maximal mid-expiratory flow rate (MMEF), forced expiratory flow at 50% (FEF50) or 75% (FEF75) of the vital capacity was decreased to less than 60%, 55%, or 45% of predicted values, respectively.Results: Small airway obstruction was found in 62.5% of RA patients. No differences were found in age, sex, disease duration, or disease activities. TNF inhibitors dramatically reduced disease activities. However, TNF inhibitors failed to improve small airway obstructions but worsened them.Conclusions: TNF blockade fails to improve small airway obstruction, suggesting that TNF-independent pathways play important roles in the development of small airway obstruction in RA.

To clarify the differences and similarities in the cytokine profiles of macrophage activating syndrome (MAS) between systemic lupus erythematosus (SLE) and adult-onset Still's disease (AOSD). The study participants included 9 patients with MAS-SLE, 22 with non-MAS-SLE, 9 with MAS-AOSD, and 13 with non-MAS-AOSD. Serum cytokine levels were measured using a multiplex bead assay. Cytokine levels were compared between patients with SLE and AOSD with/without MAS. Moreover, cytokine patterns were examined using principal component analysis (PCA) and cluster analysis. IL-6, IL-8, IL-18, and TNF-α levels were elevated in patients with SLE and AOSD. IFN-α levels were elevated in SLE, whereas IL-1β and IL-18 levels were elevated in AOSD. In SLE, IFN-α and IL-10 levels were higher in MAS than in non-MAS and controls. PCA revealed distinctive cytokine patterns in SLE and AOSD, SLE with IFN-α and IP-10, AOSD with IL-1β, IL-6, and IL-18, and enhanced cytokine production in MAS. PCA and cluster analysis showed no differences in cytokine patterns between the MAS and non-MAS groups. However, serum ferritin levels were correlated with IFN-α levels in SLE. Cytokine profiles differed between SLE and AOSD but not between MAS and non-MAS. MAS is induced by the enhancement of underlying cytokine abnormalities rather than by MAS-specific cytokine profiles. Type I IFN may be involved in MAS development in patients with SLE.

Urinary exosomes, one of several types of extracellular vesicles, contain specific molecules involved in both renal function and disease. Therefore, in the field of nephrology, exosome research is expected to shed new light on physiological functions and lead to discovery of new diagnostic and therapeutic methods. In this chapter, we outline the application of molecules contained in urinary exosomes as biomarkers of renal disease.

Background Chemokines are involved not only in regulating leucocyte recruitment, but also in other activities. However, functions other than cell recruitment remain poorly understood. We have already shown that the production of CC chemokine ligand (CCL)17 and CCL22 by antigen-stimulated naive CD4+ T cells was higher in asthmatic patients than in healthy controls. However, the role of these chemokines in stimulated naive CD4+ T cells remains unclear. Objective To clarify the biological function of CCL17 and CCL22 on naive CD4+ T, we examined effects of these two chemokines on naive CD4+ T cells expressing CC chemokine receptor (CCR)4 (a receptor for CCL17 and CCL22) during differentiation of Th2 cells in asthmatic patients as allergic subjects. Methods Naive CD4+ T cells were prepared from healthy controls and patients with asthma. We analysed effect of CCL17 and CCL22, and blocking their receptor on differentiation of Th2 cells. Results Production of CCL17 and CCL22 by activated naive CD4+ T cells under Th2 condition was much more in asthmatic patients than in healthy controls. Proliferation and survival of the Th2 differentiating cells and restimulation-induced IL-4 production were much greater in asthmatic patients than in healthy controls. These cell biological phenomena were inhibited by blockade of CCR4. The biological effects of exogenous CCL17 and CCL22 were apparently observed in both healthy controls and asthmatic patients. The effectiveness of these chemokines on naive CD4+ T cells from healthy controls was stronger than those from asthmatic patients. We found that thymic stromal lymphopoietin (TSLP), a Th2 promoting chemokine, is involved in the activation of CD4+ naive T cells via production of CCL17 and CCL22. Conclusions and clinical relevance These data suggest that CCL17 and CCL22 produced by TSLP-primed naive CD4+ T cells in asthma might contribute to an increase in Th2 cells via autocrine loops.

Objective We aimed to determine the outcome of combination therapy with tofacitinib (TOF) in a case series of refractory rapidly progressive interstitial lung disease (ILD) associated with anti-melanoma differentiation-associated 5 gene (MDA5) antibody-positive (Ab+) DM. Patients who had poor prognostic factors and failed to respond to immunosuppressive therapy were selected for TOF treatment. Methods Five patients with anti-MDA5 Ab+ DM-ILD who failed to respond to triple therapy with high dose glucocorticoids, CSA and CYC were given additional TOF (10 mg/day). To identify the poor prognostic factors, data from 15 consecutive patients (seven survived and eight died) with anti-MDA5 Ab+ DM-ILD before induction of TOF were analysed. Results Three poor prognostic factors were identified: serum ferritin level >1000 ng/ml before therapy; ground-glass opacities in all six lung fields before therapy; and worsening of pulmonary infiltrates during therapy. All six patients who had all of the three factors and received triple therapy died before TOF therapy. There were five patients who had all of the three prognostic factors and failed to respond to triple therapy, but were able to receive the combination therapy with TOF; among them, three survived and two died. The survival rate of patients who received TOF was significantly better than that of the historical controls with immunosuppressive therapy before TOF. The patients who received TOF experienced complicated adverse events, particularly viral infection. Conclusion Combination therapy with TOF might have the potential to control refractory anti-MDA5 Ab+ DM-ILD.

Background: By autoantibodies, myositis is recently classified into subgroups with distinctive clinical features. Cytokines play critical roles in the development of the clinical features. However, it is not fully unknown whether myositis subgroups defined by autoantibodies have unique cytokine profiles. Objectives: To clarify the relationship between cytokine profiles and autoantibodies in myositis. Methods: Subjects were myositis patients who admitted Dokkyo Medical University and whose serum before starting therapy were available. Serum cytokine levels (IL-1a, 1b, 2, 4,5, 6, 10,12, 13,15,17, 23,IFN-g, TNF-a, IFN-a, b,IP-10 and MCP-1) in sera from the patients and controls were measured using Q-Plex™ Multiplex Arrays. Demographic and clinical data were obtained by reviewing medical record retrospectively. Results: Subjects were 45 myositis patients with male/female; 22/23 and 59.4 years of mean age. Amyopathic dermatomyositis (ADM), classical DM and polymyositis (PM) were 21, 11 and12 cases, respectively. Autoantibodies were anti-MDA5+ in 21, anti-ARS+ in18 and anti-TIF1g+ in 5 cases. ILD and malignancy was complicated in 39 and 5 cases, respectively. Serum cytokine levels were measured. As shown in Fig.1, IP-10 levels were increased in all types of myositis patients, but not in controls. In ant-MDA5 Ab + myositis, IL-6, IL-10, IL-15, TNF-a, IFN-a and MCP-1 levels were increased. In anti-ARS Ab+ cases, IFN-b levels were elevated as well as TNF-a and MCP-1. Through the correlation analysis between cytokine levels, cytokine groups were identified (Fig.2). In anti-MDA5 Ab+ cases, 2 cytokine groups were identified; one includes IFN-a, IL-15, TNF-a and IL-10, and the other involved IL-6, MCP-1. Both groups connected with IP-10. In In anti-ARS Ab+ cases, 2 groups were found; one included TNF-a, MCP-1and IP-10, and the other had IFN-b as a sole component. Conclusion: Cytokine profiles differ among subgroups in myositis. In anti-DDA5+ myositis, IL-15 and IFN-a with strong correlation and IL-6 were characteristic cytokines, while IFN-b was a unique one in anti-ARS+ myositis Disclosure of Interests: None declared

Endogenous lipoid pneumonia is an uncommon inflammatory pulmonary disease that is caused by lipids from an endogenous source, the treatment for which has not been established. We report the first case of endogenous lipoid pneumonia presenting as lung consolidation and which was associated with rheumatoid arthritis. Treatment was successful with intensive immunosuppressive therapy. When a physician finds lung consolidation in a patient with active rheumatic disease, lipoid pneumonia should be considered.

To clarify the clinical features of organizing pneumonia (OP) associated with rheumatoid arthritis (RA) and to determine whether development of OP is related to RA activity.A cross-sectional study was conducted, in which medical records of 499 consecutive RA patients who visited our hospital during one month were reviewed. OP was diagnosed by pathological findings by trans-bronchial biopsy or by clinical features (typical computed tomography findings, no causative agents, good response to glucocorticoids, and lack of response to antibiotics).Among 499 patients, OP was found in 19 patients and the estimated prevalence was 1.9-4.8%. No differences in clinical features were noted between the OP and non-OP groups. The mean age of OP development was 57.2 years and the period from the onset of RA to OP ranged from -4 to +34 years. Although 14 patients presented OP after the onset of RA, two developed OP before RA and three developed OP simultaneously with RA. Patients receiving tumor necrosis factor inhibitors also developed OP. RA disease activity just before onset of OP was low in 8 of 14 RA cases. At the onset of OP, only two patients showed exacerbations of arthritis, whereas most patients presented with fever and serum C-reactive protein (CRP) elevations. Glucocorticoids were effective for OP in all patients who received them. Relapse occurred in 4 of 19 cases.OP develops in approximately 4% of RA patients, which occurs independently from arthritis activity and at any time in RA patients.

Background:Interstitial lung disease (ILD), frequent lung involvement, determine the prognosis of patients with RA. The presence of ILD also influences the selection of RA therapy. However, it is not fully elucidated what groups of RA-ILD patients worsen ILD.Objectives:To identify the risk factors for worsening of ILD in RA patients under biological DMARD (bDAMARDs) therapy; particularly to determine whether types of bDMARDs are associated with exacerbation of ILD.Methods:A retrospective cohort study was conducted. Subjects were consecutive 91 RA-ILD patients who received HR-CT examination at starting and during bDMARDs therapy. Clinical data were collected by reviewing.ILD was diagnosed, when patients showed ground-glass opacity (GGO), consolidation, reticular pattern or honeycomb; the former two were acute and the latter two were chronic ILD lesions. The extent of each lesion was scored and recorded. When the score was increased, the lesion was judged as worsened. The incidence of exacerbation of ILD under an indicated agent was calculated as follows; the sum of (exposure period of the agent/exposure period of any agent) in patients with the exacerbation was divided by total exposure period of the agent.Results:Subjects were 36 males and 55 females with a mean age of 66.8 years. At the entry, GGO, consolidation, reticular and honeycomb were found in16, 20, 63 and 17, respectively. Acute and chronic ILD were observed in 34 and 68, respectively. ILD worsened in 35 (38.5%). Between patients with and without ILD exacerbation, no differences were found in demographics (sex, age, disease duration, the positivity for anti-CCP ab and RF) and radiographic findings of pulmonary abnormalities. Disease activities at as 1st and sequential CT examinations were similar in both groups.The incidences of the worsening of ILD varied according to bDMARDs (Fig 1). The incidence of total ILD worsening was high in TCZ compared to TNF and ABT. The incidence of acute ILD worsening was similar among the three groups. However, the worsening of chronic ILD was more frequently found in TCZ than other bDMARDs. The incidence of death by respiratory failure was similar in TNF, ABT, and TCZ (Fig. 2).Fig. 1Conclusion:TCZ might be a risk factor for exacerbation of ILD, particularly of chronic ILD.Fig. 2Disclosure of Interests:None declared

Background Rheumatoid arthritis (RA) is a systemic inflammatory disease that affects joints and various organs. The lung is one of the most common site of extra-articular involvement. Interstitial lung disease (ILD) is one form of the lung involvement and influences prognosis of the patients. RA-ILD frequently developed acute exacerbation. Once acute exacerbation occurs, the prognosis of patients is poor. However, it is not fully elucidated what the risk factors for severe exacerbation of ILD are. Objectives The purpose of thisstudy was to identify the risk factors for severe exacerbation of ILD in RApatients during bDMARDs therapy.The purpose of this study was to identify therisk factors for exacerbation of ILD in RA patients under the bDMARDs therapy. Methods A retrospective cohort study. Subjects were consecutive 208 RA patients who were treated with bDMARDs from 2004 to 2015 in our department and received HRCT scan before and during the therapy. Based on HR-CT imaging, pulmonary abnormalities were classified into 4 categories (ILD, nodular lesions, airway disease (AD) and others) and 20 lesions such as ground-glass opacity (GGO), reticular pattern, bronchiolitis and bronchiectasis. We recorded their existence and distribution before andduring the therapy and examined their changes. Severe exacerbation of ILD was judged when patients revealed newly emerging/worsening of ILD lesions regardless of pre-existing pulmonary lesions and deceased or had to stop bDMARDs due to exacerbation of ILD. To identify risk factors, logistic regression analysis was conducted. Results Subjects were 208 RApatients, M/F; 64/144, mean age; 59.2 years old, disease duration; 7.9 years positive for RF in 84.1%. bDMARDs used for the longest period were TNFinhibitors in 79.8% of the subjects, abatacept in 15.4% and tocilizmab in 4.8%. Pulmonary abnormalities were found in 146 (70.2%) of RA patients before bDMARDs (ILD 81,(38.9%); nodular lesions 45, (21.6%); and ADs, 115, (55.3%)). GGO, consolidation, reticular pattern and honey combing was found in 14.8%, 19.8%, 51.9%, and 17.3% of ILD patients. Most of ILD lesions coexisted with ADs. During the observation period (3.26±2.61 years), newly emerging/worsening pulmonary lesions were found in 42.3% of patients and the incidence of which was 13.7/100person year. The incidence of newly development and worsening of ILD were 7.0/100 person years and 1.5/100 person years, respectively. Severe exacerbation of ILD was developed in 13 patients (6.3%) and the incidence of which was 2.0/100 person year. Of 13 patients, 9 died of respiratory failure. All patients with severe exacerbation revealed GGO/consolidation.Interestingly, all these active ILD lesions were newly emerging lesions, however, most of the patients had pre-existing pulmonary lesions. We identified risk factors for severe exacerbation of ILD (Table1). The risk factors for severe exacerbation of ILD were older age and pre-existing pulmonary lesions of ILD and of ADs, particularly reticular pattern, honeycomb and bronchiectasis. Sero-positivity and choice of biologics were not identified as risk factors. Conclusions The incidence of severe exacerbation of ILD was 2.0/100 person years. The risk factors forsevere exacerbation of ILD were older age and pre-existing reticular pattern,honeycomb and bronchiectasis. Disclosure of Interest: None declared

Chemokines are involved not only in regulating leucocyte recruitment, but also in other activities. However, functions other than cell recruitment remain poorly understood. We have already shown that the production of CC chemokine ligand (CCL)17 and CCL22 by antigen-stimulated naïve CD4To clarify the biological function of CCL17 and CCL22 on naïve CD4Naïve CD4Production of CCL17 and CCL22 by activated naive CD4These data suggest that CCL17 and CCL22 produced by TSLP-primed naïve CD4

Background Rheumatoid arthritis (RA) is a systemic inflammatory disease that affects joints and various organs including the lung. The pulmonary involvement is critical for prognosis of the patients and decision of the treatment. Moreover, the pulmonary involvement showed various abnormalities such as interstitial pneumonia (ILD) and airway disease (AD). Importantly, a pulmonary abnormality coexists with other ones in RA patients. There have been large numbers of studies on the prevalence of pulmonary abnormalities and clinical features of patients with these lesions. However, it remains to be elucidated what existence pattern of pulmonary abnormalities RA patients have. Objectives To reveal the existence pattern of the pulmonary abnormalities in RA patients using cluster analysis, and to clarify the clinical features of patients with multiple pulmonary abnormalities. Methods Subjects were consecutive 208 RA patients who were treated with bDMARDs as the first one from Feb. 2004 to Sep. 2015 in our department and received HRCT scan before and after the therapy. Pulmonary abnormalities were classified into 4 categories (ILD, nodular lesions, AD and other) and 20 lesions such as ground-glass opacity, reticular pattern, bronchiolitis and bronchiectasis and were examined their existence and distribution. Cluster analysis was conducted according to the existence of the lesions by Ward method. Clinical features were analyzed through reviewing medical records. Results Subjects were 208 RA cases (M/F; 64/144, mean age 59.2 year-old, disease duration 13.1 years). Pulmonary lesions were found in 146 (70.2%) of RA patients before treatment Imaging findings were 81of ILD (39%),45 of nodular lesion (21.6%) and 115 of AD (55%). Cluster analysis showed 6 clustered (Fig.), 1; no pulmonary lesions, 2; AD without bronchoectasia, 3; AD with bronchoectasia, 4; AD with curved linear opacities, 5; AD with nodular lesions, and 6; reticular pattern with AD. AD was common abnormalities and coexisted with other pulmonary lesions in RA. AD was found in 79%, 78% and 71% of patients with pulmonary abnormalities, ILD and nodular lesions, respectively. AD alone, AD with ILD, and AD with nodular pattern were found in 16.3%, 8.6% and 28.9%, respectively, while patients without pulmonary lesions were 29.8% in RA. AD was frequently associated with ILD and nodule compare to non-AD. No differences were found in gender, smoking history, disease duration and disease activity between patients with and without AD. New emergence or exacerbation of pulmonary abnormalities developed in AD patients compared to those without pulmonary abnormalities or AD. No significant differences were found in clinical features, among AD alone, AD with ILD and AD with nodules. Conclusions Pulmonary abnormalities were found in 70% in RA. AD was found in 55% of RA patients and coexisted with other pulmonary lesions such as ILD and nodular lesions. Patients with AD frequently showed newly emerging or worsening pulmonary lesions, regardless of the coexistence of other pulmonary lesions. Thus, AD is shared and critical pulmonary abnormality in RA. Disclosure of Interest None declared

79 歳男性.発熱,咳嗽で発症し,鼻閉の既往,空洞を伴う両下肺の浸潤影,顕微鏡的血尿,急速進行する腎機能障害およびPR3-ANCA 高値を示し,Wegener 肉芽腫症( WG) と診断された.一時シクロフォスファミド併用を含む,高用量副腎ステロイド療法が開始され,臨床および検査所見は改善した.ステロイド薬は漸減され,3 ヶ月後にはC-ANCA は陰性化した.WG は寛解していたが,8 ヶ月後に胸部下行大動脈瘤形成を認め,その1 ヶ月後に突然大喀血し,死亡した.動脈瘤形成は疾患活動性とは関連なく,経過中注意深い観察の継続を要する., A 79-year-old man was admitted because of fever andcough. He had been diagnosed with Wegener's granulomatosis(WG) on the basis of 10-year history of stuffy nose,pulmonary infiltrates with cavity formation, rapidly progressedrenal dysfunction with microscopic hematuria, andhigh level of PR3-ANCA. He was treated with high dose ofglucocorticoid( GC) temporarily in combination with cyclophosphamide,accordingly, the clinical manifestation and thelaboratory results had been recovering. The dose of GC wastapered down, and 3 months after the initiation of therapy,C-ANCA testing was negative. Despite remission was obtained,8 months after, chest computed tomography (CT)scan showed aneurysm formation on the descending aorta.One month after that, the patient had an abrupt onset ofcoughing up a massive amount of blood, and died. Aneurysmformation may develop independently of disease activityin WG and may be unpredictable, so that a carefulobservation whether aneurysm is formed should be continuouslyconsidered in the follow-up during the entire course.

Background Granulocyte-macrophage colony-stimulating factor (GM-CSF) is an important hematopoietic growth factor, particularly for differentiation of alveolar macrophage, and immune modulator including inflammation. Neutralizing autoantibodies against GM-CSF antibodies cause pulmonary alveolar proteinosis (PAP). We have previously reported a SLE patient with anti-GM-CSF antibody (Ab) and PAP (Lupus 2013;22, 1060). However, it remains unknown whether there exist autoantibodies against GM-CSF in connective tissue disease (CTD) patients without PAP. Objectives To determine whether anti-GMF autoantibody is detected in sera from CTD patients. If so, to determine whether the antibody has neutralizing activity and to clarify the clinical features of patients with the antibody. Methods Subjects were 245 CTD patients (SLE; 63, DM/PM; 49, RA; 106, vasculitis; 27) and 34 healthy controls. A EIA system to detect anti-GM-CSF Abs was developed; GM-CSF was coated on the plate that was then blocked with BSA, diluted serum with PBS was added, and after washing the Ab was detected by anti-IgG Ab. To avoid non-specific binding, the optic density (OD) of the Ab was calculated as follows; OD (GM-CSF coated well) -OD (GM-CSF negative well). The neutralizing activity of the Ab was examined through inhibition of CD11b expression on neutrophils by GM-CSF; neutrophils were incubated in media with GM-CSF (5ng/ml) and serum from the subjects, and CD11b expression was examined using flowcytometry. Association of clinical features with the Ab was assessed by reviewing medical records. Results Anti-GM-CSF Ab was detected in 60% of CTD (frequencies of positivity: SLE; 70%, DM/PM; 57%, RA; 57%, vasculitis 55%) (Fig.1). PAP was not found in all cases except one SLE that has been reported previously. Neutralizing activity was not detected except the case of SLE with PAP. Pulmonary involvements including interstitial lung disease, bronchial disease and pleuritis was frequently found in RA patients positive for the Abs compared to negative patients (positive 47%, vs negative 18%; p Conclusions Non-neutralising Autoantibody against GM-CSF was frequently detected in CTD patients, particularly those with pulmonary involvements. These findings suggest that the autoantibody, which might work as carrier protein of GM-CSF, plays roles in the development of CTDs, especially pulmonary involvements in RA and SLE. Disclosure of Interest None declared

Background IgG4 related disease (IgG4RD) is a multi-organ affecting disease characterized by fibro-sclerosing lesions with IgG4+ cell infiltration. Serum IgG4 elevation is a critical clue for diagnosis of IgG4RD. However, there are patients of non-IgG4RDs whose IgG4 levels are elevated. Clinical features of these patients have not been fully elucidated. Objectives To clarify clinical features of patients with serumIgG4 elevation, particularly, to determine whether IgG4 RD is a distinctive disease in patients with IgG4 elevation and whether there is a subset of non-IgG4RDs which have strong similarity to IgG4RD. Methods For determination of the prevalence of patients with serum IgG4 elevation, consecutive 185 patients who admitted our department during 2 months were enrolled. For clarification of clinical features of patients with IgG4 elevation (IgG4>135mg/dl), 68 cases with IgG4 elevation were selected from consecutive 350 patients receiving IgG4 test in clinical practice at our hospital and their medical records were reviewed retrospectively. IgG4RD was diagnosed by comprehensive diagnostic criteria for IgG4-related disease (Mod Rheumatol.22:21-30; 2012). Cluster analysis according to organ involvements was performed through Ward9s method. Results Prevalence of serum IgG4 elevation in general inpatients was 9/1850 (4.9%). Only 1 of 9 was IgG4RD. Among 68 cases with IgG4 elevation who received IgG4 test in clinical practice, IgG4RD was 41%. Cluster analysis according to organ involvements revealed 5 clusters; cluster1: multiple sclerosing lesions with salivary, orbital and retroperitoneal lesions, 2: sclerosing lesions with autoimmune pancreatitis, 3: pulmonary inflammation, 4 varieties of inflammatory diseases including connective tissue diseases, and 5: pleuritis. Cluster 1 and 2 were divided from other ones at 1st node. IgG4RD existed only cluster 1 and 2. In addition to sclerosing lesions, non-sclerosing lesions were found in 13% of cluster 1, 43% of cluster 2, 33% of IgG4RD and 70% of non-IgG4RDs. Non-sclerosing lesions were developed as organ limited inflammation in most cases of IgG4RD and non-IgG4RDs, which was contrast to sclerosing lesions in IgG4RD affecting multi-organs. Among non-sclerosing lesions, IgG4+ cells were observed. Pathological examination of non-sclerosing lesions revealed IgG4+ cell inflammation in 4/5 in IgG4RD and 8/21 in I non-IgG4RDs. The lesions in non-IgG4RDs were indistinguishable from those in IgG4RD. Patients of non-IgG4RDs with IgG4+ cells had similar clinical features to IgG4RD including high IgG4 levels, low CRP levels and good response to glucocorticoid in addition to pathological findings. Conclusions IgG4RD is a minor, but a distinctive disease in patients with serum IgG4elevation, characterized by sclerosing lesions affecting multiple organs. IgG4RD causes non-sclerosing lesions with IgG4+ cells as well as sclerosing ones. Among non-IgG4RDs, there are patients with IgG4 + non-sclerosing lesions indistinguishable from those of IgG4RD and they had similar clinical features to IgG4RD. It might be necessary to provide a position to these cases in “the extended spectrum of IgG4RD” defined as having IgG4+ cell inflammation and serum IgG4 elevation. Disclosure of Interest : None declared DOI 10.1136/annrheumdis-2014-eular.4032