Magro-Checa César, Ramiro Sofia, Rúa-Figueroa Iñigo, Jimenez Norman, Del Campo-Pérez Víctor, Martinez-Barrio Julia, Galindo-Izquierdo María, Calvo-Alén Jaime, Uriarte-Isacelaya Esther, Tomero-Muriel Eva, Freire-González Mercedes, Martínez-Taboada Víctor, Salgado Eva, Vela Paloma, Mena-Vázquez Natalia, Olivé Alejandro, Narváez Javier, Menor-Almagro Raúl, Santos-Soler Gregorio, Hernández-Beriaín José A, Manero-Ruiz Javier, Aurrecoechea-Aguinaga Elena, Ibarguengoitia Oihane, Montilla-Morales Carlos, Bonilla-Hernán Gema, Torrente-Segarra Vicente, Salman-Monte Tarek, Ros-Vilamajo Inmaculada, García-Villanueva María Jesús, Moriano-Morales Clara, Fito-Manteca Concepción, Lozano-Rivas Nuria, Bohórquez Cristina, Pego-Reigosa José M
Department of Rheumatology, Zuyderland Medical Center, Heerlen and Sittard-Geleen, the Netherlands.
Department of Rheumatology, Zuyderland Medical Center, Heerlen and Sittard-Geleen, the Netherlands; Department of Rheumatology, Leiden University Medical Center, Leiden, the Netherlands.
Semin Arthritis Rheum. 2023 Feb;58:152121. doi: 10.1016/j.semarthrit.2022.152121. Epub 2022 Oct 22.
To analyze the prevalence, incidence, survival and contribution on mortality of major central nervous system (CNS) involvement in systemic lupus erythematosus (SLE).
Patients fulfilling the SLE 1997 ACR classification criteria from the multicentre, retrospective RELESSER-TRANS (Spanish Society of Rheumatology Lupus Register) were included. Prevalence, incidence and survival rates of major CNS neuropsychiatric (NP)-SLE as a group and the individual NP manifestations cerebrovascular disease (CVD), seizure, psychosis, organic brain syndrome and transverse myelitis were calculated. Furthermore, the contribution of these manifestations on mortality was analysed in Cox regression models adjusted for confounders.
A total of 3591 SLE patients were included. Of them, 412 (11.5%) developed a total of 522 major CNS NP-SLE manifestations. 61 patients (12%) with major CNS NP-SLE died. The annual mortality rate for patients with and without ever major CNS NP-SLE was 10.8% vs 3.8%, respectively. Individually, CVD (14%) and organic brain syndrome (15.5%) showed the highest mortality rates. The 10% mortality rate for patients with and without ever major CNS NP-SLE was reached after 12.3 vs 22.8 years, respectively. CVD (9.8 years) and organic brain syndrome (7.1 years) reached the 10% mortality rate earlier than other major CNS NP-SLE manifestations. Major CNS NP-SLE (HR 1.85, 1.29-2.67) and more specifically CVD (HR 2.17, 1.41-3.33) and organic brain syndrome (HR 2.11, 1.19-3.74) accounted as independent prognostic factors for poor survival.
The presentation of major CNS NP-SLE during the disease course contributes to a higher mortality, which may differ depending on the individual NP manifestation. CVD and organic brain syndrome are associated with the highest mortality rates.
分析系统性红斑狼疮（SLE）主要中枢神经系统（CNS）受累的患病率、发病率、生存率及其对死亡率的影响。
纳入多中心回顾性RELESSER - TRANS（西班牙风湿病学会狼疮登记处）中符合1997年美国风湿病学会（ACR）SLE分类标准的患者。计算主要中枢神经系统神经精神（NP）-SLE作为一个整体以及个体NP表现（脑血管疾病（CVD）、癫痫发作、精神病、器质性脑综合征和横贯性脊髓炎）的患病率、发病率和生存率。此外，在针对混杂因素进行调整的Cox回归模型中分析这些表现对死亡率的影响。
共纳入3591例SLE患者。其中，412例（11.5%）出现了总共522例主要中枢神经系统NP - SLE表现。61例（12%）主要中枢神经系统NP - SLE患者死亡。有和没有主要中枢神经系统NP - SLE病史的患者年死亡率分别为10.8%和3.8%。单独来看，CVD（14%）和器质性脑综合征（15.5%）的死亡率最高。有和没有主要中枢神经系统NP - SLE病史的患者分别在12.3年和22.8年后达到10%的死亡率。CVD（9.8年）和器质性脑综合征（7.1年）比其他主要中枢神经系统NP - SLE表现更早达到10%的死亡率。主要中枢神经系统NP - SLE（风险比[HR] 1.85，1.29 - 2.67），更具体地说CVD（HR 2.17，1.41 - 3.33）和器质性脑综合征（HR 2.11，1.19 - 3.74）是生存不良的独立预后因素。
在疾病过程中出现主要中枢神经系统NP - SLE会导致更高的死亡率，这可能因个体NP表现而异。CVD和器质性脑综合征的死亡率最高。
