Overview of the clinical manifestations of systemic lupus erythematosus in adults
- Dafna D Gladman, MD, FRCPC
Dafna D Gladman, MD, FRCPC
- Professor of Medicine
- University of Toronto
Systemic lupus erythematosus (SLE) is a chronic inflammatory disease of unknown cause that can affect virtually every organ. Immunologic abnormalities, particularly the production of a number of antinuclear antibodies, are another prominent feature of the disease. Women are affected more frequently than men.
SLE has protean clinical manifestations that can affect virtually every organ, and can vary dramatically from patient to patient. The most common pattern is a mixture of constitutional complaints with skin, musculoskeletal, mild hematologic, and serologic involvement (table 1). However, some patients have predominately hematologic, renal, or central nervous system manifestations. The pattern that dominates during the first few years of illness tends to prevail throughout the disease course. The clinical course of SLE is highly variable among patients and may be characterized by periods of remissions and of chronic or acute relapses.
The clinical manifestations of SLE in adults are presented here. The diagnosis, differential diagnosis, and management of SLE in adults are discussed elsewhere (see "Diagnosis and differential diagnosis of systemic lupus erythematosus in adults" and "Overview of the management and prognosis of systemic lupus erythematosus in adults"). The clinical manifestations of SLE in children are also presented separately. (See "Systemic lupus erythematosus (SLE) in children: Clinical manifestations and diagnosis".)
Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease with a wide range of clinical and serological manifestations that can affect any organ (table 1). The disease course is marked by remissions and relapses and may vary from mild to severe.
The clinical manifestations of SLE are presented below. Most of these manifestations have been included as part of classification criteria for SLE, as a means of categorizing patients for study purposes (table 2). The criteria can be useful for helping clinicians recognize and document some of the key disease manifestations of SLE. The classification criteria are discussed in detail separately. (See "Diagnosis and differential diagnosis of systemic lupus erythematosus in adults", section on 'Classification criteria'.)
- McKinley PS, Ouellette SC, Winkel GH. The contributions of disease activity, sleep patterns, and depression to fatigue in systemic lupus erythematosus. A proposed model. Arthritis Rheum 1995; 38:826.
- Tench CM, McCurdie I, White PD, D'Cruz DP. The prevalence and associations of fatigue in systemic lupus erythematosus. Rheumatology (Oxford) 2000; 39:1249.
- Jump RL, Robinson ME, Armstrong AE, et al. Fatigue in systemic lupus erythematosus: contributions of disease activity, pain, depression, and perceived social support. J Rheumatol 2005; 32:1699.
- Wang B, Gladman DD, Urowitz MB. Fatigue in lupus is not correlated with disease activity. J Rheumatol 1998; 25:892.
- Iaboni A, Ibanez D, Gladman DD, et al. Fatigue in systemic lupus erythematosus: contributions of disordered sleep, sleepiness, and depression. J Rheumatol 2006; 33:2453.
- Cervera R, Khamashta MA, Font J, et al. Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestations in a cohort of 1,000 patients. Medicine (Baltimore) 2003; 82:299.
- Rovin BH, Tang Y, Sun J, et al. Clinical significance of fever in the systemic lupus erythematosus patient receiving steroid therapy. Kidney Int 2005; 68:747.
- Greco CM, Rudy TE, Manzi S. Adaptation to chronic pain in systemic lupus erythematosus: applicability of the multidimensional pain inventory. Pain Med 2003; 4:39.
- Budhram A, Chu R, Rusta-Sallehy S, et al. Anti-cyclic citrullinated peptide antibody as a marker of erosive arthritis in patients with systemic lupus erythematosus: a systematic review and meta-analysis. Lupus 2014; 23:1156.
- Richter JG, Sander O, Schneider M, Klein-Weigel P. Diagnostic algorithm for Raynaud's phenomenon and vascular skin lesions in systemic lupus erythematosus. Lupus 2010; 19:1087.
- Barile-Fabris L, Hernández-Cabrera MF, Barragan-Garfias JA. Vasculitis in systemic lupus erythematosus. Curr Rheumatol Rep 2014; 16:440.
- Ramos-Casals M, Nardi N, Lagrutta M, et al. Vasculitis in systemic lupus erythematosus: prevalence and clinical characteristics in 670 patients. Medicine (Baltimore) 2006; 85:95.
- Dhillon PK, Adams MJ. Thrombosis in systemic lupus erythematosus: role of impaired fibrinolysis. Semin Thromb Hemost 2013; 39:434.
- Sarabi ZS, Chang E, Bobba R, et al. Incidence rates of arterial and venous thrombosis after diagnosis of systemic lupus erythematosus. Arthritis Rheum 2005; 53:609.
- Jung H, Bobba R, Su J, et al. The protective effect of antimalarial drugs on thrombovascular events in systemic lupus erythematosus. Arthritis Rheum 2010; 62:863.
- Danila MI, Pons-Estel GJ, Zhang J, et al. Renal damage is the most important predictor of mortality within the damage index: data from LUMINA LXIV, a multiethnic US cohort. Rheumatology (Oxford) 2009; 48:542.
- Tian XP, Zhang X. Gastrointestinal involvement in systemic lupus erythematosus: insight into pathogenesis, diagnosis and treatment. World J Gastroenterol 2010; 16:2971.
- Miner JJ, Kim AH. Cardiac manifestations of systemic lupus erythematosus. Rheum Dis Clin North Am 2014; 40:51.
- Romero-Díaz J, García-Sosa I, Sánchez-Guerrero J. Thrombosis in systemic lupus erythematosus and other autoimmune diseases of recent onset. J Rheumatol 2009; 36:68.
- Silpa-archa S, Lee JJ, Foster CS. Ocular manifestations in systemic lupus erythematosus. Br J Ophthalmol 2016; 100:135.
- Rosenbaum JT, Trune DR, Barkhuizen A, et al.. Ocular, aural, and oral manifestations. In: Dubois' lupus erythematosus and related syndromes, Eigth, Wallace DJ and Hahn BH. (Ed), Elsevier, Philadelphia 2013. p.393.
- Newman K, Owlia MB, El-Hemaidi I, Akhtari M. Management of immune cytopenias in patients with systemic lupus erythematosus - Old and new. Autoimmun Rev 2013; 12:784.
- Gallais Sérézal I, Bouillet L, Dhôte R, et al. Hereditary angioedema and lupus: A French retrospective study and literature review. Autoimmun Rev 2015; 14:564.
- Abu-Shakra M, Gladman DD, Urowitz MB, Farewell V. Anticardiolipin antibodies in systemic lupus erythematosus: clinical and laboratory correlations. Am J Med 1995; 99:624.
- Haliloglu S, Carlioglu A, Akdeniz D, et al. Fibromyalgia in patients with other rheumatic diseases: prevalence and relationship with disease activity. Rheumatol Int 2014; 34:1275.
- Ehmke TA, Cherian JJ, Wu ES, et al. Treatment of osteonecrosis in systemic lupus erythematosus: a review. Curr Rheumatol Rep 2014; 16:441.
- Zhou WJ, Yang CD. The causes and clinical significance of fever in systemic lupus erythematosus: a retrospective study of 487 hospitalised patients. Lupus 2009; 18:807.
- Nived O, Sturfelt G, Wollheim F. Systemic lupus erythematosus and infection: a controlled and prospective study including an epidemiological group. Q J Med 1985; 55:271.
- Hidalgo-Tenorio C, Jiménez-Alonso J, de Dios Luna J, et al. Urinary tract infections and lupus erythematosus. Ann Rheum Dis 2004; 63:431.
- Cuchacovich R, Gedalia A. Pathophysiology and clinical spectrum of infections in systemic lupus erythematosus. Rheum Dis Clin North Am 2009; 35:75.
- Feldman CH, Hiraki LT, Winkelmayer WC, et al. Serious infections among adult Medicaid beneficiaries with systemic lupus erythematosus and lupus nephritis. Arthritis Rheumatol 2015; 67:1577.
- Hellmann DB, Petri M, Whiting-O'Keefe Q. Fatal infections in systemic lupus erythematosus: the role of opportunistic organisms. Medicine (Baltimore) 1987; 66:341.
- Zandman-Goddard G, Shoenfeld Y. SLE and infections. Clin Rev Allergy Immunol 2003; 25:29.
- Chen HS, Tsai WP, Leu HS, et al. Invasive fungal infection in systemic lupus erythematosus: an analysis of 15 cases and a literature review. Rheumatology (Oxford) 2007; 46:539.
- Barber CE, Barnabe C. Another consequence of severe lupus: invasive fungal disease. J Rheumatol 2012; 39:1772.
- CLINICAL MANIFESTATIONS
- Constitutional symptoms
- Arthritis and arthralgias
- Skin and mucous membrane involvement
- Vascular disease
- - Raynaud phenomenon
- - Vasculitis
- - Thromboembolic disease
- Renal involvement
- Gastrointestinal involvement
- Pulmonary involvement
- Cardiac disease
- Neuropsychiatric involvement
- Ophthalmologic involvement
- Hematologic abnormalities
- Lymphadenopathy and splenomegaly
- OTHER ASSOCIATED CONDITIONS AND COMPLICATIONS
- Antiphospholipid syndrome
- SOCIETY GUIDELINE LINKS
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS