Department of Rheumatology, Tokyo Women's Medical University School of Medicine, Tokyo, Japan.
Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
Mod Rheumatol. 2022 Jan 5;32(1):32-40. doi: 10.1080/14397595.2021.1899570.
To describe the clinicopathological characteristics of lymphoproliferative disorders (LPDs) in patients with rheumatoid arthritis (RA).
In this multicenter case series, we retrospectively reviewed the medical records of RA patients who were newly diagnosed as having LPDs with or without biopsy confirmation between 2000 and 2017 in eight hospitals in Japan.
We included 232 patients with LPDs. The median age was 67 years (interquartile range [IQR], 60-73 years), and 77.1% were female. At the time of LPD diagnosis, 94.8% and 62.6% of the patients were methotrexate users and in remission or had low RA disease activity, respectively; lymphadenopathy and extranodal involvement were present in 77.1% and 51.9%, respectively. Major extranodal sites were the lungs and oral/oropharyngeal mucosa. The most common LPD pathological subtype was diffuse large B-cell lymphoma (40.5%), followed by classic Hodgkin lymphoma (10.8%), Epstein-Barr virus-positive mucocutaneous ulcer (7.7%), and reactive lymphoid hyperplasia (6.2%). The clinical and laboratory characteristics varied across the pathological subtypes.
LPD occurred mainly in methotrexate users, while RA disease activity did not seem to be associated with LPD development. Although the clinical manifestations vary among pathological subtypes, manifestations of LPD in patients with RA can include lymphadenopathy, extranodal mass, and mucocutaneous ulcer.
描述类风湿关节炎（RA）患者中发生的淋巴增殖性疾病（LPD）的临床病理特征。
在这项多中心病例系列研究中，我们回顾性分析了 2000 年至 2017 年间日本 8 家医院中诊断为 LPD 的新发病例（有或无活检证实）的 RA 患者的病历。
我们纳入了 232 例 LPD 患者。中位年龄为 67 岁（四分位距[IQR]，60-73 岁），77.1%为女性。在 LPD 诊断时，94.8%和 62.6%的患者分别正在使用甲氨蝶呤和处于缓解期或 RA 疾病活动度低；77.1%和 51.9%的患者分别存在淋巴结病和结外累及。最常见的结外部位是肺部和口腔/口咽黏膜。最常见的 LPD 病理亚型是弥漫性大 B 细胞淋巴瘤（40.5%），其次是经典霍奇金淋巴瘤（10.8%）、EB 病毒阳性黏膜溃疡（7.7%）和反应性淋巴组织增生（6.2%）。不同病理亚型的临床和实验室特征存在差异。
LPD 主要发生在甲氨蝶呤使用者中，而 RA 疾病活动度似乎与 LPD 发展无关。尽管不同病理亚型的临床表现存在差异，但 RA 患者的 LPD 表现可能包括淋巴结病、结外肿块和黏膜溃疡。
