Lupus: A Primary Care Primer

Jun 05, 2018

Your patient is a young woman who presents with a facial rash, joint pain, and fatigue. How will you proceed?

References1. Kado R. Systemic lupus erythematosus for primary care. Prim Care. 2018;45:257-270. doi: 10.1016/j.pop.2018.02.011.2. Pons-Estel GJ, AlarcÃ³n GS, Scofield L, et al. Understanding the epidemiology and progression of systemic lupus erythematosus. Semin Arthritis Rheum. 2010;39:257-268. doi: 10.1016/j.semarthrit.2008.10.007.3. Oku K, Atsumi T, Akiyama Y, et al. Evaluation of the alternative classification criteria of systemic lupus erythematosus established by Systemic Lupus International Collaborating Clinics (SLICC). Mod Rheumatol. 2017:1-7. doi: 10.1080/14397595.2017.1385154.4. Petri M, Orbai AM, AlarcÃ³n GS, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012;64:2677-2686. doi: 10.1002/art.34473.

A young woman comes into your office with a rash on her face; she also reports joint pain and fatigue. How will you proceed?

Because many of the symptoms are nonspecific, systemic lupus erythematosus (SLE) can be difficult to diagnose in the primary care setting. Here I describe common presenting features and other manifestations, and I outline a suggested approach. Scroll through the slides for the details.

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Systemic lupus erythematosus (SLE) commonly manifests in the skin, joints, marrow, heart, lungs, kidneys, and brain. The prevalence of this multiorgan, chronic inflammatory disorder ranges from 0.07% to 0.18%.¹

The strongest risk factor is female gender; the risk is about 10 times higher in women than in men.² In addition, the prevalence is higher in blacks than in whites. Blacks often have a younger age at presentation. In addition, the risk of end-organ damage is higher in blacks and other ethnic minorities.

Rash, fatigue, and joint pain are common presenting features of SLE.

The two systems of classification are the 1997 American College of Rheumatology (ACR) Criteria and the Systemic Lupus International Collaborating Clinics (SLICC) criteria.^3,4 The SLICC criteria have an immunologic focus and require meeting at least one immunologic criterion.

Early diagnosis is key to implementing treatment and delaying disease progression.

Antinuclear antibody (ANA) positivity is necessary but not sufficient for diagnosis. Anti-Smith and anti-dsDNA antibodies are specific to SLE.

Suggested tests in primary care for a young woman with SLE features are an ANA screen, extractable nuclear antigen (ENA) 10 panel, ADNA, and C3 and C4 levels. Routine testing for hematuria, proteinuria, impaired renal function, and cytopenia is recommended. Tests for diseases involving other organs should be ordered based on symptoms.

Standard therapy consists of immunosuppression with corticosteroids and the use of hydroxychloroquine, which requires ophthalmic screening because of the risk of retinal toxicity. (For strategies to minimize the risk of ocular toxicity, please see Hydroxychloroquine in Lupus: A Clearer View of Eye Safety.)

Cutaneous lupus erythematosus (CLE) occurs in most patients with SLE.

Types of cytopenia seen in patients with SLE include leukopenia, thrombocytopenia, and autoimmune hemolytic anemia.

RBC, red blood cell.

Nephritis is more likely to occur early after diagnosis and predicts a worse prognosis.

The most common pleuropulmonary disease associated with SLE is pleuritis. Other related pulmonary disorders include interstitial lung disease and acute pneumonitis. Diffuse alveolar hemorrhage is less common but is life-threatening.

The most common cardiac manifestation of SLE is pericarditis. Keep in mind that patients with SLE are at increased risk for coronary artery disease (CAD) and myocardial infarction (MI).

Arthralgia and arthritis are common musculoskeletal manifestations of SLE. The most common neuropsychiatric manifestations are seizures and psychosis.