Retinal and Inflammatory Eye Diseases, Centre for Ophthalmic Specialized Care (COS), 1003 Lausanne, Switzerland.
Medicina (Kaunas). 2022 Jan 21;58(2):165. doi: 10.3390/medicina58020165.
Primary inflammatory choriocapillaropathies (PICCPs) belong to a group of intraocular inflammatory diseases with the common characteristic of inflammatory choriocapillaris hypo- or non-perfusion as the main clinicopathological mechanism. The purpose of our article is to describe clinical characteristics and multimodal imaging, that can help the diagnosis and treatment of PICCPs.
Narrative review with multimodal imaging analysis.
Choriocapillaris non-perfusion can affect the end-choriocappilaries, at the benign end of the PICCP spectrum (MEWDS), to larger choriocapillaris vessels or precapillary vessels at the origin of more severe forms such as acute posterior multifocal placoid pigment epitheliopathy (APMPPE), idiopathic multifocal choroiditis (MFC) and Serpiginous Choroiditis (SC). Diagnosis is mostly based on multimodal imaging and especially on indocyanine green angiography (ICGA), fundus autofluorescence (FAF) and spectral-domain optical coherence tomography (SD-OCT)/OCT-angiography (OCT-A). ICGA shows the typical pattern of patchy lobular hypofluorescence reflecting hypo- or non-perfusion of the choriocapillaris that can also take the aspect of geographic areas in the more severe forms. Treatment depends on the severity of the disease and goes from observation in MEWDS and some mild cases of APMPPE, to oral corticosteroid and/or immunomodulator agents in the more severe conditions of APMPPE and MFC and SC cases. Close multimodal monitoring is crucial in order to introduce or adjust treatment.
PICCPs are resulting from one common clinicopathological mechanism, inflammatory choriocapillaris hypo- or non-perfusion. ICGA findings are essential for the diagnosis and follow-up of PICCPs, but non-invasive methods such as FAF and SD-OCT/OCT-A also have their role especially in follow-up of the diseases. Treatment should be individualized according to the pathology and the evolution of lesions.
原发性炎症性脉络膜毛细血管病变（PICCP）属于一组具有共同特征的眼内炎症性疾病，其主要的临床病理机制是炎症性脉络膜毛细血管低灌注或无灌注。本文的目的是描述有助于 PICCP 诊断和治疗的临床特征和多模态成像。
进行多模态成像分析的叙述性综述。
脉络膜毛细血管无灌注可影响脉络膜终末，在 PICCP 谱的良性端（MEWDS），影响范围可扩大到更严重形式的较大脉络膜毛细血管血管或小动脉前血管，如急性后部多灶性脉络膜炎（APMPPE）、特发性多灶性脉络膜炎（MFC）和匐行性脉络膜炎（SC）。诊断主要基于多模态成像，特别是吲哚菁绿血管造影（ICGA）、眼底自发荧光（FAF）和频域光学相干断层扫描（SD-OCT）/OCT 血管造影（OCT-A）。ICGA 显示出典型的斑片状叶状低荧光模式，反映了脉络膜毛细血管的低灌注或无灌注，在更严重的形式中也可以呈现出地理区域的外观。治疗取决于疾病的严重程度，从 MEWDS 的观察和一些轻度 APMPPE 病例，到更严重的 APMPPE 和 MFC 和 SC 病例中口服皮质类固醇和/或免疫调节剂。为了引入或调整治疗，密切的多模态监测至关重要。
PICCP 是由一个共同的临床病理机制引起的，即炎症性脉络膜毛细血管低灌注或无灌注。ICGA 发现对于 PICCP 的诊断和随访至关重要，但非侵入性方法，如 FAF 和 SD-OCT/OCT-A，在疾病的随访中也有其作用。治疗应根据病变的病理和演变情况个体化。
