Damian Laura Otilia, Manole Simona, Pamfil Cristina Ana Maria, Rogojan Liliana, Rednic Simona, Maniu Alma Aurelia, Poenaru Mărioara
Department of Otorhinolaryngology, "Iuliu Haţieganu" University of Medicine and Pharmacy, Cluj-Napoca, Romania;
Rom J Morphol Embryol. 2018;59(2):595-599.
Giant cell arteritis (GCA) is a large-vessel vasculitis with rarely described respiratory initial manifestations. We report such a case presenting with hoarseness, stridor, cough and dyspnea, in which a tracheomegaly with tracheomalacia was found. No signs of relapsing polychondritis were present. The respiratory symptoms rapidly improved after glucocorticoids and Azathioprine. Tracheomegaly or Mounier-Kuhn syndrome is characterized by marked dilatation of trachea and central bronchi. The differential diagnosis and the possible relationship between tracheomegaly and GCA involving metalloproteinase-induced elastolysis are discussed. This is the first case, to our knowledge, of Mounier-Kuhn syndrome in vasculitis. The association of tracheomegaly with GCA may be underestimated, as the diagnosis is not always obvious on conventional radiographs. A tracheal enlargement finding in GCA requires monitoring to ensure early detection and prevention of spontaneous tracheal rupture. Adding a metalloproteinase inhibitor like Doxycycline to GCA therapy would be rational for the prophylaxis of complications.
巨细胞动脉炎（GCA）是一种大血管血管炎，其呼吸道初始表现鲜有描述。我们报告了这样一例以声音嘶哑、喘鸣、咳嗽和呼吸困难为表现的病例，该病例发现有气管扩张伴气管软化。无复发性多软骨炎的迹象。使用糖皮质激素和硫唑嘌呤治疗后，呼吸道症状迅速改善。气管扩张或穆尼耶 - 库恩综合征的特征是气管和中央支气管明显扩张。讨论了鉴别诊断以及气管扩张与涉及金属蛋白酶诱导的弹性蛋白溶解的GCA之间的可能关系。据我们所知，这是血管炎中首例穆尼耶 - 库恩综合征病例。气管扩张与GCA的关联可能被低估，因为在传统X线片上诊断并不总是明显。GCA中发现气管增大需要进行监测，以确保早期发现并预防自发性气管破裂。在GCA治疗中添加如多西环素这样的金属蛋白酶抑制剂对预防并发症是合理的。
