Clinical features of systemic lupus erythematosus (SLE)

Systemic lupus erythematosus (SLE) can present with symptoms and signs in any organ- or body-system and can be difficult to diagnose. The common presenting symptoms of SLE are musculoskeletal symptoms, fatigue and rash. In SLE, organs are often affected sequentially rather than concurrently.

Note: In SLE, organs are often affected sequentially rather than concurrently.

For a list of clinical features of SLE, see Clinical features of systemic lupus erythematosus (SLE). Clinical features of SLE in children and adolescents are similar; however, multisystem involvement is more common in children and adolescents than adults, particularly kidney and central nervous system (CNS) diseaseMassias, 2020. See the separate topic on SLE in children and adolescents for more information.

Table 1. Clinical features of systemic lupus erythematosus (SLE)
[NB1]
Group of features	Clinical features shared by SLE and other inflammatory connective tissue diseases	Clinical features more specific to SLE
constitutional	fatigue	unexplained fever [NB2]
cutaneous and mucosal	rash, photosensitivity oral and nasopharyngeal ulcers sicca symptoms	malar rash nonscarring alopecia hard palate ulcers
musculoskeletal	arthralgias, myalgias	inflammatory arthritis in 2 or more joints
vascular	Raynaud phenomenon	premature atherosclerotic cardiovascular disease lupus-associated vasculitis
serosal		serositis, including pleuritis and pleural effusion acute pericarditis pericardial effusion peritonitis
haematological	positive antiphospholipid antibodies [NB3]	leucopenia thrombocytopenia haemolytic anaemia antiphospholipid syndrome
neuropsychiatric		cerebral vasculopathy (may be associated with premature atherosclerosis, thromboembolism, and antiphospholipid syndrome) psychosis seizure delirium
kidney		haematuria proteinuria more than 0.5 g/24 hours lupus nephritis
presence of autoantibodies	positive antiphospholipid antibodies [NB3]	antinuclear antibody (ANA) anti-dsDNA antibody anti-Smith antibody
complement proteins		low C3 and C4
Note: CLE = cutaneous lupus erythematosus; dsDNA = double-stranded DNA NB1: This list is not exhaustive but represents the common and more specific clinical and immunological findings in people with SLE. This includes children, adolescents and adults with SLE. NB2: Coexistent infection must always be actively excluded in patients with SLE and fever. NB3: Antiphospholipid (aPL) antibodies are autoantibodies seen in approximately 40% of people with SLE; however, they are not specific for SLE. They only reflect coexisting antiphospholipid syndrome if the person has experienced episodes of thromboembolism or pregnancy-related complications AND has persistent aPL antibodies.

SLE can coexist with other organ-specific autoimmune disease, such as:

thyroid disease—for example, autoimmune Hashimoto thyroiditis and Graves disease
antiphospholipid syndrome—can predate the development of the systemic symptoms of SLE.