Binaghi M, Kamoun M M, Coscas K G
J Fr Ophtalmol. 1984;7(1):19-30.
Periarteritis Nodosa (P.A.N.) is a systemic connective tissue disease with a variety of manifestations that includes ocular involvement in 20% of cases. The diagnosis of this condition is difficult due to the absence of any specific clinical signs or laboratory findings. However, histologic studies have demonstrated a segmental vasculitis that is often necrotic. Ocular findings frequently include choroidal involvement that is characteristic. Nevertheless, angiographic studies of this disease are extremely rare. The findings in three patients suspected of having P.A.N. are presented. Fluorescein angiography established the diagnosis of P.A.N. in two cases and ruled-out its presence in the third case. In the first case angiography demonstrated a retinal vasculitis with multiple arteriolar and capillary occlusions. There was also ischemic involvement of the choriocapillaris and a mild anterior optic nerve vasculitis. All findings resolved, leaving numerous Elschnig spots. In the second case the angiogram showed acute multifocal ischemia of the choriocapillaris. The ocular examination and fluorescein angiogram in the third case were entirely normal, thereby ruling-out P.A.N. on the basis of insufficient criteria. Acute multifocal choroidal ischemia is present in a variety of rare conditions: Toxemia of pregnancy, Disseminated Intravascular coagulopathy, Moskowitz Disease (T.T.P.), Leukemia and Malignant Hypertension. However, the presence of multifocal choroidal ischemia in the presence of a systemic connective tissue disorder strongly favors the diagnosis of P.A.N. The relative contributions of co-existent Malignant Hypertension and P.A.N. in producing choroidal ischemia are discussed. The spectrum of clinical manifestations and laboratory findings in P.A.N. as well as hypotheses concerning pathogenesis (immune-complex deposition) are described. Among all systemic vasculitis , only P.A.N., and rarely Scleroderma, feature choroidal involvement. This is possibly due to the fact that the degree of vasculitis in P.A.N. is sufficiently severe to cause clinically significant choroidal involvement.
结节性多动脉炎（P.A.N.）是一种具有多种表现的系统性结缔组织疾病，20%的病例会累及眼部。由于缺乏任何特异性临床体征或实验室检查结果，该病的诊断较为困难。然而，组织学研究显示其为节段性血管炎，常呈坏死性。眼部表现常包括具有特征性的脉络膜受累。尽管如此，关于该疾病的血管造影研究极为罕见。本文报告了3例疑似结节性多动脉炎患者的检查结果。荧光素血管造影在2例中确诊了结节性多动脉炎，在第3例中排除了该病。第1例血管造影显示视网膜血管炎伴多发小动脉和毛细血管阻塞。脉络膜毛细血管也有缺血性改变，且存在轻度前部视神经血管炎。所有病变均消退，留下大量Elschnig斑。第2例血管造影显示脉络膜毛细血管急性多灶性缺血。第3例的眼部检查和荧光素血管造影完全正常，因此根据诊断标准不足排除了结节性多动脉炎。急性多灶性脉络膜缺血可见于多种罕见情况：妊娠中毒症、弥散性血管内凝血、Moskowitz病（血栓性血小板减少性紫癜）、白血病和恶性高血压。然而，在系统性结缔组织疾病存在的情况下出现多灶性脉络膜缺血强烈提示结节性多动脉炎的诊断。文中讨论了并存的恶性高血压和结节性多动脉炎在导致脉络膜缺血中的相对作用。描述了结节性多动脉炎的临床表现谱和实验室检查结果以及关于发病机制（免疫复合物沉积）的假说。在所有系统性血管炎中，只有结节性多动脉炎以及很少见的硬皮病会累及脉络膜。这可能是因为结节性多动脉炎的血管炎程度严重到足以引起具有临床意义的脉络膜受累。
