From the Guidelines
Systemic lupus erythematosus (SLE) is a multisystem disease that presents with diverse clinical features, including constitutional, cutaneous, musculoskeletal, renal, neuropsychiatric, hematologic, cardiovascular, pulmonary, and gastrointestinal manifestations, as noted in the 2019 update of the EULAR recommendations for the management of SLE 1.
Clinical Features of SLE
The clinical features of SLE can be categorized into several key areas, including:
- Constitutional symptoms: fatigue, fever, and weight loss, which occur in most patients
- Cutaneous involvement: classic malar "butterfly" rash, discoid lesions, photosensitivity, and oral ulcers
- Musculoskeletal symptoms: arthralgia or non-erosive arthritis in the small joints of hands, wrists, and knees, affecting up to 90% of patients
- Renal involvement: mild proteinuria to lupus nephritis, which can progress to end-stage renal disease if untreated, occurring in about 50% of patients
- Neuropsychiatric manifestations: headaches, cognitive dysfunction, seizures, psychosis, and stroke
- Hematologic abnormalities: leukopenia, lymphopenia, anemia, and thrombocytopenia
- Cardiovascular complications: pericarditis, myocarditis, and accelerated atherosclerosis
- Pulmonary involvement: pleuritis, pneumonitis, or pulmonary hypertension
- Gastrointestinal symptoms: abdominal pain, nausea, and hepatosplenomegaly
Management and Prognosis
The management of SLE is challenging due to the heterogeneity of disease phenotype and variable severity of involvement, as discussed in the 2023 update of the EULAR recommendations 1. Treatment goals include long-term patient survival, prevention of organ damage, and optimization of health-related quality of life, as outlined in the 2019 EULAR recommendations 1. Prognosis is highly variable, and determination of prognosis is important, with several clinical involvement manifestations, laboratory tests, and immunological tests correlating with outcome, as noted in the 2008 EULAR recommendations 1.
Diagnosis and Monitoring
Diagnosis and monitoring of SLE require a comprehensive approach, including clinical evaluation, laboratory tests, and imaging studies, as discussed in the 2008 EULAR recommendations 1. New clinical manifestations, laboratory tests, and immunological tests may provide prognostic information for the outcome in general and involvement of major organs, and thus should be considered in the evaluation of these patients 1.
From the Research
Clinical Features of SLE
The clinical features of Systemic Lupus Erythematosus (SLE) are diverse and can affect multiple organ systems, including:
- Mucocutaneous system 2, 3, 4, 5
- Musculoskeletal system 2, 3, 4, 5
- Cardiopulmonary system 2, 3, 4
- Renal system 2, 3, 4
- Gastrointestinal system 2
- Central nervous system 2, 3, 4
- Hematologic system 3
Organ Involvement
Approximately 40% of people with SLE develop lupus nephritis, and an estimated 10% of people with lupus nephritis develop end-stage kidney disease after 10 years 3. The disease can also cause immune-mediated damage to multiple organs, leading to increased mortality 3.
Symptoms
Common symptoms of SLE include:
Diagnosis
The 2019 European Alliance of Associations for Rheumatology/American College of Rheumatology classification criteria are used to diagnose SLE, and include both clinical factors and immunologic measures 3. These criteria are estimated to be 96.1% sensitive and 93.4% specific for SLE 3.