Impact of EUSTAR standardized training on accuracy of modified Rodnan skin score in patients with systemic sclerosis.

OBJECTIVES: To investigate the impact of European Scleroderma Trials and Research (EUSTAR) standardized training on the accuracy of modified Rodnan skin score (mRSS) in patients with systemic sclerosis (SSc). METHODS: Eight SSc patients (four diffuse, four limited) and 10 physicians (4 fellows, 6 professors) were included. Gold-standard mRSS was performed by a senior instructor. Training comprised a video presentation and a live demonstration. Each physician performed mRSS with no clinical information in all patients: (a) before training; (b) after video session; and (c) after live demonstration. Primary outcome was the change in scoring accuracy, which was defined as the difference from the gold-standard skin score, as analyzed using a linear mixed model. RESULTS: Mean (standard deviation) difference from the gold-standard score in all measurements by participants before the training was 7.7 (9.5). Completion of training significantly enhanced mRSS accuracy (adjusted ß = -7.61; 95% CI: -11.91 to -3.32). This was largely attributable to the video presentation (adjusted ß = -5.47; -9.16 to -1.78), although the live demonstration was associated with numerical reduction in the difference from the gold-standard score (adjusted ß = -2.15; -5.84 to 1.55). Effect of training was prominent in fellows whereas professors showed an increase in the difference from gold-standard score after training (P value for interaction <0.001). The intraclass correlation coefficient for physician skin scores was acceptable. However, no significant change was observed after training. CONCLUSION: New EUSTAR standardized mRSS training significantly enhanced mRSS accuracy, especially in participant with less previous experience in skin scoring.

Application of 2012 EULAR/ACR criteria for polymyalgia rheumatica to Korean patients previously classified by Chuang and Hunder criteria or Healey criteria.

AIM: To apply 2012 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) criteria to Korean patients previously classified as polymyalgia rheumatica (PMR) by Chuang and Hunder criteria or Healey criteria and investigated whether they might be still reclassified as PMR or not. METHODS: We retrospectively reviewed the medical records of 113 previously classified PMR patients. We applied 2012 EULAR/ACR criteria without ultrasonography to PMR patients, and fulfilment required at least 4 points. We evaluated odds ratios (OR) using logistic regression analyses. RESULTS: The mean age was 61.7 years. Seventy-one patients (62.8%) fulfilled Chuang and Hunder criteria, and 113 patients (100%) met Healey criteria. When we applied 2012 EULAR/ACR criteria, 98 patients fulfilled essential items (≥50 years, bilateral shoulder aching and abnormal C-reactive protein or erythrocyte sedimentation rate), and only 80 patients achieved points ≥4. Eight patients fulfilling the criteria exhibited higher frequencies of all the detailed items than those who did not. In multivariate logistic regression analysis, absence of rheumatoid factor or anti-citrullinated peptide antibodies was the only independent contributing item to the fulfilment of 2012 EULAR/ACR criteria (OR 23.571, 95% CI 6.357-87.407, P < .001). When we reclassified 33 excluded patients, the most common newly classified disease was generalized osteoarthritis (24.2%), followed by osteoporosis with compression fracture (15.2%). CONCLUSION: Eighty of 113 patients (81.6%) previously classified by Chuang and Hunder criteria or Healey criteria fulfilled 2012 EULAR/ACR criteria for PMR.

Renal outcome of kidney-transplantation in Korean recipients with ANCA-associated vasculitis.

OBJECTIVES: We investigated renal outcome of kidney-transplantation in Korean recipients with biopsy-proven renal involvement of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in a single centre. METHODS: We reviewed the medical records of 144 Korean patients and included 3 female patients with microscopic polyangiitis (MPA) and one male patient with eosinophilic granulomatosis with polyangiitis (EGPA) in this study. We obtained clinical and laboratory data related to kidney-transplantation, analysed renal outcome of kidney-transplantation in 4 recipients with AAV and compared it with those of previous studies. RESULTS: The mean age at diagnosis was 37.8 years and that at kidney-transplantation was 40.8 years. Time-gap from AAV to ESRD ranged from 1 to 48 months and that from AAV to kidney-transplantation ranged from 2 to 95 months. All kidney-recipients with had been followed-up for two years or greater. At diagnosis, MPO-ANCA was detected in only MPA patients, while, at transplantation, MPA-ANCA was detected in 2 MPA patients and an EGPA patient. All patients have received tacrolimus and mycophenolate mofetil based on glucocorticoid after kidney-transplantation. Among 4 kidney-recipients with AAV, one MPA patient underwent renal re-biopsies three times due to renal function deterioration during the follow-up. Renal histology revealed T cell-mediated and antibody-mediated rejection rather than relapse of MPA. Neither relapse nor graft failure was observed in our study. CONCLUSIONS: Renal outcome of kidney-transplantation in recipients with AAV was good and kidney-transplantation is deserved to be recommend as a safe and effective therapeutic modality to AAV patients with ESRD.

Echocardiographic features in patients with ANCA-associated vasculitis within 3 months before and after diagnosis.

We investigated the all-inclusive echocardiographic features in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) within 3 months before and after diagnosis. We reviewed the medical records of 89 AAV patients taking echocardiography and 35 age- and gender-matched controls. We collected clinical and laboratory data and echocardiographic results. We compared the variables between patients with AAV of each variant and controls and among those with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). The mean age and period from diagnosis to echocardiography were 54.5 years (30 men) and 1.4 months. The mean age of controls was 51.6 years. AAV patients exhibited lower mean left ventricle ejection fraction (LVEF) (64.0 vs. 69.1%, P = 0.002) and higher mean E/E' ratio (11.7 vs. 8.8, P = 0.001) and right ventricle systolic pressure (RVSP) (30.2 vs. 23.2 mm Hg, P < 0.001) than the controls. Each variant of AAV presented a different pattern of echocardiographic features in comparison with controls. MPA patients exhibited systolic and diastolic dysfunctions and pulmonary arterial hypertension more often than controls. Meanwhile, GPA patients exhibited only diastolic dysfunction, and EGPA patients had systolic dysfunction and pulmonary arterial hypertension more frequently than controls. No meaningful differences in echocardiographic features appeared among AAV variants. AAV patients exhibited reduced systolic function and advanced diastolic dysfunctions and pulmonary arterial hypertension near the time of diagnosis compared with controls. Each variant of AAV presented a different pattern in echocardiographic features.

Decreased ex vivo production of interferon-gamma is associated with severity and poor prognosis in patients with lupus.

BACKGROUND: Lupus pathogenesis is closely associated with interferon gamma (IFN-γ), which plays a central role in innate and adaptive immunity. The aim of this study was to evaluate the ex vivo production of IFN-γ after stimulation of peripheral blood mononuclear cells with phytohemagglutinin (PHA) in patients with lupus, according to disease activity. METHODS: This study included 118 patients with lupus who had undergone IFN-γ-releasing assays (IGRAs) to screen for tuberculosis. Data on IFN-γ production in negative (nil) and positive (mitogen with PHA) controls were collected and analysed. The difference (mitogen minus nil) was used to calculate ex vivo IFN-γ production. Disease activity was evaluated using the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2 K). Poor hospitalisation outcome was defined as in-hospital mortality or intensive care unit admission. Associations among disease activity, poor hospitalisation outcome, and ex vivo IFN-γ production were assessed. RESULTS: The level of ex vivo IFN-γ production was significantly lower in patients with active systemic lupus erythematosus (SLE) (n = 64) than in those with inactive SLE (n = 54) (median 0.92 vs. 11.06 IU/mL, p < 0.001). Ex vivo IFN-γ production was correlated with the SLEDAI-2 K (r = - 0.587, p < 0.001). Results of multivariate logistic regression analysis showed that ex vivo IFN-γ production ≤ 7.19 IU/mL was an independent predictor for discriminating active and inactive lupus. In addition, patients with ex vivo IFN-γ production ≤ 0.40 IU/mL had more frequent poor hospitalisation outcomes than those with ex vivo IFN-γ production > 0.40 (40.0% vs. 9.3%, p = 0.001). The proportion of indeterminate IGRA results was higher in patients with active lupus than in those with inactive lupus (45.3% vs. 0.0%, p < 0.001) because of decreased ex vivo IFN-γ production. CONCLUSIONS: Ex vivo IFN-γ production is a useful biomarker for assessing disease activity and predicting poor clinical outcomes of SLE.

Birmingham vasculitis activity score at diagnosis is a significant predictor of relapse of polyarteritis nodosa.

The objective of this study was to investigate whether clinical and laboratory data, Birmingham vasculitis activity score (BVAS) and five factor scores (FFS) at diagnosis could predict relapse in 30 patients with polyarteritis nodosa (PAN) having the follow-up duration for over 12 months. We reviewed the medical charts of 30 patients with PAN. We obtained clinical and laboratory data at diagnosis, and we compared them between the two groups based on relapse. The optimal cut-off values of BVAS and FFS (1996) at diagnosis to predict relapse were extrapolated. The mean age of patients (15 men) was 50.8 years, and the mean follow-up duration was 64.1 months. Nine patients (30.0%) had experience relapse after remission. Patients having relapse showed the higher frequency of weight loss and ocular symptoms and the less frequency of diastolic hypertension than those having not (p < 0.005 for all). On multivariate logistic regression analysis, weight loss was the only independent predictor of relapse, but on Cox Hazard model analysis, its statistical significance disappeared. The mean initial BVAS and FFS (1996) of patients in relapse group were higher than those of patients in no relapse group (p < 0.005 for all). Patients having initial BVAS over 13.5 and FFS (1996) over 1 exhibited significantly higher risk of relapse than those having not (RR 40.0 and RR 7.0, respectively). However, initial BVAS over 13.5 only remained significant in Kaplan-Meier survival analysis. In conclusion, BVAS over 13.5 at diagnosis was the only independent predictor of relapse of PAN.

Chest and renal involvements, Birmingham vascular activity score more than 13.5 and five factor score (1996) more than 1 at diagnosis are significant predictors of relapse of microscopic polyangiitis.

OBJECTIVES: We investigated whether specified organ involvements, antineutrophil cytoplasmic antibody (ANCA) positivity, Birmingham vasculitis activity score (BVAS) and five factor scores (FFS) at diagnosis could predict relapse of microscopic polyangiitis (MPA). METHODS: We reviewed the medical records of 90 patients with MPA. We collected clinical and prognostic data, (MPO)-ANCA and proteinase 3 (PR3)-ANCA, BVAS and FFS at diagnosis, and we compared them between the two groups. The optimal cut-off values of BVAS and FFS (1996) for predicting relapse were extrapolated. RESULTS: The mean age of patients (63 women) was 62.3 years and the mean follow-up duration was 41.7 months. At diagnosis, the mean BVAS, FFS (1996) and FFS (2009) of patients in no remission group were higher than those of patients in remission group (p<0.005 for all). Patients in relapse group exhibited chest and renal manifestations more frequently than those in no relapse group and the mean BVAS and FFS (1996) of patients in relapse group were significantly higher than those of patients in remission group (p<0.005 for all). There were no differences in MPO-ANCA and PR3-ANCA between the two groups. On multivariate logistic regression analysis, chest and renal manifestations were all independent predictors of relapse (OR 2.013 and OR 3.517). Patients who had BVAS ≥13.5 and FFS ≥ 1 exhibited a significantly increased risk of relapse than those who did not (RR 4.408 and RR 3.030). CONCLUSIONS: Chest and renal involvements, BVAS ≥13.5 and FFS ≥1 at diagnosis were independent predictors of relapse of MPA.