2026-07-06 | Microscopic Polyangiitis With Temporal Artery Involvement Mimicking Giant Cell Arteritis.
A 63-year-old woman presented with a four-month history of bilateral hearing loss and three months of progressive, painful peripheral neuropathy. Her clinical course was complicated by deep vein thrombosis with pulmonary emboli and an incomplete response to initial empiric glucocorticoid therapy. Following glucocorticoid taper, she developed temporal headaches, jaw claudication, and scalp tenderness, raising concern for giant cell arteritis (GCA). Laboratory evaluation revealed markedly elevated inflammatory markers (erythrocyte sedimentation rate and C-reactive protein), microcytic anemia, mild hematuria, and strongly positive myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) by antigen-specific immunoassay with negative proteinase-3 (PR3)-ANCA. Temporal artery biopsy demonstrated transmural inflammation affecting all vessel layers without giant cells or granulomata. The combination of multisystem small-vessel involvement, MPO-ANCA seropositivity with negative PR3-ANCA, and characteristic histopathological findings established the diagnosis of microscopic polyangiitis (MPA) with temporal arteritis rather than granulomatosis with polyangiitis (GPA) or GCA. Treatment with high-dose glucocorticoids (prednisone 80 mg daily), avacopan (30 mg twice daily), and rituximab (375 mg/m² weekly for four weeks) resulted in rapid improvement of constitutional and cranial symptoms. This case highlights the importance of integrating MPO-ANCA serology and histopathological findings when temporal arteritis is the presenting feature of systemic vasculitis. It illustrates the utility of avacopan-based, glucocorticoid-sparing combination immunosuppression in this context.
Open article ↗
2026-06-25 | No benefit of adding glucocorticoids to maintenance treatment in reducing the risk of major relapses in ANCA-associated vasculitis: real life data from a longitudinal cohort study.
To examine the role of low-dose glucocorticoids (GCs) in major relapse, hospitalisation and damage accumulation risk during maintenance therapy in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAVs). Retrospective cohort study of newly diagnosed patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) followed in three tertiary referral centres. We recorded relapses (Birmingham Vasculitis Activity Score increase >0), increases in Vasculitis Damage Index (VDI) and hospitalisations. We used time-varying and mixed-effects Cox models to examine the effect of GCs on the risk of major relapses, VDI accumulation and hospitalisations. Sensitivity analysis was also conducted to address potential confounding. 171 patients (GPA: 107, MPA: 64, median age: 61 years) were included with a median follow-up of 88.2 months (803.4 patient years (PY)). We recorded 65 major relapses (8.1/100 PY) in 48 patients (28%), 65 events of new damage (8.1/100 PY) and 132 hospitalisations (16.4/100 PY). By multivariable analysis, rituximab use was associated with a lower risk (HR=0.21, 95% CI 0.09 to 0.47, p=0.0001) while disease activity at diagnosis was associated with an increased risk (HR=1.17, 95% CI 1.03 to 1.33, p=0.013) of major relapses. Low-dose GCs were not associated with a reduced major relapse risk (HR=0.96, 95% CI 0.88 to 1.05, p=0.36) but they were associated with a risk of damage accumulation (HR=1.52, 95% CI 1.19 to 1.93, p=0.0007) and hospitalisations (HR=1.24, 95% CI 1.06 to 1.45, p=0.006). Higher GC exposure during maintenance therapy was not significantly associated with a lower major relapse risk whereas it was associated with damage accrual and hospitalisation. These findings may support decision making regarding long-term GC use in patients with GPA/MPA.
Open article ↗
2026-06-22 | Laryngeal manifestations of microscopic polyangiitis in a pediatric patient: a case report
Microscopic polyangiitis is a rare small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies.Otorhinolaryngological manifestations are uncommon, particularly in pediatric patients, which may delay diagnosis and appropriate treatment.We report the case of a 16 year-old female with a history of Graves disease on methimazole who presented to the emergency department with nonspecific otorhinolaryngological symptoms, followed by rapid clinical deterioration that required advanced resuscitation and admission to the pediatric intensive care unit.Clinical evaluation revealed epiglottic edema, yellowish lesions on the oropharyngeal mucosa, and right vocal fold paralysis.Laboratory findings demonstrated positive anti-myeloperoxidase antibodies, confirming the diagnosis of microscopic polyangiitis.The patient received two cycles of rituximab with marked clinical improvement and preserved vocal fold mobility.This case underscores the diagnostic challenge of microscopic polyangiitis with initial laryngeal involvement in a pediatric patient and highlights the importance of close observation and an interdisciplinary approach.
Open article ↗
