Gallagher K T, Shaham B, Reiff A, Tournay A, Villablanca J P, Curran J, Nelson M D, Bernstein B, Rawlings D J
Department of Rheumatology, Children's Hospital Los Angeles, California, USA.
J Rheumatol. 2001 Mar;28(3):616-23.
We describe 5 children who meet criteria for primary angiitis of the central nervous system (PACNS). All patients presented with headache and/or focal neurologic deficits and exhibited clinical and/or radiographic evidence of disease progression. Two patients had disease progression prior to combined treatment with cyclophosphamide and corticosteroids; one progressed while receiving intravenous cyclophosphamide and stabilized after a change to daily oral dosing; one progressed after discontinuing therapy after less than 12 months and improved after retreatment; and one progressed on steroid therapy alone but was lost to followup. Children who have frequent or severe headaches or focal neurologic deficits should be carefully evaluated and those meeting criteria for PACNS should be treated aggressively.
我们描述了5名符合中枢神经系统原发性血管炎（PACNS）标准的儿童。所有患者均表现为头痛和/或局灶性神经功能缺损，并呈现出疾病进展的临床和/或影像学证据。2例患者在接受环磷酰胺和皮质类固醇联合治疗前病情进展；1例在接受静脉注射环磷酰胺时病情进展，改为每日口服给药后病情稳定；1例在治疗不到12个月后停药病情进展，再次治疗后病情改善；1例仅接受类固醇治疗病情进展，但失访。对于经常出现严重头痛或局灶性神经功能缺损的儿童，应进行仔细评估，符合PACNS标准的儿童应积极治疗。
