多発血管炎性肉芽腫症

BACKGROUND/AIMS: Cryoglobulinaemic vasculitis (CryoVas) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV) share the common characteristic of being small vessel vasculitides but exhibit significant differences in pathologic findings. This study investigated the incidence rate of cryoglobulinaemia and the concurrence of AAV and CryoVas in AAV patients without chronic viral hepatitis or haematologic malignancies. METHODS: Among the 324 patients diagnosed with AAV, 136 with available cryoglobulin test results were included. Their clinical data were reviewed, and clinical and laboratory data, including AAV-specific indices at diagnosis, were recorded. Repeated cryoglobulinaemia was defined as cryoglobulin positivity in sera on at least two occasions separated by an interval of ≥ 12 weeks. The classification criteria for CryoVas were applied to all 136 patients with AAV. RESULTS: The median age of the patients was 61.0 years (50.0-69.0), and 41.2% were male patients. Of the 136 patients, 75, 31, and 30 were diagnosed with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic GPA, respectively. Cryoglobulin was detected in sera of only two patients, yielding a detection rate of 1.5%. Both patients exhibited repeated cryoglobulinaemia and presented with red skin spots, constitutional symptoms, articular involvement, and vascular involvement; enabling their classification as having CryoVas alongside MPA. Additionally, one patient displayed peripheral neuropathy, reduced serum C4 levels, and rheumatoid factor positivity. CONCLUSION: This study represents the first demonstration that the incidence rates of cryoglobulinaemia and CryoVas are 1.5% in patients with AAV without chronic viral hepatitis or haematologic malignancies.

OBJECTIVES: Clinical heterogeneity in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is not fully captured by conventional disease subtypes or ANCA serotypes. We aimed to identify data-driven, clinically meaningful disease phenotypes based on patterns of organ involvement in microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA), and to evaluate their prognostic and therapeutic implications. METHODS: We conducted a multicenter retrospective cohort study using two nationwide Japanese registries (J-CANVAS as derivation; JPVAS as validation). Ensemble clustering was applied to high-dimensional organ involvement data derived from the Birmingham Vasculitis Activity Score and additional clinically relevant manifestations. The resulting clusters were replicated using classification and regression tree (CART) across the derivation and validation cohorts, and associations between cluster memberships and clinical outcomes (overall survival and relapse), as well as response to induction therapy, were evaluated. RESULTS: Among 726 patients with newly diagnosed MPA or GPA, ensemble clustering identified four reproducible clinical phenotypes: (a) renal-dominant without interstitial lung disease (ILD); (b) renal-dominant with ILD; (c) systemic multi-organ; and (d) ear, nose, and throat (ENT)-dominant. CART reproduced these clusters with good concordance. Overall survival and relapse incidence differed across clusters. The ENT-dominant cluster demonstrated favorable survival but a higher relapse risk and showed a trend toward improved relapse-free survival with rituximab compared with cyclophosphamide, even among myeloperoxidase-ANCA-positive patients. CONCLUSION: Organ involvement-based ensemble clustering identifies clinically meaningful and reproducible AAV phenotypes with distinct prognostic and therapeutic profiles. Integrating phenotypic patterns with ANCA serotype may enhance risk stratification and support more individualized management strategies in AAV.

OBJECTIVES: To analyse the clinical manifestations and treatment of childhood-onset antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and to identify risk factors for progression to end-stage renal disease (ESRD). METHODS: We retrospectively enrolled patients aged <18 years diagnosed with AAV at the First Affiliated Hospital of Zhengzhou University. Comparative analyses were performed between microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) groups regarding clinical manifestations, laboratory findings, disease activity, treatment regimens, and progression to ESRD. Statistical methods included the t-test, chi-square test, Cox regression analysis, and Kaplan-Meier curves. RESULTS: A total of 29 patients were included, of whom 22 were female and 23 had MPA. The median age at diagnosis was 13.9 years (IQR 12.3-16.5 years), and the median diagnostic interval was 1 month (IQR 0.6-1.5 months). The most common clinical manifestations were renal, respiratory, and musculoskeletal involvement, and the presentations were similar between MPA and GPA. Fifteen patients progressed to ESRD, 12 of whom had ESRD at initial diagnosis. Glucocorticoids served as the mainstay of treatment. At 12 months post-treatment, 12 patients achieved remission, 3 achieved renal remission, 8 remained on dialysis, and 6 underwent renal transplantation. Long-term survival was achieved in most patients, with one death. Multivariate Cox regression analysis identified elevated serum creatinine as an independent risk factor for progression to ESRD. CONCLUSIONS: In this cohort, childhood-onset AAV occurred predominantly in females, with MPA as the major subtype and the kidneys being the most frequently involved organ. Glucocorticoids formed the mainstay of treatment. Approximately half of the children achieved remission following treatment. Patients with elevated serum creatinine levels are at higher risk of progressing to ESRD. Most children achieved long-term survival.

OBJECTIVES: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare vasculitis characterised by eosinophilic inflammation, asthma, and often anti-neutrophil cytoplasmic antibody (ANCA) positivity. Monitoring disease activity is challenging because conventional tools, mainly the Birmingham Vasculitis Activity Score (BVAS), account for manifestations not relevant to EGPA while insufficiently capturing respiratory and eosinophilic features.This scoping review aims to systematically evaluate current methods, biomarkers, imaging studies or other procedures to monitor disease activity or treatment response in EGPA. METHODS: Eligible studies were those published between January 2005 and March 2025, including at least five EGPA patients (≥10% of the cohort), and focusing on disease activity or treatment response. Non-human studies, case reports, reviews, and investigations focused on diagnosis were excluded. RESULTS: Of 874 records screened, 58 studies met inclusion criteria. Clinical studies frequently adopted MIRRA trial definitions of remission (BVAS=0, prednisone ≤4 mg/day), but criteria varied across studies, with the most adopted secondary endpoints being oral glucocorticoid sparing, changes in eosinophil count and in pulmonary function. Biomarker investigations explored conventional lab parameters as well as emerging molecular candidates, but none achieved consistent reliability in distinguishing active from inactive disease. Imaging and procedures such as pulmonary function tests, high-resolution CT, FeNO, echocardiography, and cardiac MRI showed promise but lacked validation. CONCLUSIONS: No robust tool currently exists for EGPA monitoring, even though interesting biomarkers and imaging techniques warrant further validation. Future research should prioritise harmonising definitions of remission and relapse, distinguishing systemic from organ-specific activity, and integrate clinical, biomarker, and imaging approaches to develop EGPAspecific monitoring strategies.

AIM: To investigate seasonal patterns in new-onset antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) during the coronavirus disease 2019 (COVID-19) pandemic in Japan. METHODS: We conducted a retrospective cohort study using data from the nationwide multicenter registry of patients with AAV. Patients newly diagnosed with AAV between 2017 and 2022 were included, comprising microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA). We compared the seasonal distribution of AAV onset between the pre-COVID-19 (2017-2019) and COVID-19 (2020-2022) periods. RESULTS: A total of 871 patients were included (499 before and 372 during the COVID-19 period). Poisson regression analysis revealed a seasonal variation in AAV onset in the pre-COVID-19 period (p < 0.001), with lower onset in autumn [incidence rate ratio (IRR) 0.64, p < 0.001] and higher onset in spring and summer (IRR 1.16, p = 0.048 and IRR 1.23, p = 0.005) compared to winter. Importantly, a significant interaction between season and period (p for interaction = 0.032) indicated that the seasonal pattern differed between periods, with a relative increase in spring (IRR 1.16, p = 0.010). In the subanalyses, seasonal variation was consistently observed in MPA, but not in GPA and EGPA. The interaction between season and period was not statistically significant in each disease group. CONCLUSION: AAV exhibited a clear seasonal pattern, which differed during the COVID-19 period. However, these differences were not consistently observed across individual disease subtypes, and the factors underlying them remain unclear.