Mean platelet volume can estimate the current vasculitis activity of microscopic polyangiitis.

Mean platelet volume (MPV) was known to be associated with the inflammatory burdens in various diseases, but not microscopic polyangiitis (MPA) yet. In this study, we investigated the association between MPV and Birmingham vasculitis activity score (BVAS) in 88 patients with MPA. We retrospectively reviewed the medical records of 88 patients with MPA. Clinical and laboratory data at diagnosis and partially at follow-up were collected. Linear regression analyses were used to clarify the association between MPV and BVAS. We also stratified MPA patients into three groups according to tertile of BVAS and define the lower limit of the highest tertile as the cut-off of BVAS for severe MPA. The area under the receiver operator characteristic curve (AUROC) was used to identify the cut-off of MPV for severe MPA (8.85 fL). The mean age was 60.5 years and 25 patients were male (28.4%). In univariable linear regression analysis, MPV was negatively correlated with BVAS (r = - 0.326). In multivariable linear regression analysis, MPA was independently associated with BVAS (ß = - 0.260). When we classified MPA patients into two groups based on the cutoff of MPV for severe MPA, patients having MPV ≤ 8.85 fL exhibited the higher frequency of severe MPA than those having MPV > 8.85 fL (RR 5.000). In addition, MPV increased along with improvement of vasculitis activity during the follow-up. In conclusion, MPV is significantly associated with BVAS and it can estimate the current BVAS in patients with MPA.

Red Blood Cell Distribution Width Can Predict Vasculitis Activity and Poor Prognosis in Granulomatosis with Polyangiitis.

PURPOSE: We investigated whether red blood cell distribution width (RDW) predicts vasculitis activity based on Birmingham vasculitis activity score (BVAS) or BVAS for granulomatosis with polyangiitis (GPA) at diagnosis and poor prognosis during follow-up in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). MATERIALS AND METHODS: We reviewed the medical records of 150 patients with AAV. We defined severe GPA as BVAS for GPA ≥7 (the highest quartile). Correlation and standardised correlation coefficients were analysed by linear regression tests. The differences between groups were evaluated by Mann-Whitney test. Relative risk (RR) was assessed by chi square test and Cox hazards model. RESULTS: RDW was correlated only with the vasculitis activity of GPA among patients with AAV. An increase in RDW was associated with the absence of ear nose throat (ENT) manifestation, but not proteinase 3-ANCA. Significant differences were noted in cumulative refractory free survival according to RDW ≥15.4% (p=0.007) and the absence of ENT manifestation (p=0.036). Multivariate Cox hazards analysis identified RDW ≥15.4% as the only significant predictor of refractory disease in GPA (RR 17.573). CONCLUSION: RDW predicts vasculitis activity in GPA, and RDW ≥15.4% at diagnosis may increase the risk of severe GPA at diagnosis and predict refractory diseases during follow-up.

The utility of the ACR/EULAR 2017 provisional classification criteria for granulomatosis with polyangiitis in Korean patients with antineutrophil cytoplasmic antibody-associated vasculitis.

OBJECTIVES: We applied the ACR/EULAR 2017 provisional classification criteria for granulomatosis with polyangiitis (GPA) to 150 Korean patients with previously diagnosed antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and investigated how many patients with AAV were reclassified as GPA. METHODS: We included patients with 30 GPA, 30 eosinophilic GPA (EGPA) and 90 microscopic polyangiitis (MPA) patients. Patients can be classified as GPA, when the sum of scores is more than 5. RESULTS: At diagnosis the mean age of 150 patients with AAV was 60.1 years old, and 101 patients (67.3%) were women. Overall, 33 of 150 patients with AAV (22.0%) were classified as GPA according to the 2017 provisional criteria for GPA. The 2017 provisional criteria for GPA dropped to 10.0% of previously diagnosed GPA patients and the major factor to drop 3 GPA patients was the deletion of 2 items of the 1990 criteria, urinary sediment and infiltrates on chest radiograph. Meanwhile, one of 30 patients with EGPA (3.3%) and 5 of 90 patients with MPA (5.6%) were newly classified as GPA based on the 2017 provisional criteria for GPA. We could also find that items of the 2017 provisional criteria to contribute to reclassifying EGPA and MPA patients as GPA were PR3-ANCA, mass-like lung lesion and nasal congestion in Korean patients with AAV. CONCLUSIONS: The use of the 2017 provisional criteria for GPA excluded 10.0% of previously classified GPA patients and newly classified 3.3% of EGPA patients and 5.6% of MPA patients as GPA in Korean patients with AAV.

Decreased muscle mass is independently associated with knee pain in female patients with radiographically mild osteoarthritis: a nationwide cross-sectional study (KNHANES 2010-2011).

To evaluate the association between muscle mass and knee pain in relation to radiographic severity of knee osteoarthritis. We consulted nationwide health examination and survey records collected from 2010 to 2011 and extracted data regarding female patients aged > 50 years and diagnosed with knee osteoarthritis. Radiographic severity was assessed on plain radiographs using the Kellgren-Lawrence system, whereas appendicular skeletal mass was obtained from dual-energy X-ray absorptiometry data. We performed multivariate logistic regression to evaluate the association between knee pain and muscle mass index (appendicular skeletal muscle mass divided by body weight in percentile) in patient groups stratified by radiographic severity of knee osteoarthritis. Among 17,476 participants of the national survey, 2013 female knee osteoarthritis patients were identified and stratified by radiographic severity (grade ≤ 1, n = 1136; grade 2, n = 240; grade 3, n = 379; and grade 4, n = 258). For mild osteoarthritis (Kellgren-Lawrence grade 2), muscle mass index was significantly lower in patients with knee pain than in those without knee pain (24.9 ± 3.9 vs 26.5 ± 6.3%, P = 0.023), whereas no such difference was noted for severe osteoarthritis (Kellgren-Lawrence grade > 2). After adjusting for clinical variables by multivariate logistic regression, decreased muscle mass index remained significantly associated with knee pain in patients with mild osteoarthritis but not in those with severe osteoarthritis (regression coefficient 0.915, 95% confidence interval 0.854-0.981, P = 0.012). Lower muscle mass may be a risk factor for knee pain in patients with radiographically mild knee osteoarthritis but not in those with radiographically severe osteoarthritis.

Birmingham vasculitis activity score of more than 9.5 at diagnosis is an independent predictor of refractory disease in granulomatosis with polyangiitis.

AIM: We investigated whether clinical manifestations, anti-neutrophil cytoplasmic antibodies (ANCAs), Birmingham vasculitis activity score (BVAS) for granulomatosis with polyangiitis (GPA) and five factor score (FFS) at diagnosis can predict relapse or refractory disease in 30 histology-proven GPA patients with follow-up duration ≥ 12 weeks. METHODS: We reviewed the medical records of 30 GPA patients. We collected clinical data, ANCAs, BVAS for GPA, FFSs at diagnosis, and we compared variables between the two groups based on relapse or refractory disease. The optimal cut-offs were extrapolated. Multivariate logistic regression and Cox hazard model analyses were conducted to identify predictors of refractory disease. RESULTS: The mean age and follow-up duration of patients were 63.3 years old and 45.2 months. The mean initial BVAS for GPA, FFS (1996) and FFS (2009) were 5.4, 0.6 and 1.0. There were no significant predictors of relapse. The mean BVAS for GPA, FFS (1996) and FFS (2009) of patients with refractory disease were higher than those without (P < 0.05 for all). Patients having BVAS for GPA ≥ 9.5, FFS (1996) ≥ 2 and FFS (2009) ≥ 2 exhibited significantly enhanced risk of refractory disease than those without (relative risk 23.0, 11.0, and 55.0, respectively), and low cumulative refractory disease-free survival rates. Multivariate Cox hazard model analysis proved BVAS for GPA ≥ 9.5 was an independent predictor of refractory disease during the follow-up duration (odds ratio 12.892). CONCLUSION: BVAS for GPA ≥ 9.5 was an independent predictor of refractory disease during follow-up duration ≥ 12 weeks.

Clinical and prognostic features of Korean patients with MPO-ANCA, PR3-ANCA and ANCA-negative vasculitis.

OBJECTIVES: We reclassified Korean patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) into 3 categories of AAV including MPO-ANCA, PR3-ANCA and ANCA-negative vasculitis, and investigated clinical and prognostic features. METHODS: We reviewed the medical records of 133 patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA), who had either myeloperoxidase (MPO)-ANCA, proteinase 3 (PR3)-ANCA or no ANCA, and who had ever achieved the first remission. We compared clinical manifestations, initial Birmingham vasculitis activity score (BVAS) and five factor score (FFS), and relapse rates. RESULTS: Patients with ANCA-negative vasculitis had the youngest mean age at diagnosis (50.0 years old) among AAV categories. General, cutaneous and renal manifestations were commonly observed in MPO-ANCA vasculitis, while mucous membrane, eye, ear nose throat (ENT) and renal manifestations were often documented in PR3-ANCA vasculitis. ENT manifestation was also frequently observed in ANCA-negative vasculitis. However, there were no significant differences in pulmonary and nervous system manifestations among 3 AAV categories. There were no significant differences in cumulative relapse free survival according to the presence of MPO-ANCA or PR3-ANCA or no ANCA. Meanwhile, initial BVAS or BVAS for GPA ≥13.5 in MPO-ANCA vasculitis and initial FFS (1996) ≥1 in MPO-ANCA and ANCA-negative vasculitis were significant predictors of relapse of each AAV category. CONCLUSIONS: Clinical manifestations varied AAV categories, and neither MPO-ANCA nor PR3-ANCA significantly affected relapse of AAV. Initial BVAS or BVAS for GPA and FFS (1996) helped to predict relapse of specified AAV categories.

Spontaneous rupture of hepatic metastasis from a thymoma: A case report.

Bleeding resulting from spontaneous rupture of the liver is an infrequent but potentially life threatening complication that may be associated with an underlying liver disease. A hepatocellular carcinoma or hepatic adenoma is frequently reported is such cases. However, hemoperitoneum resulting from a hepatic metastatic thymoma is extremely rare. Here, we present a case of a 62-year-old man with hypovolemic shock induced by ruptured hepatic metastasis from a thymoma. At the first hospital admission, the patient had a 45-mm anterior mediastinal mass that was eventually diagnosed as a type A thymoma. The mass was excised, and the patient was disease-free for 6 years. He experienced sudden-onset right upper quadrant pain and was again admitted to our hospital. We noted large hemoperitoneum with a 10-cm encapsulated mass in S5/8 and a 2.3-cm nodular lesion in the right upper quadrant of the abdomen. He was diagnosed with hepatic metastasis from the thymoma, and he underwent chemotherapy and surgical excision.