Prepare for the PANCE with this challenging clinical scenario that tests your ability to diagnose and manage a common yet serious condition. Devise a stepwise, evidence-based approach to treating an adult with systemic lupus erythematosus, including hydroxychloroquine, NSAIDs, glucocorticoids, and other immunomodulating agents, as well as ACE inhibitors and angiotensin receptor blockers.
A 24-year-old woman with a history of systemic lupus erythematosus (SLE) presents to the clinic with fatigue, joint pain, and a malar rash that has worsened over the past two weeks. She was diagnosed with SLE two years ago and takes hydroxychloroquine regularly. She has no shortness of breath or lower extremity swelling. Temperature is 37.2°C (99.0°F), heart rate is 88/min, blood pressure is 130/80 mmHg, respiratory rate is 16/min, and oxygen saturation is 98% on room air. Physical examination is significant for a pronounced malar rash and bilateral joint swelling and tenderness in her wrists, hands, knees, and elbows. Laboratory tests are shown below.
Which of the following is the best next step in management?
A. Oral glucocorticoids
B. Increased dose of hydroxychloroquine
C. Cyclophosphamide
D. Belimumab
E. Angiotensin receptor blocker
Scroll down to find the answer!
The correct answer to today’s PANCE® Question is…
A. Oral glucocorticoids
Before we get to the Main Explanation, let’s look at the incorrect answer explanations. Skip to the bottom if you want to see the correct answer right away!
Incorrect Answer Explanations
B. Increased dose of hydroxychloroquine
Rationale: While hydroxychloroquine is beneficial for long-term management of systemic lupus erythematosus, it does not provide immediate control of moderate disease flares. This patient’s worsening symptoms, especially the moderate hematologic abnormalities (i.e., thrombocytopenia), require the addition of a more potent, rapid-acting treatment like glucocorticoids.
C. Cyclophosphamide
Rationale: Cyclophosphamide is indicated in severe, organ-threatening, and life-threatening cases of systemic lupus erythematosus (e.g., lupus nephritis). In a moderate flare without evidence of severe organ damage, as seen in this patient, the risks and side effects of cyclophosphamide outweigh its benefits.
D. Belimumab
Rationale: Belimumab is an add-on therapy for patients with active systemic lupus erythematosus. It is not initially indicated to treat moderate disease flares. It is typically used when standard therapies fail to control symptoms. This patient’s flare, while moderate, can be managed effectively with standard treatment, such as medium-dose glucocorticoids.
E. Angiotensin receptor blocker
Rationale: Angiotensin II receptor blockers are used to treat hypertension and renal disease in patients with systemic lupus erythematosus. This patient does not have evidence of lupus nephritis or hypertension.
Main Explanation
This patient with a history of systemic lupus erythematosus (SLE) presents with increased fatigue, extensive joint inflammation, a worsening malar rash, anemia, and moderate thrombocytopenia; indicating moderate disease severity. In addition to hydroxychloroquine, oral glucocorticoids (like prednisone) would be the preferred treatment for moderate disease activity without major organ-threatening disease. The potent anti-inflammatory and immunosuppressive effects of glucocorticoids can significantly reduce the symptoms of a lupus flare and prevent further progression of the disease.
Treatment for SLE depends on disease severity. All patients with SLE should be started on hydroxychloroquine due to its ability to reduce the frequency of flares and prevent long-term organ damage. For mild SLE flares, characterized by symptoms such as mild skin and joint involvement and mild cytopenias, hydroxychloroquine should be used and NSAIDs or low-dose glucocorticoids may be added. For moderate disease flares, which may present with more pronounced symptoms and moderate cytopenias, as seen in this patient, treatment includes the addition of oral moderate-dose glucocorticoids. If response is inadequate despite combined therapy, additional immunosuppressants (e.g. azathioprine, mycophenolate mofetil) are indicated. For severe SLE with organ-threatening complications, a more intensive treatment strategy is indicated. This consists of a combination of hydroxychloroquine, high-dose glucocorticoids, and immunosuppressive therapy (e.g., mycophenolate mofetil, cyclophosphamide). ACE inhibitors or angiotensin receptor blockers (ARBs) are also used to treat renal disease.
Major Takeaway
In moderate systemic lupus erythematosus (SLE) without organ-threatening disease, medium-dose oral glucocorticoids can be prescribed in addition to hydroxychloroquine to provide symptom relief and prevent disease progression.
References
- Aringer M, Costenbader K, Daikh D, et al. 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Arthritis Rheumatol. 2019;71(9):1400-1412. doi:10.1002/art.40930
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