Université Paris Descartes-Sorbonne Paris Cité, France; AP-HP, Centre de référence maladies auto-immunes et systémiques rares de l'île de France, Service de médecine interne Pôle médecine, Hôpital Cochin, 27 rue du Faubourg Saint Jacques, 75679 Paris cedex 14, France.
AP-HP, Centre de référence maladies auto-immunes et systémiques rares de l'île de France, Service de médecine interne Pôle médecine, Hôpital Cochin, 27 rue du Faubourg Saint Jacques, 75679 Paris cedex 14, France.
Autoimmun Rev. 2018 Sep;17(9):866-872. doi: 10.1016/j.autrev.2018.03.011. Epub 2018 Jul 10.
To assess the limitations of the SLICC (Systemic Lupus International Collaborating Clinics) classification criteria for systemic lupus erythematosus (SLE), in patients with primary antiphospholipid syndrome (PAPS).
Retrospective study of a cohort of APS patients (Sydney criteria). We successively excluded patients with (1) at least one "SLE-specific" manifestation (biopsy-proven SLE nephropathy, arthritis, cutaneous, or neurologic SLE manifestations, pericarditis, autoimmune haemolytic anaemia, oral and nasal ulcers, non-scarring alopecia, anti-dsDNA, and anti-Sm antibodies), (2) any other autoimmune connective tissue disease, and/or (3) antinuclear antibodies >1/320. Careful file review confirmed PAPS among the remaining patients. We then assessed the number of SLICC criteria each patient met.
Among these 214 APS patients, we excluded 85 with at least one SLE-specific manifestation, 8 with another connective tissue disease, and 21 with antinuclear antibodies >1/320, leaving 100 patients with primary APS. Among them, 28% met at least 4 SLICC classification criteria including one clinical and one immunological criterion (antiphospholipid antibodies, aPL, by definition) and could thus theoretically be classified with SLE. Fourteen had an arterial phenotype (50%), 9 a history of catastrophic APS (32%), and 18 a triple-positive profile for aPL (64%). None had developed SLE during a median follow-up of 12 [6.5-17] years.
Because 28% of our patients with longstanding and strictly defined PAPS could be mistakenly classified as SLE, they were at risk of deleterious therapeutic management. We therefore suggest that any future classification for SLE should specifically require at least one SLE-specific criterion for patients with aPL.
评估 SLICC（系统性红斑狼疮国际合作临床）分类标准对原发性抗磷脂综合征（PAPS）患者系统性红斑狼疮（SLE）的局限性。
回顾性研究一组 APS 患者（悉尼标准）。我们依次排除了以下患者：（1）至少有一种“SLE 特异性”表现（活检证实的狼疮肾炎、关节炎、皮肤或神经狼疮表现、心包炎、自身免疫性溶血性贫血、口腔溃疡、非瘢痕性脱发、抗 dsDNA 和抗 Sm 抗体），（2）任何其他自身免疫性结缔组织病，和/或（3）抗核抗体 >1/320。仔细的病历审查证实了其余患者的 PAPS。然后，我们评估了每位患者符合的 SLICC 标准数。
在这 214 名 APS 患者中，我们排除了 85 名至少有一种 SLE 特异性表现的患者、8 名患有其他结缔组织病的患者和 21 名抗核抗体 >1/320 的患者，留下 100 名原发性 APS 患者。其中，28%的患者符合至少 4 项 SLICC 分类标准，包括一项临床和一项免疫学标准（抗磷脂抗体，aPL，根据定义），因此理论上可以归类为 SLE。14 名患者有动脉表型（50%），9 名患者有灾难性 APS 病史（32%），18 名患者 aPL 呈三阳性（64%）。在中位数为 12[6.5-17]年的随访期间，没有患者发展为 SLE。
由于我们的 28%具有长期和严格定义的 PAPS 的患者可能被错误地归类为 SLE，他们存在有害治疗管理的风险。因此，我们建议任何未来的 SLE 分类标准都应特别要求有 aPL 的患者至少有一项 SLE 特异性标准。
