Moreira Sónia, Crespo Jorge, Santos Lèlita
Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
BMJ Case Rep. 2018 Jun 15;2018:bcr-2017-224148. doi: 10.1136/bcr-2017-224148.
Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterised by vascular abnormalities, immune system activation and fibrosis. Lymphatic involvement in SSc was described more recently and starts in early stages. This report describes a 46-year-old patient who developed over the last 2 years asymmetric lymphedema in lower extremities. Compromise in lymphatic drainage was confirmed by lymphoscintigraphy. She also presented Raynaud's phenomenon, a scleroderma pattern in nailfold capillaroscopy, cutaneous thickening and anticentromere antibodies, which together resulted in a new diagnosis of limited cutaneous SSc. Treatment with methotrexate, prednisolone and lymphatic drainage resulted in lymphedema improvement. To our knowledge, this is the first case of grade 2 lymphedema in the setting of anticentromere-positive limited cutaneous SSc. We highlight the importance of considering rheumatic diseases in the differential diagnosis of lymphedema.
系统性硬化症（SSc）是一种自身免疫性结缔组织疾病，其特征为血管异常、免疫系统激活和纤维化。SSc中的淋巴系统受累是最近才被描述的，且始于疾病早期。本报告描述了一名46岁患者，在过去2年中出现了双下肢不对称性淋巴水肿。淋巴闪烁造影证实了淋巴引流受损。她还出现了雷诺现象、甲襞毛细血管镜检查中的硬皮病样表现、皮肤增厚以及抗着丝点抗体，这些共同导致了有限皮肤型SSc的新诊断。甲氨蝶呤、泼尼松龙治疗以及淋巴引流使淋巴水肿得到改善。据我们所知，这是抗着丝点抗体阳性的有限皮肤型SSc患者出现2级淋巴水肿的首例病例。我们强调在淋巴水肿的鉴别诊断中考虑风湿性疾病的重要性。
