Rheumatology Unit, Azienda Ospedaliero Universitaria Pisana, Pisa, Italy.
Department of Rheumatology, Hôpitaux Universitaires de Strasbourg, INSERM UMR-S 1109, Centre National de Référence des Maladies Auto-immunes Systémiques Rares (RESO), Strasbourg, France.
Clin Exp Rheumatol. 2022 May;40 Suppl 134(5):93-102. doi: 10.55563/clinexprheumatol/jrz47c. Epub 2022 Apr 29.
The purpose of this study was to review the frequency and clinical presentation of the rarest clinical manifestations of systemic lupus erythematosus (SLE).
A list of 6 rare SLE manifestations were defined: gastrointestinal, liver, pulmonary, cardiac, ocular and neurological manifestations. Each topic was assigned to a pair of authors to perform a literature search and article review.
In total, 149 articles were included in the literature review: 37 for gastrointestinal manifestations, 6 for liver manifestations, 27 for pulmonary manifestations, 50 for cardiac manifestations, 16 for ocular manifestations, 13 for neurological manifestations. Gastrointestinal disorders included several clinical presentations with variable frequency (from 0.5% to 10.7% of the cases); liver involvement included lupus-related hepatitis (9.3%) and autoimmune hepatitis (2.3%). The rarest pulmonary manifestations identified were shrinking lung syndrome, described in 1.5% of patients, while interstitial lung disease and lupus pneumonia were reported in 4% and 3% of patients respectively. Myocarditis and pulmonary hypertension were also rarely described in SLE patients although ranging from 0.4-16% and 1-14% respectively, depending on the methodology used for its identification. Ocular manifestations in SLE included some rare manifestations (reported in less than 5% of patients) and lupus retinopathy that is described in 1.2-28.8% of patients depending on methods of ascertainment. Aseptic meningitis and chorea were also confirmed as very rare manifestations being reported in less than 1% and in 0.3-2.4% of cases respectively.
The results of this literature review provide the basis for a better understanding of some less-known manifestations of SLE and for stressing the need for a higher awareness in diagnostic and therapeutic protocols regarding these rare disease aspects.
本研究旨在回顾系统性红斑狼疮（SLE）最罕见临床表现的频率和临床表现。
确定了 6 种罕见的 SLE 表现：胃肠道、肝脏、肺部、心脏、眼部和神经系统表现。每个主题都分配给一对作者进行文献检索和文章回顾。
共有 149 篇文章纳入文献综述：胃肠道表现 37 篇，肝脏表现 6 篇，肺部表现 27 篇，心脏表现 50 篇，眼部表现 16 篇，神经系统表现 13 篇。胃肠道疾病包括多种临床表现，其频率各不相同（占病例的 0.5%至 10.7%）；肝脏受累包括狼疮相关性肝炎（9.3%）和自身免疫性肝炎（2.3%）。最罕见的肺部表现为缩肺综合征，在 1.5%的患者中发现，而间质性肺病和狼疮性肺炎分别在 4%和 3%的患者中报道。心肌炎和肺动脉高压在 SLE 患者中也很少描述，尽管根据其识别方法，范围分别为 0.4-16%和 1-14%。SLE 中的眼部表现包括一些罕见表现（少于 5%的患者报告）和狼疮性视网膜病变，根据确定方法，其在 1.2-28.8%的患者中报告。无菌性脑膜炎和舞蹈病也被证实为非常罕见的表现，不到 1%和 0.3-2.4%的病例分别报告。
本文献复习的结果为更好地了解 SLE 的一些鲜为人知的表现提供了基础，并强调了在这些罕见疾病方面的诊断和治疗方案中需要提高认识。
