The relationship between initial clinical manifestation and long-term prognosis of patients with systemic lupus erythematosus

Abstract

The relationship between clinical manifestations and prognosis was examined and evaluated among systemic lupus erythematosus (SLE) patients. A total of 542 patients with SLE were selected and divided into nine groups according to their main clinical manifestation at the time of initial diagnosis. The relationship between these clinical manifestations and long-term prognosis was evaluated in respect to the survival, remission, relapse rates, the development of a new clinical manifestation, and/or damage index. Patients with neuropsychiatric SLE (NPSLE), accompanied with acute confusional state/seizure disorder, cerebral vascular disease, or pneumonitis had poor survival rates with cause of death related to their major organ involvement. Patients with nephropathy or leukopenia had lower remission rates, and an increase in relapse rates was frequently recognized in patients with pneumonitis. Body damage (damage index) was higher in patients with lupus psychosis, pneumonitis, and/or arthritis. The translation of the main manifestations after diagnosis was confirmed in 64 patients (11.8%), and often observed in patients with autoimmune hemolytic anemia and arthritis. The majority of these manifestations were nephropathy, NPSLE, thrombocytopenia, and pneumonitis, and the prognosis of patients with nephropathy and thrombocytopenia as a new main manifestation had a poor outcome. The results of long-term prognosis in SLE greatly differed with respect to the initial clinical manifestation at the time of diagnosis.

The Long-Term Prognosis of Lupus Nephritis Patients Treated with Intravenous Cyclophosphamide

“…Our previous study first demonstrated the efficacy in Japanese patients, and these results were related to the lymphocyte marker (7). However (10), as patients with no nephritis at the time of the initial diagnosis and who had nephritis during the disease course. These initial main manifestations were defined in our previous study (10 …”

“…However (10), as patients with no nephritis at the time of the initial diagnosis and who had nephritis during the disease course. These initial main manifestations were defined in our previous study (10 …”

Cognitive Performance Scores for the Pediatric Automated Neuropsychological Assessment Metrics in Childhood‐Onset Systemic Lupus Erythematosus

“…Childhood-onset SLE is often associated with major organ involvement, including neuropsychiatric disease (NPSLE) affecting between 43% and 95% of patients (1,2). Although it is a risk factor for poor disease outcomes in all age groups (3)(4)(5)(6)(7)(8), NPSLE is of special concern in pediatric populations. Children with NPSLE may experience school failure due to attention and learning difficulties (9).…”

Brain MRI in patients with acute confusional state of diffuse psychiatric/neuropsychological syndromes in systemic lupus erythematosus

“…Notably, the presence of brain MRI abnormalities significantly increased the mortality in patients with diffuse NPSLE [16]. Among a variety of manifestations in diffuse NPSLE, ACS is the most serious one, sometimes resulting in poor prognoses [10,17]. However, there was no significant association of brain MRI abnormalities with certain types of diffuse NPSLE [16].…”

“…The frequency of ACS ranges from 50% to 58% of patients with diffuse NPSLE [15,16]. Among a variety of manifestations in NPSLE, ACS is the most serious one, requiring extensive immunosuppressive therapy and sometimes resulting in poor prognosis [17,18].…”