Nobuyuki Ono

Subsets of RP105-negative plasmablasts in IgG4-related disease

We read with interest the paper by Wallace ZS, plasmablasts as a biomarker for IgG4-related disease (IgG4-RD), independent of serum IgG4 concentrations.1 Evidently, the study showed that patients with active, untreated IgG4-RD have elevations in their circulating plasmablast counts. We strongly approve their results and propose new insights into plasmablast subsets in …

Persistent expression of CXCR5 on plasmablasts in IgG4-related disease

We read with interest the papers by Fox and Fox,1 and Wallace et al 2 on IgG4 levels and plasmablasts as a marker for IgG4-related disease (IgG4-RD). We strongly approve their results and have reported plasmablasts lacking RP105 (CD180) in IgG4-RD in your journal.3 Recently, we have found an important and additional result about plasmablasts in IgG4-RD. RP105-negative B cells are assigned as five subsets of activated late B cells (subset 1), early or pre-plasmablasts (subset 2), plasmablasts (subset 3), early plasma cells (subset 4) and plasma cells (subset 5).4 However, precise phenotype of RP105-negative B cells in IgG4-RD was hitherto unknown.5 To clarify the phenotype of RP105-negative B cells, multicolour analysis (more than 200 antigens …

Effectiveness of Certolizumab Pegol in Treating Rheumatoid Arthritis Patients with Persistent Inflamed Residual Mono- or Oligosynovitis Resistant to Prior TNF-α Inhibitors

We report four cases of successful treatment with certolizumab pegol (CZP) of rheumatoid arthritis (RA) patients with persistent inflamed residual mono- or oligosynovitis resistant to prior TNF-α inhibitors. Although the patients were in a moderate disease activity, a low activity, or a remission of RA, they sustained inflammatory mono-/oligoarthritis even after treatment with prior TNF inhibitors. They were then all treated with CZP and observed in a serial ultrasonography. In all cases, the positive power Doppler signals in the joint have disappeared promptly and all of the patients were able to retain remission in the long term. The treatment of CZP to the refractory mono-/oligoarthritis of inflammatory synovitis in RA patients has not been previously described. The cases suggest that it may be associated with the feature of CZP, possible effective penetration into the site of inflammation.

Are the 2016 EULAR/ACR/PRINTO classification criteria for macrophage activation syndrome applicable to patients with adult-onset Still’s disease?

The objectives of this study are to determine whether the 2016 European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organization classification criteria for macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (SJIA) can be used to identify MAS in patients with adult-onset Still’s disease (AOSD). Using laboratory data from 76 AOSD patients with and without MAS, we analyzed the ability of the collective and individual constitutive elements of the 2016 MAS in SJIA criteria and additional laboratory measures to discriminate between AOSD patients with (n = 16) and without (n = 60) MAS. Cutoff values to determine the sensitivity, specificity, and predictive values were calculated from receiver operating characteristic curves, and modified classification criteria for MAS in AOSD were evaluated. The 2016 MAS in SJIA classification criteria had an overall sensitivity of 100%, specificity of 70.0%, positive predictive value of 47.1%, and negative predictive value of 100% to discriminate between AOSD patients with and without MAS based on laboratory data. Among the individual criteria, the sensitivity of triglycerides (46.7%) and the specificity of ferritin (15.0%) for MAS in AOSD were particularly low. The sensitivity and specificity for classifying MAS in AOSD patients were increased to 100 and 93%, respectively, by excluding triglycerides and changing the cutoff values for other criteria in the 2016 MAS in SJIA classification. The 2016 classification criteria for MAS in SJIA had higher sensitivity but lower specificity to identify MAS in AOSD patients compared with SJIA patients.

A case of granulomatosis with polyangiitis accompanied with strawberry gingivitis, and a review of the literatures

Abstract Hyperplastic granular gingivitis, also known as “strawberry gingivitis” (SG), is a characteristic oral presentation of granulomatosis with polyangiitis (GPA). We report a case of a 38-year-old woman who complained of swollen gums, which had an over-ripe strawberry appearance. Examination revealed pulmonary involvement and a positive finding for proteinase 3 anti-neutrophil cytoplasmic (PR3-ANCA) antibody. A gingival biopsy showed granulomatous infiltration, neovascularisation, inflammatory cell infiltration and pseudoepitheliomatous hyperplasia. These clinical features suggested a diagnosis of GPA. A moderate dose of systemic corticosteroids and methotrexate was effective. To confirm her diagnosis, we conducted a literature review of SG accompanied with GPA. The physical and pathological presentations of SG were typical for GPA. Most cases were positive for PR3-ANCA. Some showed pathological proof of vasculitis with granulomatous formations, suggesting that SG is a typical feature of GPA. Most reviewed cases were classified as being localised or at the early systemic stage, and required less intensive treatment and resulted in a favourable outcome. The findings suggest that SG is an early manifestation of GPA, and GPA should be considered for patients who present with swollen gums.

Successful golimumab therapy in four patients with refractory Takayasu’s arteritis

Abstract Recent studies suggested that anti-TNF-α biological therapies are effective in treating Takayasus arteritis (TA) refractory to conventional immunosuppressive therapy. However, the efficacy of golimumab (GLM) for TA therapy is unknown. We report four women with TA who were successfully treated with GLM. GLM was prescribed as induction therapy for three patients and as maintenance therapy for one patient. GLM showed therapeutic value and might be useful, together with other anti-TNF-α agents, in treating TA.

SAT0285 Characteristics of MPO-ANCA Positive Granulomatosis with Polyangitis

Objectives Through the investigation of the actual situation of ANCA associated vasculitis in Japan, we attempted to clarify the characteristics of MPO-ANCA positive Granulomatosis with polyangitis (MPO-GPA). Methods Retrospectively we recruited 38 GPA cases from 8 hospitals, and 41 Microscopic polyangitis (MPA) cases from one hospital. To exclude diagnostic overlaps, GPA and MPA were classified by EMA classification. Their clinical courses were analyzed based on sex, age, ANCA, organ involvements and treatment outcomes. Results The mean age of GPA and MPA were 64.9 and 72.3 years old respectively. Among GPA, 15 cases (39%) were positive for PR3-ANCA, 17 (45%) cases were positive for MPO-ANCA, and 6 cases (16%) were ANCA negative. All MPA were MPO-ANCA positive. The mean ages are 60.4, 69.6, 63.0 and 72.3 years old respectively. The ratios of female are 20%, 82%, 50% and 56%. Compared to PR3-ANCA positive GPA (PR3-GPA), MPO-GPA had more otitis media, less arthritis, lower serum creatinine levels and more neuronal involvement. Compared to MPA, MPO-GPA had significantly more ENT involvements, pulmonary involvements and lower creatinine level. Both GPAs experienced more relapses than MPA. MPA showed significantly poor outcome than GPA (p=0.008). The mortality rates of GPA and MPA were 5% (mean follow-up time 1576 days) and 24.4% (mean follow-up time 778.2 days). MPO-GPA showed significantly fairer outcome than MPA even though their higher age (p=0.013). The univariate analysis selected following factors as predictors of a poor outcome: pulmonary UIP pattern (P=0.001), Cr1.7mg/dl (p=0.005), absence of ENT involvement (p=0.032), higher age (65 years old, p=0.062) and pulmonary hemorrhage (p=0.081). Conclusions MPO-GPA is characterized by older female patients with otitis media, neuronal involvement and less renal injuries. Like PR3-GPA, MPO-GPA showed fairer treatment outcome but more relapse than MPA. Because of their higher age and fairer outcome, we need to manage MPO-GPA differently from PR3-GPA and MPA. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.1283