1st Neurology Department, Aristotle University, AHEPA Hospital, Thessaloniki, Greece.
1st Internal Medicine Department, Aristotle University, AHEPA Hospital, Thessaloniki, Greece.
Headache. 2018 Apr;58(4):589-595. doi: 10.1111/head.13277. Epub 2018 Feb 15.
The objective of this article is to report a rare case of headache as the initial symptom of granulomatosis with polyangiitis (GPA) and to review the recent literature.
Granulomatosis with polyangiitis is a rare, systemic, autoimmune disease of unknown etiology. GPA has a wide spectrum of clinical symptomatology, including involvement of the nervous system, even as the initial manifestation. Symptoms of the peripheral nervous system used to dominate the clinical symptomatology. However, recent reports are focusing increasingly in granulomatous lesions of the central nervous system, and especially on the increased frequency of patients with hypertrophic pachymeningitis (HP). We report the case of a patient with headache linked to intracranial hypertension and hypertrophic pachymeningitis as the initial and dominant presentation of GPA and we review the recent literature.
A 54-year-old male, without any related medical history developed a severe headache. In the following 2 months, he gradually developed hoarseness and diplopia at the left and lower fields of vision. A brain MRI revealed wide-spread fattening and meningeal enhancement over the left hemisphere and the left cerebellar hemisphere. An endoscopy of the pharynx revealed the presence of a tumor-like mass in the left half of the nasopharynx. A biopsy showed inflammation with presence of polykaryocyte Langhans giant cells. The laboratory testing revealed important albuminuria and microhematuria, positive c-ANCA and negative p-ANCA. A diagnosis of GPA was established.
A steroid treatment was administered initially, which improved the headache drastically, followed by the administration of a combination of cyclophosphamide and corticosteroid, which led to a gradual resolve of the remaining symptomatology. A follow-up brain MRI showed a decrease in meningeal enhancement, whereas a second one, 2 years later, was completely normal.
HP was considered an extremely rare manifestation of GPA. However, recent studies are reporting an increased frequency of HP and are distinguishing a granulomatous and a vasculitic phenotype, with different localization and relapse rates, that may eventually constitute a different clinical spectrum of GPA.
本文旨在报告一例以头痛为首发症状的肉芽肿性多血管炎（GPA）病例，并复习近期文献。
GPA 是一种罕见的、系统性的、病因不明的自身免疫性疾病。GPA 具有广泛的临床症状谱，包括神经系统受累，甚至作为首发表现。过去，外周神经系统的症状常主导临床症状谱。然而，近期的报告越来越关注中枢神经系统的肉芽肿性病变，特别是肥厚性硬脑膜炎（HP）的发病率增加。我们报告一例头痛伴颅内压增高和肥厚性硬脑膜炎的患者，其 GPA 的首发和主要表现，并复习近期文献。
一名 54 岁男性，无任何相关病史，出现严重头痛。在接下来的 2 个月中，他逐渐出现左侧及下视野的声音嘶哑和复视。脑部 MRI 显示左侧大脑半球和左侧小脑半球广泛脑膜增厚和强化。咽部内镜检查显示左侧鼻咽部有肿瘤样肿块。活检显示炎症伴多核朗格汉斯巨细胞。实验室检查显示大量白蛋白尿和镜下血尿，c-ANCA 阳性，p-ANCA 阴性。诊断为 GPA。
最初给予激素治疗，头痛明显改善，随后给予环磷酰胺和皮质类固醇联合治疗，症状逐渐缓解。随访脑 MRI 显示脑膜强化减轻，两年后复查完全正常。
HP 被认为是 GPA 极为罕见的表现。然而，近期研究报告 HP 的发病率增加，并区分出肉芽肿性和血管炎性表型，其定位和复发率不同，可能最终构成 GPA 的不同临床谱。
