Taormina Giuseppe, Andolina Giuseppe, Banco Maria Aurelia, Costanza-Gaglio Edy Julia, Bonura Alice, Buscemi Silvio
Internal Medicine and Hypertension Unit - Dipartimento Biomedico di Medicina Interna e Specialistica (DIBIMIS), P, Giaccone Policlinico of the University of Palermo, Via del Vespro 129, 90127 Palermo, Italy.
J Med Case Rep. 2014 Jun 13;8:190. doi: 10.1186/1752-1947-8-190.
Eosinophilic granulomatosis with polyangiitis is a rare and potentially fatal disease if not readily diagnosed. Cerebral involvement is extremely rare and clinical presentation as hemorrhagic stroke is even rarer.
A 58-year-old Caucasian man was admitted to our medical unit because of a computed tomography-diagnosed hemorrhagic stroke with right-sided hemiparesis and fever. A chest computed tomography scan also revealed multiple bilateral pulmonary infiltrates; coronary artery, and carotid and left vertebral artery calcifications were also observed. Empiric antimicrobial therapy with cephalosporins was promptly undertaken; low-molecular-weight heparin was introduced as prophylaxis for venous thromboembolism. Over the following days, magnetic resonance imaging scans showed a regression of the hemorrhagic framework, also revealing hypoxic areas consistent with acute ischemic lesions. With a computed tomography scan showing a worsening of his pulmonary framework, antimicrobial therapy was modified and corticosteroids were introduced. A new blood cell count revealed further increased leukocytosis (17.49 × 10(3) μL), characterized by a surprising rise of eosinophilic cells (32.8%). Angiography of the coronary arteries found diffuse dilatations with severe signs of endothelial damage. Such an unexpected framework induced a strong suspicion that the stroke was the expression of a systemic vasculitis, which had triggered his cerebral, coronary, and pulmonary frameworks. The search for antineutrophil cytoplasmic antibody was positive for perinuclear antineutrophil cytoplasmic antibody, and eosinophilic granulomatosis with polyangiitis was diagnosed. Explaining to the patient the rarity of his disease, and what the most typical presentations of eosinophilic granulomatosis with polyangiitis were, he revealed that before admission he had had scalp injuries, in the nuchal region, and had taken corticosteroids as self-medication, with subsequent disappearance of the lesions. Therefore, high-dose corticosteroid treatment was started, and at discharge he was in good clinical condition with a slight right-sided hyposthenia.
A diagnosis of eosinophilic granulomatosis with polyangiitis is often difficult, but we are convinced that intake of corticosteroids on a self-prescribed basis may have obscured the clinical presentation. Therefore, this case also suggests how the growing phenomenon of self-medication can be harmful, and that a careful investigation of clinical history is still an act of paramount importance.
嗜酸性肉芽肿性多血管炎是一种罕见疾病，若未及时诊断，可能会致命。脑部受累极为罕见，以出血性中风为临床表现的情况则更为罕见。
一名58岁的白种男性因计算机断层扫描诊断为出血性中风伴右侧偏瘫和发热入住我院内科。胸部计算机断层扫描还显示双侧肺部有多处浸润；同时观察到冠状动脉、颈动脉和左椎动脉钙化。立即开始使用头孢菌素进行经验性抗菌治疗；引入低分子量肝素以预防静脉血栓栓塞。在接下来的几天里，磁共振成像扫描显示出血灶有所消退，同时也发现了与急性缺血性病变相符的缺氧区域。计算机断层扫描显示其肺部情况恶化，于是调整了抗菌治疗并引入了皮质类固醇。新的血细胞计数显示白细胞增多进一步加重（17.49×10³/μL），其特征是嗜酸性细胞惊人地升高（32.8%）。冠状动脉血管造影发现弥漫性扩张，并伴有严重的内皮损伤迹象。这种意外情况强烈怀疑中风是系统性血管炎的表现，该血管炎引发了他的脑部、冠状动脉和肺部病变。抗中性粒细胞胞浆抗体检测显示核周抗中性粒细胞胞浆抗体呈阳性，诊断为嗜酸性肉芽肿性多血管炎。在向患者解释其疾病的罕见性以及嗜酸性肉芽肿性多血管炎最典型的表现后，他透露入院前颈部头皮有损伤，并自行服用了皮质类固醇，随后损伤消失。因此，开始了高剂量皮质类固醇治疗，出院时他临床状况良好，右侧轻度肌力减退。
嗜酸性肉芽肿性多血管炎的诊断通常很困难，但我们确信自行服用皮质类固醇可能掩盖了临床表现。因此，该病例也表明自行用药这一日益普遍的现象可能有害，仔细调查临床病史仍然至关重要。
