Update οn the diagnosis and management of systemic lupus erythematosus

Ann Rheum Dis. 2021 Jan;80(1):14-25. doi: 10.1136/annrheumdis-2020-218272. Epub 2020 Oct 13.


Clinical heterogeneity, unpredictable course and flares are characteristics of systemic lupus erythematosus (SLE). Although SLE is-by and large-a systemic disease, occasionally it can be organ-dominant, posing diagnostic challenges. To date, diagnosis of SLE remains clinical with a few cases being negative for serologic tests. Diagnostic criteria are not available and classification criteria are often used for diagnosis, yet with significant caveats. Newer sets of criteria (European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019) enable earlier and more accurate classification of SLE. Several disease endotypes have been recognised over the years. There is increased recognition of milder cases at presentation, but almost half of them progress overtime to more severe disease. Approximately 70% of patients follow a relapsing-remitting course, the remaining divided equally between a prolonged remission and a persistently active disease. Treatment goals include long-term patient survival, prevention of flares and organ damage, and optimisation of health-related quality of life. For organ-threatening or life-threatening SLE, treatment usually includes an initial period of high-intensity immunosuppressive therapy to control disease activity, followed by a longer period of less intensive therapy to consolidate response and prevent relapses. Management of disease-related and treatment-related comorbidities, especially infections and atherosclerosis, is of paramount importance. New disease-modifying conventional and biologic agents-used alone, in combination or sequentially-have improved rates of achieving both short-term and long-term treatment goals, including minimisation of glucocorticoid use.

Keywords: lupus erythematosus; lupus nephritis; systemic; therapeutics.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Anemia, Hemolytic, Autoimmune / physiopathology
  • Anemia, Hemolytic, Autoimmune / therapy
  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Autoantibodies / immunology
  • Azathioprine / therapeutic use
  • Calcineurin Inhibitors / therapeutic use
  • Cardiovascular Diseases / epidemiology
  • Cyclophosphamide / therapeutic use
  • Disease Management
  • Female
  • Glucocorticoids / therapeutic use
  • Heart Valve Diseases / physiopathology
  • Heart Valve Diseases / therapy
  • Humans
  • Hydroxychloroquine / therapeutic use
  • Hypertension, Pulmonary / physiopathology
  • Hypertension, Pulmonary / therapy
  • Immunosuppressive Agents / therapeutic use*
  • Lupus Erythematosus, Systemic / diagnosis
  • Lupus Erythematosus, Systemic / immunology
  • Lupus Erythematosus, Systemic / physiopathology
  • Lupus Erythematosus, Systemic / therapy*
  • Lupus Nephritis / physiopathology
  • Lupus Nephritis / therapy
  • Lupus Vasculitis, Central Nervous System / physiopathology
  • Lupus Vasculitis, Central Nervous System / therapy
  • Macrophage Activation Syndrome / physiopathology
  • Macrophage Activation Syndrome / therapy
  • Methotrexate / therapeutic use
  • Mycophenolic Acid / therapeutic use
  • Myocarditis / physiopathology
  • Myocarditis / therapy
  • Outcome Assessment, Health Care
  • Pericarditis / physiopathology
  • Pericarditis / therapy
  • Phenotype
  • Pregnancy
  • Pregnancy Complications / epidemiology
  • Pregnancy Complications / physiopathology
  • Pregnancy Complications / therapy
  • Prognosis
  • Purpura, Thrombocytopenic, Idiopathic / physiopathology
  • Purpura, Thrombocytopenic, Idiopathic / therapy
  • Quality of Life
  • Recurrence
  • Rituximab / therapeutic use
  • Severity of Illness Index
  • Survival Rate
  • Uterine Cervical Neoplasms / epidemiology


  • Antibodies, Monoclonal, Humanized
  • Autoantibodies
  • Calcineurin Inhibitors
  • Glucocorticoids
  • Immunosuppressive Agents
  • Rituximab
  • Hydroxychloroquine
  • belimumab
  • Cyclophosphamide
  • Mycophenolic Acid
  • Azathioprine
  • Methotrexate