Objective: Patients characterized with antinuclear antibodies (ANA) and disease symptoms related to one organ system can be described as having incomplete systemic lupus erythematosus (SLE). The aim of this multicentre study was to describe the outcome of these so-called incomplete SLE patients. Two aspects of the outcome were studied: (i) the disease course, defined by the presence or absence of clinical symptoms; and (ii) the number of patients that eventually developed full SLE.
Methods: Outcome parameters were the ACR criteria, the SLE disease Activity Index (SLEDAI), the European Consensus Lupus Activity Measure (ECLAM) and the requirement for treatment. In 10 European rheumatology centres, patients who had been evaluated in the last 3 months of 1994 and had been diagnosed as having incomplete SLE on clinical grounds for at least 1 yr were included in the study. All 122 patients who were included in the study were evaluated annually during 3 yr of follow-up.
Results: Our results are confined to a patient cohort defined by disease duration of at least 1 yr, being under clinical care at the different centres in Europe. These patients showed disease activity that was related mostly to symptoms of the skin and the musculoskeletal system, and leucocytopenia. During the follow-up, low doses of prednisolone were still being prescribed in 43% of the patients. On recruitment to the study, 22 of the 122 incomplete SLE patients already fulfilled the ACR criteria for the diagnosis of SLE. In the 3 yr of follow-up only three patients developed SLE.
Conclusions: A high proportion of patients in our cohort defined on clinical grounds as having incomplete SLE eventually showed disease activity defined by the SLEDAI as well as ECLAM. However, only three cases developed to SLE during the follow-up. This suggests that incomplete SLE forms a subgroup of SLE that has a good prognosis.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1093/rheumatology/40.1.89 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
A20 haploinsufficiency diagnosis beyond systemic lupus erythematosus: A systematic review of the literature.
Autoimmun Rev
January 2025
Department of Clinical Immunology and Internal Medicine, CHU of Caen Normandie, 14000 Caen, France; Normandie Univ, UNICAEN, CHU de Caen Normandie, 14000 Caen, France.
Systemic lupus erythematosus (SLE) is an autoimmune disease whose pathophysiology remains incompletely understood, involving genetic and epigenetic factors. However, an increasing small subset of patients present with monogenic lupus, providing insight into the pathogenesis of the disease. This systematic review focuses on SLE associated with A20 haploinsufficiency (HA20), a monogenic disorder associated with tumor necrosis factor alpha-induced protein 3 gene (TNFAIP3) variants.
View Article and Find Full Text PDFACK1 and BRK non-receptor tyrosine kinase deficiencies are associated with familial systemic lupus and involved in efferocytosis.
Elife
November 2024
Immunology Program, Sloan Kettering Institute, Memorial Sloan Kettering Cancer Center, New York, United States.
Systemic lupus erythematosus (SLE) is an autoimmune disease, the pathophysiology and genetic basis of which are incompletely understood. Using a forward genetic screen in multiplex families with SLE, we identified an association between SLE and compound heterozygous deleterious variants in the non-receptor tyrosine kinases (NRTKs) and . Experimental blockade of ACK1 or BRK increased circulating autoantibodies in vivo in mice and exacerbated glomerular IgG deposits in an SLE mouse model.
View Article and Find Full Text PDFIsolated central nervous system lymphomatoid granulomatosis in an older adult patient with systematic lupus erythematosus: A case report.
Mod Rheumatol Case Rep
January 2025
Department of Nephrology and Rheumatology, Aichi Medical University, Nagakute, Aichi, Japan.
Lymphomatoid granulomatosis (LYG) is a rare, T-cell-rich Epstein-Barr virus (EBV)-positive B-cell lymphoproliferative systemic disease. Only a few cases of LYG have been described in patients with autoimmune disorders, with only one case described in a patient with systemic lupus erythematosus (SLE). However, no cases of isolated central nervous system (CNS)-LYG have been reported in patients with autoimmune diseases.
View Article and Find Full Text PDFIntegration of genetic and chromatin modification data pinpoints autoimmune-specific remodeling of enhancer landscape in CD4 T cells.
Cell Rep
October 2024
Structural and Computational Biology Unit, European Molecular Biology Laboratory, Heidelberg, Germany. Electronic address:
Article Synopsis
- CD4 T cells are essential for adaptive immunity and are linked to autoimmune diseases, but how they malfunction in these diseases is still unclear.
- Researchers used advanced techniques to analyze the active chromatin and gene expression in CD4 T cells from both healthy individuals and those with autoimmune disorders like lupus and arthritis.
- The study identified specific gene regulatory networks affected by enhancers and associated genetic variations, suggesting that changes in CD4 T cell regulation could play a key role in autoimmune disease development and may lead to new treatments.
Female-bias in systemic lupus erythematosus: How much is the X chromosome to blame?
Biol Sex Differ
October 2024
iBB - Institute for Bioengineering and Biosciences, Department of Bioengineering, Instituto Superior Técnico, Universidade de Lisboa, Lisbon, Portugal.
Systemic lupus erythematosus (SLE or lupus) is an immune-mediated disease associated with substantial medical burden. Notably, lupus exhibits a striking female bias, with women having significantly higher susceptibility compared to men, up to 14-fold higher in some ethnicities. Supernumerary X chromosome syndromes, like Klinefelter (XXY) and Triple X syndrome (XXX), also present higher SLE prevalence, whereas Turner syndrome (XO) displays lower prevalence.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!