好酸球性多発血管炎性肉芽腫症

BACKGROUND: The global resurgence of syphilis poses growing challenges for clinical diagnosis, particularly when atypical presentations without classic mucocutaneous lesions mimic systemic autoimmune diseases. Pulmonary involvement in secondary syphilis is rare and frequently misdiagnosed, leading to inappropriate immunosuppressive therapy. CASE PRESENTATION: A 24-year-old male presented with one month of inflammatory back pain and two weeks of cough, purulent sputum, and intermittent low-grade fever. He initially denied high-risk sexual exposure. Physical examination revealed no rash, chancre, or lymphadenopathy. Laboratory workup showed marked eosinophilia (15.70%), elevated C-reactive protein, and immunoglobulin E (1,290 IU/mL). Chest computed tomography demonstrated bilateral patchy infiltrates and multiple small upper-lobe nodules. While this presentation suggested eosinophilic granulomatosis with polyangiitis (EGPA), unexpected positive syphilis serology (rapid plasma reagin 1:128) redirected the diagnostic workup. Upon further confidential questioning, the patient acknowledged high-risk sexual exposures. Bronchoalveolar lavage fluid targeted next-generation sequencing (tNGS) detected a high load of Treponema pallidum (11,732 reads) alongside co-infecting respiratory pathogens. EGPA was rendered highly unlikely by complete clinical and radiological resolution following antimicrobial therapy alone, without corticosteroids. CONCLUSION: This case illustrates that non-rash secondary pulmonary syphilis can closely mimic EGPA, and that tNGS provides a valuable adjunctive tool for resolving such diagnostic challenges, thereby preventing potentially harmful immunosuppressive treatment. As syphilis continues to re-emerge globally, clinicians should maintain a high index of suspicion for syphilitic pulmonary involvement in patients with unexplained eosinophilia and pulmonary infiltrates, even in the absence of dermatological manifestations.

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: 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.

Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis) is a rare systemic vasculitis that predominantly affects small- to medium-sized vessels, with large-vessel involvement being exceptional. We report a 72-year-old woman with Churg-Strauss syndrome and severe low-flow, low-gradient aortic stenosis (left ventricular ejection fraction 15%) who underwent surgical aortic valve replacement following multidisciplinary Heart Team assessment. Intraoperatively, an unusual blue discolouration of the ascending aortic intima was observed. Postoperatively, the patient developed cardiac tamponade requiring urgent reintervention and had a prolonged recovery before being discharged to rehabilitation. This case expands the spectrum of vascular manifestations in Churg-Strauss syndrome and highlights potential perioperative challenges during cardiac surgery.