Hypersensitivity Patient B


From N Engl J Med 2013; 368:1528-1535 April 18, 2013

A 20-year-old woman was evaluated by her rheumatologist because she was disabled by flares of systemic lupus erythematosus (SLE). A diagnosis of SLE had been made 2 years earlier, on the basis of a photosensitive malar rash, oral ulcers, polyarthritis, pericarditis, and positive assays for antinuclear antibodies (ANA) and anti-double-stranded DNA (dsDNA) antibodies.


QUESTION: What is a malar rash?

Answer

QUESTION: Why was the issue of photosensitivity of the rash raised with the patient?

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QUESTION: Can the polyarthritis have anything to do with the SLE?

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QUESTION: Why were antinuclear antibodies (ANA) and anti-double-stranded DNA (dsDNA) antibodies tested?

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Treatment with analgesics and hydroxychloroquine for 6 months was not beneficial; prednisone at a dose of 40 mg daily resulted in some improvement, but the patient gained 6.8 kg (15 lb) and required two hospitalizations for infections. SLE flares occurred when the prednisone dose was less than 30 mg daily. Trials of methotrexate (and also two other synthetic immunosuppressive drugs) either had unacceptable side effects or failed to control flares or permit prednisone tapering. On examination, she had cushingoid features with 20 swollen, tender joints and 3 oral ulcers. The ANA titer was positive, at 1:320. An assay for anti-dsDNA antibodies was negative, and the serum complement level was normal. The rheumatologist recommended a trial of belimumab.



QUESTION: Are hydroxychloroquine and methotrexate disease modifying anti-rheumatic drugs (DMARDs)?)

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QUESTION: What type of drug is prednisone?

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QUESTION: What is the cause of the "cushingoid features"?

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QUESTION: What type of drug is belimumab?

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In the patient, the clinical activity of SLE declined after 16 weeks of belimumab treatment. Her arthritis and fatigue were substantially improved, and her flares were milder and less frequent. Her prednisone dose was tapered from 30 mg to 15 mg a day over a period of 24 weeks. However, when the prednisone dose was reduced further, to 12.5 mg daily, she had a flare of SLE and again required 30 mg of prednisone per day. Disappointed, she elected to discontinue belimumab.



QUESTION: Is beclimumab taken orally or infused?

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QUESTION: Why do you suppose the patient discontinued the belimumab?

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