Armengot M, García-Lliberós A, Gómez M J, Navarro A, Martorell A
Department of Pathology, General and University Hospital, Ear, Nose, and Throat and Medical School, and Departments of Surgery and Pathology, Valencia University, Valencia, Spain.
Allergy Rhinol (Providence). 2013 Summer;4(2):e94-9. doi: 10.2500/ar.2013.4.0051.
Multiple systemic diseases produce various clinical manifestations in the sinonasal area. They usually appear as difficult-to-diagnose disease processes with slow, atypical clinical courses. The aim of this study was to evaluate the sinonasal manifestations of systemic vasculitides, highlighting key points for diagnosis and differential diagnosis with other pathological entities, especially cocaine-induced midline destructive lesions (CIMDL). A retrospective study was performed of 10 patients treated in our hospital during the last 5 years with an initial diagnosis of systemic vasculitides with sinonasal involvement: eight patients with granulomatosis with polyangiitis (GPA; new nomenclature for Wegener granulomatosis) and two patients with Churg-Strauss syndrome (CSS). The study variables were clinical presentation, nasal endoscopy results, maxillofacial scan results, nasal biopsy results, erythrocyte sedimentation rate, and autoimmune antibody levels. The definitive diagnosis was GPA in six (60%) patients, CSS in two (20%) patients, and CIMDL in two (20%) patients. Nasal symptoms were similar in all patients, but nasal polyps were present in only one patient with CSS. Systemic manifestations were absent in patients with CIMDL. Likewise, peripheral eosinophilia was observed only in the two patients with CSS. Specific positive biopsy specimens were obtained in six patients (all six patients with GPA, one with CSS, and one with CIMDL). Antineutrophil cytoplasmic antibodies (ANCA) were positive in all patients with GPA (proteinase 3 antigen in five patients and myeloperoxidase in one patient), and perinuclear ANCA was positive in one patient with CIMDL; however, this patient showed an undefined pattern. Finally, the response to treatment was adequate in all patients excluding those with CIMDL. GPA and CIMDL syndromes pose a difficult differential diagnosis because they have common clinical, serological, and histological presentations. Negative histological results do not exclude the diagnosis of sinonasal vasculitides. The absence of systemic manifestations and the lack of response to treatment will lead to the confirmation of CIMDL syndrome in a cocaine user. Otolaryngologists play an important role in the early and differential diagnosis of these diseases.
多种全身性疾病可在鼻窦区域产生各种临床表现。它们通常表现为诊断困难的疾病过程，临床病程缓慢且不典型。本研究的目的是评估系统性血管炎的鼻窦表现，强调诊断要点以及与其他病理实体（尤其是可卡因所致中线破坏性病变，CIMDL）的鉴别诊断。对我院过去5年中最初诊断为累及鼻窦的系统性血管炎的10例患者进行了一项回顾性研究：8例肉芽肿性多血管炎（GPA；韦格纳肉芽肿的新命名）患者和2例变应性肉芽肿性血管炎（CSS）患者。研究变量包括临床表现、鼻内镜检查结果、颌面扫描结果、鼻活检结果、红细胞沉降率和自身免疫抗体水平。最终诊断为GPA的患者有6例（60%），CSS的患者有2例（20%），CIMDL的患者有2例（20%）。所有患者的鼻部症状相似，但仅1例CSS患者有鼻息肉。CIMDL患者无全身表现。同样，仅2例CSS患者观察到外周血嗜酸性粒细胞增多。6例患者获得了特异性阳性活检标本（6例GPA患者、1例CSS患者和1例CIMDL患者）。所有GPA患者的抗中性粒细胞胞浆抗体（ANCA）均为阳性（5例患者为蛋白酶3抗原阳性，1例患者为髓过氧化物酶阳性），1例CIMDL患者的核周型ANCA阳性；然而，该患者表现为不明确的模式。最后，除CIMDL患者外，所有患者对治疗的反应均良好。GPA和CIMDL综合征的鉴别诊断困难，因为它们具有共同的临床、血清学和组织学表现。组织学结果阴性不能排除鼻窦血管炎的诊断。无全身表现且对治疗无反应将导致确诊可卡因使用者的CIMDL综合征。耳鼻喉科医生在这些疾病的早期诊断和鉴别诊断中发挥着重要作用。
