Eosinophilic granulomatosis with polyangiitis (EGPA), a rare autoimmune disease affecting multiple systems, is prone to misdiagnosis and missed diagnosis due to its diverse and complex clinical manifestations, which a...
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Eosinophilic granulomatosis with polyangiitis (EGPA), a rare autoimmune disease affecting multiple systems, is prone to misdiagnosis and missed diagnosis due to its diverse and complex clinical manifestations, which affect the prognosis. Therefore, early diagnosis of the disease, development of standardized treatment protocols, management of complications, and multidisciplinary team collaboration are crucial. The writing group of the Multidisciplinary Expert Consensus on the Diagnosis and Treatment of Eosinophilic Granulomatosis with Polyangiitis, has comprehensively updated and revised the 2018 edition of the “Multidisciplinary Expert Consensus on the Diagnosis and Treatment of Eosinophilic Granulomatosis with Polyangiitis” based on the latest research findings. The revision adds etiology and pathogenesis, updates diagnosis and assessment, treatment, and prognosis, and compiles 13 recommendations. This revision aims to improve the diagnostic and therapeutic capabilities of clinicians for EGPA, highlight the importance of multidisciplinary collaboration in EGPA management, provide the most up-to-date guidance for clinical practice, and consequently improve treatment outcomes and patients′ quality of life. The recommendations are listed below. Recommendation 1: Patients with asthma, peripheral blood eosinophilia, and damage to other systems should be suspected of having EGPA (1, B). Recommendation 2: Patients suspected of having EGPA should undergo a full examination and assessment. ANCA testing should be performed in all such patients. If feasible, biopsy of the affected tissue is also recommended (1, B). Recommendation 3: The diagnosis of EGPA should be based on highly suggestive indicative clinical features, laboratory tests, imaging, and objective evidence of vasculitis. Differential diagnosis and multidisciplinary assessment are essential. The 1990 ACR or 2022 ACR/EULAR classification criteria for EGPA are recommended for diagnosis (1, B). Recommendation 4: All
目的本研究旨在探讨涎液化糖链抗原-6(krebs von den lungen-6,KL-6)在特发性肺含铁血黄素沉着症(idiopathic pulmonary hemosiderosis,IPH)患儿辅助诊断中的临床应用价值。方法收集2014年6月至2024年7月期间于广州医科大学附属第一医...
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目的本研究旨在探讨涎液化糖链抗原-6(krebs von den lungen-6,KL-6)在特发性肺含铁血黄素沉着症(idiopathic pulmonary hemosiderosis,IPH)患儿辅助诊断中的临床应用价值。方法收集2014年6月至2024年7月期间于广州医科大学附属第一医院就诊的140例患儿,分为病例组与对照组,其中病例组根据疾病类型细分为IPH组(32例)、间质性肺炎(interstitial lung disease,ILD)组(22例)、肺炎(pneumonia,PN)组(60例),对照组为非肺部疾病(non-pulmonary disease,NPD)组(26例)。以上患儿检测血清KL-6水平,分析KL-6在各组患儿中的表达差异。结果KL-6阳性率在各组患儿中从高到低分别为IPH(68.75%)、ILD(45.45%)、PN(1.69%)和NPD(0.00%),组间阳性率差异有统计学意义(χ^(2)=66.10,P<0.001)。IPH组患儿血清KL-6平均水平高于PN组患儿(Ζ=-6.92,P<0.001)。诊断试验结果显示ROC曲线下面积为0.940(95%CI:0.89~1.00,P<0.001),截断值为392.00 U/mL,灵敏度为81.30%,特异度为95.00%。结论KL-6在鉴别IPH患儿与PN和NPD患儿中具有较高的诊断价值,可作为IPH辅助诊断的血液生物标志物。
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