Deng Shan, Xing Yida, Wang Hongjiang, Kong Xiaodan
Department of Rheumatology, The Second Affiliated Hospital, Dalian Medical University, Dalian 116023, China.
Medicine (Baltimore). 2019 Jan;98(3):e14189. doi: 10.1097/MD.0000000000014189.
The precise cause of granulomatosis with polyangiitis (GPA) remains unclear. Herein we present a case of refractory GPA occurring after trauma.
A 43-year-old man suffered fractures of the left orbital in an accident, and then received surgical repair. At a postoperative follow-up, he developed maxillary sinusitis. Seven months later, he was hospitalized with symptoms of cough, high-grade fever, and loss of weight.
He was diagnosed with GPA based on the symptoms of upper and lower respiratory tract involvement, granulomas in a lung biopsy specimen, and presence of PR3-ANCA.
Initially the patient responded well to the treatment of glucocorticoids and cyclophosphamide (CYC). Forty days later, he was hospitalized again with symptoms of fever, cephalgia, and recurrent ulcerated subcutaneous nodules, due to the vasculitic manifestations of GPA. This time he was treated with cyclophosphamide and glucocorticoids with no improvement, and then switched to rituximab.
After 4 doses of rituximab, the symptom of cephalgia disappeared and subcutaneous ulcerations and conjunctival congestion diminished.
Trauma may act as an inflammatory stimulus to affect the course of disease in GPA. The combination of glucocorticoids and cyclophosphamide is the standard therapy for GPA. Nevertheless, patients who have no response to CYC, especially with predominantly vasculitic manifestations, could resort to rituximab.
肉芽肿性多血管炎(GPA)的确切病因尚不清楚。在此我们报告一例创伤后发生的难治性GPA病例。
一名43岁男性在一次事故中左侧眼眶骨折,随后接受了手术修复。术后随访时,他患上了上颌窦炎。七个月后,他因咳嗽、高热和体重减轻症状入院。
根据上下呼吸道受累症状、肺活检标本中的肉芽肿以及PR3-ANCA的存在,他被诊断为GPA。
最初患者对糖皮质激素和环磷酰胺(CYC)治疗反应良好。40天后,由于GPA的血管炎表现,他再次因发热、头痛和复发性皮下结节溃疡症状入院。这次他接受了环磷酰胺和糖皮质激素治疗但无改善,随后改用利妥昔单抗。
在使用4剂利妥昔单抗后,头痛症状消失,皮下溃疡和结膜充血减轻。
创伤可能作为一种炎症刺激影响GPA的病程。糖皮质激素和环磷酰胺联合是GPA的标准治疗方法。然而,对CYC无反应的患者,尤其是以血管炎表现为主的患者,可采用利妥昔单抗治疗。