Signs and Symptoms

Patients may present with single-organ involvement, such as nephritis, or with a multisystem disease. The characteristic cutaneous manifestation of SLE is the butterfly rash (or malar rash) across the nose and cheeks, which appears in 70%–80% of patients (Fig 9-4). Other cutaneous manifestations include discoid lesions, which often lead to scarring, and alopecia. Mucosal lesions, usually painless oral or nasal ulcers, are present in up to 40% of patients. Acute or chronic photosensitivity occurs in many patients.

Approximately 90% of SLE patients experience articular disease, as either a polyarthralgia or a nondeforming migratory polyarthritis. Constitutional symptoms, such as fatigue, fever, myalgia, and weight loss, are common. Renal disease affects approximately 50% of patients; it can present with a range of manifestations from hematuria, proteinuria, and nephrotic syndrome to fulminant glomerulonephritis and renal failure.

Figure 9-4 Malar “butterfly” rash in a patient with systemic lupus erythematosus.

(Used with permission from Mayo Foundation for Medical Education and Research. All rights reserved.)

Figure 9-5 Raynaud phenomenon. A, Sharply demarcated pallor resulting from the closure of digital arteries. B, Digital cyanosis of the fingertips in a patient with primary Raynaud phenomenon.

(Reproduced with permission from Wigley FM. Clinical practice. Raynaud’s phenomenon. N Engl J Med. 2002;347(13):1001. Copyright ©2002 Massachusetts Medical Society.)

Raynaud phenomenon occurs in up to 50% of patients with SLE (Fig 9-5). Anemia of chronic disease is common, along with reduction in leukocytes and platelets. The rate of coronary artery disease is significantly greater in SLE patients than in unaffected individuals. Other, less common cardiac manifestations include pericarditis, myocarditis and Libman-Sacks endocarditis. Valvular disease has been reported in more than 50% of patients. Thromboembolism is more common in SLE, especially in the presence of antiphospholipid antibodies and lupus anticoagulant. Pulmonary involvement includes pleuritis, interstitial lung disease, and pulmonary hypertension. Gastrointestinal manifestations are varied and include dysphagia, esophagitis, hepatitis, and pancreatitis (GI symptoms are sometimes related to medications). Central nervous system (CNS) involvement occurs in more than one-third of patients, and symptoms are typically transient. The most common presentations of neurologic lupus are headache, cognitive impairment, seizures, psychosis, and peripheral neuropathy. See “Ophthalmic considerations” for ocular manifestations.

Excerpted from BCSC 2020-2021 series: Section 1 - Update on General Medicine. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.