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Background Interstitial lung disease (ILD) is associated with high morbidity and mortality in patients with connective tissue disease (CTD). Because some patients with CTD overlap present with ILD first, with CTD diagnosed later, specific radiologic signs are needed to help differentiate each CTD or CTD-ILD from idiopathic ILD. Objectives To determine whether specific CT findings can help differentiate CTD as rheumatoid arthritis (RA), systemic sclerosis (SSc), or polymyositis/dermatomyositis (PM/DM). Methods We analyzed 143 consecutive ILD patients with RA, SSc, or PM/DM. We assessed diagnostic accuracy of CT findings of CTD-ILD, CT pattern, and signs including “anterior upper lobe honeycomb-like lesion” and “low attenuation area (LAA) within an interstitial abnormality” for each CTD-ILD. Prognostic predictors were determined using Cox regression models. Results Subjects were 78 patients with RA-ILD, 38 with SSc-ILD, 24 with PM/DM-ILD, and 3 with overlapping CTD-ILD. High frequency of anterior upper lobe honeycomb-like lesion suggests that CTD-ILD is due to RA-ILD (22%) rather than SSc-ILD (8%) or PM/DM-ILD (8%), whereas LAA within an interstitial abnormality suggests that CTD-ILD is due to SSc-ILD (26%) rather than RA-ILD (4%) or PM/DM-ILD (0%). Multivariate analysis showed that while not associated with survival, current or ex-smoker, honeycombing, and acute exacerbation were negative prognostic factors of mortality. Conclusions The tendency is high for RA-ILD, in which anterior upper lobe honeycomb-like lesion is a specific feature, to show UIP or NSIP/UIP pattern, combined emphysema, and honeycombing; SSc-ILD to show NSIP pattern and LAA within an interstitial abnormality; and PM/DM-ILD to show NSIP pattern and non-honeycombing.

Background: Interstitial lung disease (ILD) is associated with high morbidity and mortality in rheumatoid arthritis (RA). Although usual interstitial pneumonia (UIP) pattern was reported as a poor prognostic factor, in clinical practice, we often cannot classify high-resolution computed tomography (HRCT) patterns specifically as UIP or nonspecific interstitial pneumonia (NSIP). This study of RA-ILD aimed to elucidate prognosis by using our modified HRCT pattern classification according to the latest guideline on idiopathic pulmonary fibrosis (IPF). Methods: We analysed the medical records of 96 consecutive patients diagnosed as having RA-ILD. The modified HRCT classifications were defined as definite UIP, probable UIP, indeterminate for UIP (i.e., early UIP or NSIP/UIP), NSIP, organizing pneumonia (OP), NSIP+OP, and unclassifiable. Predictors of prognosis were determined using Cox regression models. Results: Our RA-ILD cohort included definite UIP (21%), probable UIP (20%), indeterminate for UIP (30%) including NSIP/UIP (27%), alternative diagnosis (29%) including NSIP (14%), and other patterns. Interrater agreement for HRCT pattern was good (κ =0.75). Multivariate analysis showed that older age, history of acute exacerbation, and radiological honeycombing were negative prognostic factors of mortality. Conclusions: NSIP/UIP pattern of indeterminate for UIP was the major pattern in RA-ILD. Although classifications of HRCT patterns were not related to survival, the presence of radiological honeycombing could be a useful predictor of poor prognosis, and acute exacerbation of ILD can seriously impact patient survival regardless of the presence of a UIP or indeterminate for UIP pattern. Our modified HRCT classification based on the latest IPF guideline might be useful to assess appropriate strategies of diagnosis in future RA-ILD studies, and radiological honeycombing could better predict poor prognosis rather than HRCT pattern.

We herein report the first case, to our knowledge, of tracheobronchial ulcer with anti-melanoma differentiation-associated gene 5 (anti-MDA 5) antibody-positive interstitial lung disease (ILD). A 53-year-old man complained of shoulder and wrist pain and was suspected of having polymyalgia rheumatica at another hospital. Thereafter, treatment with prednisolone was started. Although his arthralgia improved, he suffered from progressive dyspnea on exertion and an abnormal shadow was noted on chest X-ray, so he was transferred to our hospital. Chest computed tomography scan revealed ground-glass opacities and intralobular septal thickening. We diagnosed him as having clinically amyopathic dermatomyositis associated with ILD based on the specific skin findings and elevated anti-MDA 5 antibody titer. Fiberoptic bronchoscopy showed ulcerations of the trachea and bronchus. Treatment with dose increments of prednisolone combined with other immunosuppressive drugs resulted in improvement of his respiratory condition and tracheobronchial lesions. Clinicians should be aware that tracheobronchial ulcers can be associated with anti-MDA 5 antibody-positive interstitial lung disease. Keywords: Tracheo-bronchial ulcer, Anti-melanoma differentiation-associated gene 5, Interstitial lung disease

Catastrophic antiphospholipid syndrome (CAPS) survivors rarely relapse. We herein report a case of a second CAPS episode with an unusual subacute course and no microangiopathic hemolytic anemia (MAHA), a common CAPS symptom. During the first episode, the 69-year-old woman responded well to high-dose glucocorticoids and plasma exchange. On relapse, these treatments plus rituximab were ineffective and she died of multi-organ failure and bacterial cholangitis. The absence of MAHA and a subacute course do not exclude a CAPS recurrence.

To assess the utilities of ultrasonography (US) and low-field magnetic resonance imaging (compacTscan, cMRI) in the diagnosis of subclinical synovitis of hand joints of patients with rheumatoid arthritis (RA). A total of 1,540 joints of 77 RA patients were examined clinically, using US, using cMRI, and the baseline X-ray examination was performed. Clinical synovitis was defined as joint tenderness or swelling. Subclinical synovitis was diagnosed by US and by cMRI. The incidence of bone erosion and joint space narrowing was assessed by X-ray examination performed at approximately 40 weeks of follow-up. Of the hand joints examined, 294 (19.1 %) were diagnosed with clinical synovitis, and 218 joints (14.1 %) were diagnosed with subclinical synovitis. The remaining 1,028 joints (66.8 %) were synovitis-free on clinical examination and imaging. For the diagnosis of subclinical synovitis, cMRI (11.4 %) was significantly more sensitive than power Doppler signals detected by US (US-PD; 6.8 %) (P

IgG4-related disease (IgG4-RD) is characterized by IgG4-positive plasmacytic infiltration and fibrosis in various organs. Orbital involvement in IgG4-RD includes lacrimal glands, extra-ocular muscles, trigeminal nerve and other parts of the orbit. Immunohistochemical staining is used to diagnose IgG4-RD in patients with orbital inflammation. The purpose of this retrospective study was to clarify the clinicopathological features of IgG4-RD complicated with orbital involvement.We examined the clinical features, pathological findings and response to treatment in nine patients with IgG4-RD who underwent orbital tissue biopsy between April 2010 and August 2012 at the University of Tsukuba Hospital.Among the nine patients, eight had dacryoadenitis, one had infraorbital nerve swelling, and another one had IgG4-related orbital inflammation. Involvement of other organs was identified in all patients, including involvement of the salivary glands, lymph nodes, lung, kidney and para-aorta. In all patients, biopsy samples from orbital tissues showed lymphoplasmacytic infiltration and fibrosis, and IgG4-positive/IgG-positive plasmacyte ratio of40%. All patients were treated with prednisolone (0.6 mg/kg/day) and responded well in early phase, although relapse was noted in two patients following tapering of prednisolone, evident by swelling of lacrimal glands.Patients with IgG4-RD complicated with orbital involvement often present with involvement of other organs. The histopathological findings of orbital tissue match the characteristic features of IgG4-RD. Corticosteroid is effective for orbital and systemic involvement in IgG4-RD.

Although glucocorticoids are effective for patients with IgG4-related disease, the treatment has not yet been standardized. Therefore, the treatment strategy should be established.Patients who fulfilled the comprehensive diagnostic criteria for definite IgG4-related disease were started on prednisolone (0.6 mg/kg body weight) with the dose reduced every two weeks. The subsequent maintenance dose and need for prednisolone were determined for individual patients. The primary endpoint was the complete remission (CR) rate at one year. Secondary endpoints included overall response rate (ORR), the maintenance dose, the relapse rate, and adverse events.This study enrolled 61 patients. After clinicopathological review, three patients were excluded, and one, 13, and 44 patients were diagnosed with probable, possible, and definite IgG4-related disease, respectively. Of the 44 patients with definite IgG4-RD, 29 (65.9%) achieved CR, and the ORR was 93.2%. No patient was refractory to primary treatment. The most frequent adverse events were glucose intolerance. Six patients relapsed.Glucocorticoid treatment is usually effective for patients with IgG4-RD, and we should examine the possibility of other disorders when a patient is glucocorticoid refractory. Some patients are misdiagnosed, making central clinicopathological review of diagnosis very important in conducting clinical studies.

OBJECTIVE To devise an effective method to assess the peripheral circulation using an infrared thermographic analysis. METHODS Sequential measurements of the skin temperature before and after cold-water immersion of the hands were analyzed by a thermographic examination in healthy controls and patients diagnosed to have Raynaud phenomenon (RP). The skin temperatures of the dorsum of all fingernail folds and the metacarpophalangeal (MCP) joints were measured at baseline. Then the hands were immersed in 10°C water for 10 s, and the skin temperatures were measured at 0, 3, 5, 10, 15, 20 and 30 min after immersion. The mean temperature, recovery rate and disparity (coefficient of variation) of the nail fold temperatures were calculated. The distal-dorsal difference (DDD) was calculated by subtracting the mean MCP temperature from the mean nail fold temperature. Receiver operating characteristic (ROC) curves were generated to compare these parameters in terms of their capability to differentiate patients with RP. RESULTS Thirty-one RP patients and 25 controls were included in the study. The baseline nail fold temperature was significantly lower in RP patients than in the controls. The RP patients had a lower recovery rate, lower DDD and higher disparity than the controls. The disparity and DDD were negatively correlated (r=-0.63, p

Interstitial lung disease (ILD) is induced by various factors in humans. However, the exact mechanism of ILD remains elusive. This study sought to determine the role of natural killer (NK) 1.1(+) γδT cells in ILD. The injection of IL-18 plus IL-2 (IL-18/IL-2) into C57BL6 (B6) mice induced acute ILD that resembled early-stage human ILD. An accumulation of NK1.1(+) γδT cells similar to NK cells was evident in the lungs. The T Cell Receptor (TCR) Vγ and Vδ repertoires of NK1.1(+) γδT cells indicated polyclonal expansion. The expression of IL-2 receptor β (Rβ) and IL-18Rβ in NK1.1(+) γδT cells was higher than in NK1.1(-) γδT cells. IL-18/IL-2 stimulated the proliferation of NK1.1(+) γδT cells, but not NK1.1(-) γδT cells. The IL-18/IL-2-stimulated NK1.1(+) γδT cells produced higher concentrations of IFN-γ than did NK1.1(-) γδT cells. Moreover, NK1.1(+) γδT and NK1.1(-) γδT cells constituted completely different cell populations. The IL-18/IL-2-induced ILD was milder in TCRδ(-/-) and IFN-γ(-/-) mice, compared with B6 mice. Furthermore, cell-transfer experiments demonstrated that NK1.1(+) γδT cells could induce the expansion of NK cells and IFN-γ mRNA in the lung by IL-18/IL-2. Our results suggest that NK1.1(+) γδT cells function as inflammatory mediators in the early phase of IL-18/IL-2-induced ILD.

Low-field extremity magnetic resonance imaging (lfMRI) is currently commercially available and has been used clinically to evaluate rheumatoid arthritis (RA). However, one disadvantage of this new modality is that the field of view (FOV) is too small to assess hand and wrist joints simultaneously. Thus, we have developed a new lfMRI system, compacTscan, with a FOV that is large enough to simultaneously assess the entire wrist to proximal interphalangeal joint area. In this work, we examined its clinical value compared to conventional 1.5 tesla (T) MRI. The comparison involved evaluating three RA patients by both 0.3 T compacTscan and 1.5 T MRI on the same day. Bone erosion, bone edema, and synovitis were estimated by our new compact MRI scoring system (cMRIS) and the kappa coefficient was calculated on a joint-by-joint basis. We evaluated a total of 69 regions. Bone erosion was detected in 49 regions by compacTscan and in 48 regions by 1.5 T MRI, while the total erosion score was 77 for compacTscan and 76.5 for 1.5 T MRI. These findings point to excellent agreement between the two techniques (kappa = 0.833). Bone edema was detected in 14 regions by compacTscan and in 19 by 1.5 T MRI, and the total edema score was 36.25 by compacTscan and 47.5 by 1.5 T MRI. Pseudo-negative findings were noted in 5 regions. However, there was still good agreement between the techniques (kappa = 0.640). Total number of evaluated joints was 33. Synovitis was detected in 13 joints by compacTscan and 14 joints by 1.5 T MRI, while the total synovitis score was 30 by compacTscan and 32 by 1.5 T MRI. Thus, although 1 pseudo-positive and 2 pseudo-negative findings resulted from the joint evaluations, there was again excellent agreement between the techniques (kappa = 0.827). Overall, the data obtained by our compacTscan system showed high agreement with those obtained by conventional 1.5 T MRI with regard to diagnosis and the scoring of bone erosion, edema, and synovitis. We conclude that compacTscan is useful for diagnosis and estimation of disease activity in patients with RA.

To compare magnetic resonance imaging (MRI) and ultrasonography (US) in the detection of joint inflammation of rheumatoid arthritis (RA), 6 patients with RA were examined by US and low-field 0.3-T nonenhanced dedicated extremity MRI (compacTscan). All patients were females, with mean age of 50.2 years, mean disease duration of 13.5 years, and mean disease activity score (DAS)28-CRP of 1.78. Each patient was treated with either infliximab, etanercept, adalimumab, or tocilizumab. Intercarpal joints, radioulnar joints, second through fifth proximal interphalangeal (PIP) joints, and first through fifth metacarpophalangeal (MCP) joints (a total of 132 joints, 22 joints in each patient) were assessed by MRI for presence of joint inflammation. A total of 156 joints (24 first interphalangeal and radiocarpal joints plus the above 132 joints), were assessed by grayscale US (GS-US) and power Doppler US (PD-US) for presence of joint inflammation by two trained ultrasonographers. We assessed correlations between joint inflammations on MRI and GS-US/PD-US, and also interobserver correlation between the two ultrasonographers by calculating intraclass correlation coefficients (ICC). Synovial hypertrophy and/or synovial fluid was detected in 74/156 joints on GS-US, and synovitis was detected in 10/156 joints on PD-US and in 38/132 joints on MRI. Using PD-US as a reference, sensitivity of MRI in detection of synovitis was 80%. Using MRI as a reference, sensitivity of PD-US was 21%. Specificity of PD-US was higher than that of MRI. Overall agreement between GS-US and MRI and between PD-US and MRI was 0.56 and 0.76, respectively, suggesting that results of PD-US are close to those of MRI. ICC was 0.545 for GS-US and 0.807 for PD-US, suggesting specificity of PD-US in detecting joint inflammation. Our results show that findings of PD-US correlated with those of MRI. Low-field MRI and PD-US are useful tools for assessment of patients with RA.

Objective Interstitial pneumonia (IP) is a chronic progressive interstitial lung disease associated with high mortality and poor prognosis. However, the pathogenesis of IP remains to be elucidated. The aim of this study was to clarify the role of CD161(+) Vδ1(+) γδ T cells in SSc patients with IP. Methods The proportion of CD161(+) Vδ1(+) γδ T cells in peripheral blood mononuclear cells (PBMCs) and serum sialylated carbohydrate antigen (KL-6) levels were determined. GeneChip analysis was performed with CD161(-) and CD161(+) Vδ1(+) γδ T cells. Cytokine and chemokine expression from CD161(+) Vδ1(+) γδ T cells was measured and used to evaluate the effect of culture supernatant on fibroblast proliferation. Results The proportion of CD161(+) Vδ1(+) γδ T cells was significantly higher in SSc than healthy controls (HCs) and correlated negatively with serum KL-6 levels in IP-positive SSc patients. The gene and mRNA expression level of chemokine ligand 3 (CCL3) was markedly higher in CD161(+) Vδ1(+) γδ T cells than in CD161(-) Vδ1(+) γδ T cells. CD161(+) Vδ1(+) γδ T cells in IP-positive SSc patients showed higher production of CCL3 and lower production of IFN-γ than in HCs. Culture supernatant derived from IP-negative and IP-positive SSc patients promoted fibroblast proliferation, whereas that from HCs did not. Conclusion The small proportion and the altered cell functions of CD161(+) Vδ1(+) γδ T cells among PBMCs in SSc patients play a role in the pathogenesis of IP. These findings suggest that CD161(+) Vδ1(+) γδ T cells may play a regulatory role in the pathogenesis of IP in SSc patients via IFN-γ production.

Objective Invariant natural killer T (iNKT) cells regulate collagen-induced arthritis (CIA) when activated by their potent glycolipid ligand, alpha-galactosylceramide (α-GalCer). Glucose-6-phosphate isomerase (GPI)-induced arthritis is a closer model of human rheumatoid arthritis based on its association with CD4+ T cells and cytokines such as TNF-α and IL-6 than CIA. Dominant T cell epitope peptide of GPI (GPI325-339) can induce arthritis similar to GPI-induced arthritis. In this study, we investigated the roles of activation of iNKT cells by α-GalCer in GPI peptide-induced arthritis. Methods Arthritis was induced in susceptible DBA1 mice with GPI peptide and its severity was assessed clinically. The arthritic mice were treated with either the vehicle (DMSO) or α-GalCer. iNKT cells were detected in draining lymph nodes (dLNs) by flow cytometry, while serum anti-GPI antibody levels were measured by enzyme-linked immunosorbent assay. To evaluate GPI peptide-specific cytokine production from CD4+ T cells, immunized mice were euthanized and dLN CD4+ cells were re-stimulated by GPI-peptide in the presence of antigen-presenting cells. Results α-GalCer induced iNKT cell expansion in dLNs and significantly decreased the severity of GPI peptide-induced arthritis. In α-GalCer-treated mice, anti-GPI antibody production (total IgG, IgG1, IgG2b) and IL-17, IFN-γ, IL-2, and TNF-α produced by GPI peptide-specific T cells were significantly suppressed at day 10. Moreover, GPI-reactive T cells from mice immunized with GPI and α-GalCer did not generate any cytokines even when these cells were co-cultured with APC from mice immunized with GPI alone. In vitro depletion of iNKT cells did not alter the suppressive effect of α-GalCer on CD4+ T cells. Conclusion α-GalCer significantly suppressed GPI peptide-induced arthritis through the suppression of GPI-specific CD4+ T cells.

We encountered a disseminated sporotrichosis patient with polyarthritis and progressive skin ulcers, who had been previously treated with prednisolone, tocilizmab, tacrolims, and cyclophosphamide under the diagnosis of rheumatoid arthritis in another hospital. Making the diagnosis of leukocytoclasticvasculitis based on the clinical observation of skin ulcers, we intensified immunosuppressive therapy. Unfortunately, the patient developed septic shock. Blood culture revealed that the pathogenic organism was sporothrixschenckii. Any case of intractable arthritis or skin ulcers, which does not improve, despite adequate immunosuppressive therapy, is likely to be suspicious of sporotrichosis.

Tocilizumab (TCZ) is effective in patients with rheumatoid arthritis (RA) who are refractory to anti-tumor-necrosis-factor (anti-TNF) biologics. The Rheumatoid Arthritis Society Disease Activity Score in 28 Joints (DAS28) is used to evaluate the response to TCZ. However, DAS28 is inappropriate marker because TCZ normalizes C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) in the early stage of treatment. The aim of our study was to test the usefulness of magnetic resonance imaging (MRI)-based markers of response to TCZ treatment. Nine patients with RA who were refractory to anti-TNF inhibitors (six to infliximab, one to etanercept, one to adalimumab, and one to both) were assessed. MRI images of both hands were obtained by low-field extremity MRI at baseline, 20, and 44 weeks of treatment, in addition to assessment with DAS28–ESR. The effect of TCZ on RA was examined by compact MRI score (cMRIS). All patients showed good or moderate response to TCZ treatment, as evaluated by significant reduction in DAS28–ESR at both 20 and 44 weeks (p

We compared the characteristics of MRI imaging and US imaging on joint inflammation in patients with rheumatoid arthritis (RA) . MRI imaging is worth for diagnosis of RA at the early stage, evaluation of drug treatment effects, and a new measure for true remission. US imaging is also useful for estimation of therapy and a criteria for complete remission, and cheep machine. We hope that both imaging technologies will be a standard for diagnosis and therapy evaluation in RA patients.

IgG4-related disease is a new disease classification established in Japan in the 21st century. Patients with IgG4-related disease display hyper-IgG4-gammaglobulinemia, massive infiltration of IgG4+ plasma cells into tissue, and good response to glucocorticoids. Since IgG4 overexpression is also observed in other disorders, it is necessary to diagnose IgG4-related disease carefully and correctly. We therefore sought to determine cutoff values for serum IgG4 and IgG4/IgG and for IgG4+/IgG+ plasma cells in tissue diagnostic of IgG4-related disease.Patients and Methods. We retrospectively analyzed serum IgG4 concentrations and IgG4/IgG ratio and IgG4+/IgG+ plasma cell ratio in tissues of 132 patients with IgG4-related disease and 48 patients with other disorders.Result. Serum IgG4 >135 mg/dl demonstrated a sensitivity of 97.0% and a specificity of 79.6% in diagnosing IgG4-related disease, and serum IgG4/IgG ratios >8% had a sensitivity and specificity of 95.5% and 87.5%, respectively. IgG4+cell/IgG+ cell ratio in tissues >40% had a sensitivity and specificity of 94.4% and 85.7%, respectively. However, the number of IgG4+ cells was reduced in severely fibrotic parts of tissues.Conclusion. Although a recent unanimous consensus of all relevant researchers in Japan recently established the diagnostic criteria for IgG4-related disease, findings such as ours indicate that further discussion is needed.

Summary Alpha-carba-GalCer (RCAI-56), a novel synthetic analogue of α-galactosylceramide (α-GalCer), stimulates invariant natural killer T (NK T) cells to produce interferon (IFN)-γ. IFN-γ exhibits immunoregulatory properties in autoimmune diseases by suppressing T helper (Th)-17 cell differentiation and inducing regulatory T cells and apoptosis of autoreactive T cells. Here, we investigated the protective effects of α-carba-GalCer on collagen-induced arthritis (CIA) in mice. First, we confirmed that α-carba-GalCer selectively induced IFN-γ in CIA-susceptible DBA/1 mice in vivo. Then, DBA/1 mice were immunized with bovine type II collagen (CII) and α-carba-GalCer. The incidence and clinical score of CIA were significantly lower in α-carba-GalCer-treated mice. Anti-IFN-γ antibodies abolished the beneficial effects of α-carba-GalCer, suggesting that α-carba-GalCer ameliorated CIA in an IFN-γ-dependent manner. Treatment with α-carba-GalCer reduced anti-CII antibody production [immunoglobulin (Ig)G and IgG2a] and CII-reactive interleukin (IL)-17 production by draining lymph node (DLN) cells, did not induce apoptosis or regulatory T cells, and significantly increased the ratio of the percentage of IFN-γ-producing T cells to IL-17-producing T cells (Th1/Th17 ratio). Moreover, the gene expression levels of IL-6 and IL-23p19, Th17-related cytokines, were reduced significantly in mice treated with α-carba-GalCer. In addition, we observed higher IFN-γ production by NK T cells in α-carba-GalCer-treated mice in the initial phase of CIA. These findings indicate that α-carba-GalCer polarizes the T cell response toward Th1 and suppresses Th17 differentiation or activation, suggesting that α-carba-GalCer, a novel NK T cell ligand, can potentially provide protection against Th17-mediated autoimmune arthritis by enhancing the Th1 response.

Background Peripheral circulation is often disturbed in patients with connective tissue diseases (CTDs). Raynaud phenomenon (RP) is prevalent in and considerably characteristic of CTDs and we have long noticed colour unevenness among fingers in patients during RP attacks. We hypothesized that temperature unevenness among fingers detected by infrared thermography would be a useful parameter to evaluate peripheral circulation. Objectives To evaluate temperature unevenness among fingers as a thermographic parameter by comparing it with other parameters validated in previous studies [1-3]. Methods Patients with RP who underwent thermographic examinations at our hospital were included and compared with healthy volunteers. Skin temperatures of dorsum of hands at 10 fingers9 nail folds and MCP joints were measured at baseline. Then hands were immersed in 10°C water for 10 seconds. Skin temperatures were measured at 0, 3, 5, 10, 15, 20, and 30 min after immersion. Mean temperature, recovery rate (temperature recovery from immersion/decrease by immersion), and coefficient of variation (standard deviation/mean temperature) of nail fold temperature were calculated. Higher coefficient of variation means temperature among fingers is more uneven. Distal-dorsal difference (DDD: measured by subtracting mean temperature of MCP from that of nail fold) was also calculated and these parameters were compared between the two groups. Receiver operating characteristic (ROC) curve was generated to compare these parameters in terms of their capability of differentiating patients with RP from HCs. Results Thirty-one patients with RP (10 with primary Raynaud, 11 with systemic sclerosis (SSc), 11 with other CTDs) were included and compared with 25 healthy controls (HCs). Baseline nail fold temperature was significantly lower in patients of RP than in HC (30.8±3.1°C vs. 33.2±1.8°C, p=0.0002). Cold-water immersion of hands revealed patients with RP had lower recovery rate, lower DDD, and higher coefficient of variation than did HCs. The differences in these parameters were the most evident at 5 min after immersion (patients with RP vs HCs: recovery rate; 49.6±27.7 vs 71.5±26.8, p=0.004 DDD; -1.4±2.8 vs 0.85±2.7, p=0.0008 coefficient of variation; 0.053±0.024 vs 0.021±0.015, p=1.2x10 –6 ). On the basis of ROC curve analyses for these parameters, coefficient of variation of nail fold temperature most effectively differentiated patients with RP from HCs (Area under the curve; recovery rate: 0.64 DDD: 0.79 coefficient of variation: 0.88). Conclusions Unevenness of temperature among fingers was the most useful thermographic parameter to evaluate disturbed peripheral circulation. References O9Reilly D, et al. (1992) Measurement of cold challenge responses in primary Raynaud9s phenomenon and Raynaud9s phenomenon associated with systemic sclerosis. Ann Rheum Dis 51: 1193-1196. Anderson ME, et al. (2007) The 9distal-dorsal difference9: a thermographic parameter by which to differentiate between primary and secondary Raynaud9s phenomenon. Rheumatology (Oxford) 46: 533-538. 12. Murray AK, et al. (2009) Noninvasive imaging techniques in the assessment of scleroderma spectrum disorders. Arthritis Rheum 61: 1103-1111. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.4849

Polymorphisms in the genomic region encoding B lymphoid tyrosine kinase ( BLK ) and family with sequence similarity 167, member A ( FAM167A , also referred to as C8orf13 ) at 8p23.1 have been associated with systemic lupus erythematosus (SLE) in Caucasian1 2 and Asian3 4 populations. A recent genome-wide study in a north American population showed new associations with rheumatoid arthritis (RA), among which was a single nucleotide polymorphism (SNP) rs2736340 in the intergenic region of BLK and FAM167A .5 In the HapMap Japanese samples (http://www.hapmap.org/index.html.ja), this SNP is in absolute linkage disequilibrium ( r 2=1) with rs13277113, previously associated with SLE.1,–,4 We have shown that the population frequency of the risk genotype rs13277113A/A and the OR for SLE …

Background Magnetic resonance imaging (MRI) is able to detect synovitis, bone marrow edema and bone erosion, which X-ray radiograph is not. This modality of imaging would be helpful in clinical practice in rheumatic diseases. Objectives To assess the usefulness of low field MRI to make a diagnosis of rheumatoid arthritis (RA), and to predict future bone destruction. Methods We developed 0.3 tesla low field compact MRI (cMRI). All the patients who underwent examination by cMRI were included. T1 weighted image and short tau inversion recovery image of both hands were taken. We scored every site for 0-3 points by compact MRI score [1] as degree of affection; 23 sites for bone erosion, 23 sites for bone marrow edema and 11 sites for synovitis. Of all RA patients, those who had 2 sequential examinations with 6 to 12 months interval were analyzed to find the poor prognosis factor of bone destruction. Results Five hundreds and sixty four hands of 283 patients were included and divided into 4 groups; 168 hands of 84 patients were RA treated with biologic agents (infliximab 37 patients, etenercept 23, tocilizumab 13, adalimumab 6 and abatacept 5) (Group 1), 209 hands of 105 patients were RA treated without biologic agent (Group 2), 54 hands of 27 patients had arthralgia or synovitis caused by other connective tissue diseases (Group 3) and 133 hands of 67 patients were undifferentiated arthritis (Group 4). Erosion score, bone marrow edema score and synovitis score in group 1, 2 and 3 were significantly higher than that of Group 4 (erosion score; 19.13±14.37, 11.40±11.32, 6.98±11.67 and 1.94±2.42 for group 1 to 4 respectively, bone marrow edema score; 2.55±6.62, 1.18±4.47, 0.66±6.00 and 0.25±1.92, synovitis score; 5.16±6.43, 3.57±3.89, 1.91±3.04 and 0.64±1.35, p 2 =0.29, p=0.0031). One synovitis score at the first examination would predict 0.33 point progression in erosion score at the second examination. Conclusions cMRI examination would be of use to make diagnosis of RA from undifferentiated arthritis. Synovitis observed by cMRI would be a poor prognosis factor of bone destruction. References Suzuki, et al. Mod Rheumatol 2009, 19, 358 Disclosure of Interest None Declared