好酸球性多発血管炎性肉芽腫症

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, chronic disease that significantly affects quality of life (QoL). Despite improved survival, many patients experience persistent symptoms and complex care needs. Patient-reported data on QoL and perceived care quality remain limited, particularly in Italy. This study aimed to assess health-related QoL and patient-perceived care quality among Italian EGPA patients and to identify distinct patient profiles through cluster analysis to inform personalized, multidisciplinary care strategies. METHODOLOGY: We conducted a cross-sectional, 77-item online survey among adult EGPA patients (self-reported diagnosis) between December 2024 and January 2025. The survey, developed with APACS APS (Associazione Pazienti con Sindrome di Churg-Strauss), was distributed via the SurveyMonkey platform. It included validated instruments: SF-36 for health-related QoL and PACIC (with PACIC-5As) for perceptions of chronic care. Additional demographic, clinical, and disease impact data were collected. Descriptive statistics and group comparisons were performed. SF-36 and PACIC domains were analyzed using principal component analysis (PCA), followed by k-means clustering to identify patient subgroups. RESULTS: Seventy-two patients completed the survey (mean age 56; 65% female; median disease duration 7 years; 84.7% on biologics; 41.7% on glucocorticoids). SF-36 scores showed moderate QoL impairment (mean PCS 56.3 ± 24.6; MCS 59.3 ± 24.1), with lower QoL among females, divorced/separated individuals, and those with neurological involvement. Paresthesia had the greatest impact, affecting multiple QoL domains and daily functioning (p < 0.05). PACIC scores reflected moderate perceptions of care (2.8-3.5), with lowest scores in "Goal Setting" and "Care Coordination." PACIC-5As scores were low (mean 2.3), indicating suboptimal collaborative care. Cluster analysis identified three profiles: (1) poor QoL with high healthcare engagement (n = 12); (2) best QoL and care ratings (n = 38); (3) intermediate health status but lowest PACIC scores (n = 22), reflecting perceived lack of support. Differences across clusters were statistically significant (p < 0.001). CONCLUSIONS: Italian EGPA patients report impaired QoL and only moderate care quality, with notable heterogeneity in experiences. Personalized, multidisciplinary approaches are needed, especially for patients who feel under-supported despite moderate disease activity.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis characterized by eosinophilic infiltration and granuloma formation, affecting multiple organs. Gastrointestinal (GI) involvement is relatively uncommon and it typically presents with nonspecific symptoms, such as abdominal pain or diarrhea; in contrast, ulceration and intestinal perforation are rare, but potentially life-threatening complications that often require surgical intervention. The standard treatment for severe GI EGPA includes high-dose glucocorticoids combined with cyclophosphamide or rituximab (RTX). However, the perioperative escalation of glucocorticoids is generally avoided owing to the increased risk of postoperative complications. We report a case with a 5-year follow-up of EGPA resistant to multiple immunosuppressive agents with severe GI involvement, including intestinal perforation and multiple jejunal ulcers, which was successfully treated with RTX for postoperative remission induction and long-term maintenance therapy without any prednisolone escalation. The prednisolone dose was gradually reduced from 17.5 mg/day to 1 mg/day over two years without any escalation, and a sustained remission was achieved throughout the course. This case suggests that RTX may represent a viable therapeutic option for severe or treatment-resistant EGPA in cases with GI involvement when glucocorticoid escalation is undesirable or unsafe, such as in the perioperative setting.

Ear-nose-throat (ENT) involvement is common in ANCA associated vasculitis, particularly in granulomatosis with polyangiitis (GPA) and EGPA eosinophilic granulomatosis with polyangiitis (EGPA), with sinonasal manifestations frequently representing the earliest clinical features of disease (1).

Ear, nose and throat (ENT) involvement is a frequent and early manifestation of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). This study aimed to comprehensively evaluate and compare ENT features in patients with granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA). In this cross-sectional study, 50 consecutive patients with GPA (n = 35) and EGPA (n = 15) were recruited from Rheumatology and ENT clinics at King Saud University Medical City, Riyadh, Saudi Arabia. Patients underwent clinical, audiological, imaging, and laboratory assessments. ENT evaluation included otologic, sinonasal, and laryngeal examinations, supplemented with standardized questionnaires and audiometry. Upper airway involvement was highly prevalent (90%) in both groups. Pulmonary involvement was more frequent in EGPA, whereas renal involvement did not differ significantly between the groups. Hearing loss, including subclinical sensorineural forms, occurred in 50% of patients and was associated with older age at diagnosis (≥ 34 years) and longer disease duration (median 8 vs. 1.5 years, p = 0.01). Otologic involvement did not differ between GPA and EGPA. Sinonasal manifestations varied: nasal edema, allergic rhinitis, and polyposis were more frequent in EGPA, whereas destructive lesions, including saddle-nose deformity, occurred only in GPA. Laryngeal involvement was present in 46%; subglottic stenosis occurred only in GPA (14%) and was absent in EGPA. ENT involvement is common in both GPA and EGPA, with distinct sinonasal and laryngeal patterns. Hearing loss is frequent and often subclinical, highlighting the importance of audiometric and tailored ENT assessments. Further prospective studies are needed to determine the value of routine audiometric screening.