多発血管炎性肉芽腫症

Eosinophilic granulomatosis with polyangiitis (EGPA) and hypereosinophilic syndrome (HES) share overlapping clinical features, making early distinction difficult, especially in antineutrophil cytoplasmic antibody (ANCA)-negative disease where eosinophil-mediated injury may precede vasculitis. We report a male patient in his mid-20s who initially presented with fever, purpuric rash, neuropathy, marked eosinophilia, hepatic dysfunction and extensive multisystem thrombosis. Biopsy revealed eosinophilic infiltration without vasculitis, supporting a diagnosis of HES. He achieved remission on corticosteroids and anticoagulation but relapsed 5 years later with recurrent thrombosis, purpura, eosinophilia and biopsy-proven eosinophilic vasculitis, prompting reclassification as ANCA-negative EGPA. Treatment with corticosteroids, rituximab and anticoagulation resulted in sustained remission over 36 months. This case highlights the diagnostic continuum between HES and EGPA and the need for long-term follow-up with reassessment of diagnosis. Early recognition of evolving vasculitis is essential to guide appropriate immunosuppressive therapy.

BACKGROUND: Eosinophilic myocarditis (EM) is a rare form of myocarditis which may be caused by eosinophilic granulomatosis with polyangiitis (EGPA), a rare disease which frequently affects the heart. The case of a young woman with a rare initial manifestation of EGPA as a cause of EM is reported. CASE SUMMARY: A 39-year-old woman presented with diffuse neurological symptoms. She was diagnosed with multiple, bilateral, simultaneous ischaemic strokes. No obvious stroke origin was detectable. However, significantly elevated troponin levels and eosinophilia were noted. Cardiac computed tomography ruled out coronary artery disease. Cardiac magnetic resonance imaging (CMR) proved myocarditis. However, endomyocardial biopsy, did not confirm EM. Nevertheless, EGPA with primary cardiac involvement was diagnosed due to eosinophilia, history of asthma, evidence of pANCA and matching CMR findings. Treatment with benralizumab was initiated. Follow-up CMR after about 6 months showed a significant reduction in inflammatory activity. DISCUSSION: Eosinophilic granulomatosis with polyangiitis and EM associated with EGPA are rare but in patients with EGPA, cardiac involvement is frequent. Ischaemic strokes as an initial manifestation of EGPA is particularly rare. Other potential causes of stroke were ruled out. In the presence of CMR-proven myocarditis associated with peripheral eosinophilia, EM may be diagnosed without histological confirmation. Endomyocardial biopsy may be false negative in a relevant number of cases but CMR is usually abnormal. However, there are no specific CMR findings that occur exclusively in EM.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disease characterized by inflammation of small and medium-sized blood vessels, leading to damage in various organs. It is classically associated with asthma, eosinophilia, and multisystem involvement, including frequent peripheral nervous system manifestations. We report the case of a 72-year-old woman with a history of asthma, rhinitis, and strokes, who presented with progressive muscle weakness, myalgia, and distal sensory deficits. Neurological examination revealed proximal muscle weakness and a length-dependent sensory loss. Laboratory investigations demonstrated marked eosinophilia, elevated creatine kinase, and positive antimyeloperoxidase antibodies with an atypical cytoplasmic ANCA pattern. Imaging showed diffuse muscular hypermetabolism on positron emission tomography, while electromyography and nerve conduction studies revealed a length-dependent axonal polyneuropathy with mild myopathic features. Muscle biopsy demonstrated mild nonspecific myopathic changes without definite evidence of vasculitis or eosinophilic infiltrates. Based on the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria, a diagnosis of EGPA with neurological involvement was established. The patient was treated with high-dose corticosteroids and rituximab, resulting in significant clinical and biochemical improvement at 3 months. This case illustrates the coexistence of atypical neurological manifestations of EGPA, including prominent myopathy, length-dependent polyneuropathy, and retrospectively suspected central nervous system vasculitic involvement, emphasizing the importance of early recognition and multidisciplinary management.

Severe eosinophilic asthma (SEA) is a type-2 inflammatory phenotype driven by persistent eosinophilia, characterized by frequent exacerbations, progressive lung function decline, and substantial oral corticosteroid (OCS) dependence, for which conventional therapies remain insufficient in a significant minority of patients. Benralizumab-a humanized, afucosylated anti-interleukin-5 receptor alpha (IL-5Rα) monoclonal antibody-achieves near-complete depletion of eosinophils and basophils through dual receptor blockade and afucosylation-enhanced antibody-dependent cell-mediated cytotoxicity (ADCC), producing one of the most extensive anti-eosinophilic effects among currently approved biologics. Pivotal Phase 3 trials (SIROCCO, CALIMA, ZONDA) demonstrated annual exacerbation rate reductions of up to 51%, robust OCS-sparing with complete elimination in 52.7% of OCS-dependent patients, and significant improvements in forced expiratory volume in 1 second (FEV1). Long-term extension data over five years (MELTEMI) confirmed sustained efficacy and safety, without evidence of adverse consequences attributable to chronic eosinophil depletion. Landmark studies including PONENTE, SHAMAL, and ABRA have further extended benralizumab's clinical impact: enabling structured OCS elimination, demonstrating that well-controlled patients can reduce inhaled corticosteroids to as-needed therapy, and establishing biologic-based treatment of acute eosinophilic exacerbations as a viable new approach. Real-world evidence consistently corroborates trial findings, with clinical remission-defined as the composite achievement of exacerbation freedom, OCS independence, symptom control, and preserved lung function-reported in 17-44% of patients at 12 months and up to 91% in selected longer-term cohorts. Beyond asthma, benralizumab demonstrates meaningful activity in comorbid chronic rhinosinusitis with nasal polyps, eosinophilic granulomatosis with polyangiitis, and eosinophilic esophagitis. This review comprehensively examines benralizumab's molecular pharmacology, pivotal and real-world clinical evidence, comorbidity management, and its central role in driving the paradigm shift from symptom control toward clinical remission in severe eosinophilic airway disease.

Encephaloceles are uncommon lesions, most of which are congenital, and may be overlooked in routine radiologic practice. Failure to identify a skull base defect and characterize the herniated contents may delay recognition of associated abnormalities and clinically important complications, including cerebrospinal fluid leakage, intracranial infection, intracranial hypotension, hydrocephalus, and seizures. Granulomatosis with polyangiitis can cause destructive sinonasal disease with extension to the skull base; however, a meningoencephalocele in this setting is exceptionally rare. We report a 58-year-old woman with longstanding granulomatosis with polyangiitis who presented with new-onset seizures. Computed tomography and magnetic resonance imaging demonstrated a right transethmoidal meningoencephalocele through an ethmoidal roof defect contiguous with extensive destructive sinonasal changes, with encephalomalacia and gliosis of the herniated brain parenchyma.