Department of Internal Medicine and Clinical Immunology, CHRU Lille; Centre National de Référence Maladies Systémiques et Auto-Immunes Rares; FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMINeNT), Lille, France.
Department of Pathology, Assistance Publique-Hôpitaux de Paris, Hôpital Tenon, Paris, France; Sorbonne Universités, Université Pierre et Marie Curie, Paris, France; INSERM, UMR S 1155, Paris, France.
Clin Exp Rheumatol. 2018 May-Jun;36(3):371-375. Epub 2018 Feb 14.
Retroperitoneal fibrosis (RPF) is a rare disease, with unknown aetiology (idiopathic RPF: iRPF) in two-thirds of cases. A subset of iRPF may be a manifestation of IgG4-related disease (IgG4-RD). Thus, recognition of IgG4-RD-RPF is crucial to optimise patient's care with iRPF. The current study aimed to examine imaging specific patterns, which could help differentiate between IgG4-RD-RPF and iRPF, and thus skip performing biopsies.
This analysis included patients with iRPF and a retroperitoneal biopsy at the Lille University Hospital, France. We reviewed their baseline characteristics, clinical presentation, biological results and imaging features. Patients were classified in 3 groups according to histopathological characteristics of IgG4-RD as follows: highly suggestive of IgG4-RD, possible IgG4-RD, or non-evocative of IgG4-RD.
Of the 18 patients analysed in the study, 4 (22%) patients had highly suggestive IgG4-RD-RPF, 8 (44%) possible IgG4-RD-RPF and 6 (33%) non-evocative IgG4-RD. We found no clinical, biological features nor specific imaging pattern that could help differentiate between the 3 groups.
After ruling out all known causes of RPF, retroperitoneal biopsy is still necessary to ascertain the diagnosis of IgG4-RD-RPF. No specific pattern can be used to distinguish between IgG4-RD-RPF and iRFP.
腹膜后纤维化（RPF）是一种罕见疾病，其中三分之二的病例病因不明（特发性 RPF：iRPF）。iRPF 的一部分可能是 IgG4 相关疾病（IgG4-RD）的表现。因此，识别 IgG4-RD-RPF 对于优化 iRPF 患者的治疗至关重要。本研究旨在检查特定的影像学模式，这些模式可以帮助区分 IgG4-RD-RPF 和 iRPF，从而避免进行活检。
本分析包括在法国里尔大学医院接受 iRPF 和腹膜后活检的患者。我们回顾了他们的基线特征、临床表现、生物学结果和影像学特征。根据 IgG4-RD 的组织病理学特征，将患者分为 3 组：高度提示 IgG4-RD、可能 IgG4-RD 或非 IgG4-RD。
在本研究中分析的 18 名患者中，4 名（22%）患者为高度提示 IgG4-RD-RPF，8 名（44%）患者为可能 IgG4-RD-RPF，6 名（33%）患者为非提示性 IgG4-RD。我们未发现任何有助于区分这 3 组的临床、生物学特征或特定影像学模式。
在排除 RPF 的所有已知病因后，仍需进行腹膜后活检以确定 IgG4-RD-RPF 的诊断。没有特定的模式可以用来区分 IgG4-RD-RPF 和 iRFP。
