MKSAP Quiz: Follow-up for systemic lupus erythematosus
A 30-year-old woman is seen in follow-up. She was diagnosed with systemic lupus erythematosus 12 years ago. She was treated for lupus nephritis, which is now quiescent. What health risk assessment should be performed now?
A 30-year-old woman is seen in follow-up. She was diagnosed with systemic lupus erythematosus 12 years ago. She was treated for lupus nephritis, which is now quiescent. Clinical and laboratory findings have been stable for 5 years, with no disease flares. Medications are hydroxychloroquine and azathioprine.
Which of the following health risk assessments should be performed now?
A. Breast cancer
B. Cardiovascular disease
C. Iron overload
D. Pulmonary disease
MKSAP Answer and Critique
The correct answer is B. Cardiovascular disease. This content is available to MKSAP 19 subscribers as Question 19 in the Rheumatology section. More information about MKSAP is available online.
The most appropriate assessment to perform is for cardiovascular disease risk (Option B). Patients with systemic lupus erythematosus (SLE) have a high risk for cardiovascular disease compared with the general population, including a greater risk for myocardial infarction and ischemic stroke, even in younger patients. Control of the underlying SLE may be the most important way to minimize this risk, but patients should also be screened for other modifiable risk factors. Assessment of diet, exercise, hypertension, diabetes mellitus, smoking, and potentially lipids are indicated in patients, including young patients, with SLE.
Patients with SLE have a higher overall risk for malignancies (particularly hematologic) but not cancer-related mortality; the risk for non-Hodgkin lymphoma is at least two to three times higher than in the general population. There may be an increased risk for cancers of the vulva, cervix, lung, thyroid, and possibly liver. Malignancy risk in SLE is tied to the use of immunosuppressive agents. Breast cancer risk (Option A) does not seem to be increased, but appropriate screening should be initiated in the future as indicated by guidelines.
In patients with SLE, normocytic, normochromic inflammatory anemia is common; autoimmune hemolytic anemia occurs in approximately 10% and correlates with SLE activity. Lymphopenia/leukopenia is also common but usually mild. Thrombocytopenia occurs in 30% to 50% of patients with SLE, and approximately 10% develop severe thrombocytopenia (platelet count <50,000/μL [50 × 109/L]) in isolation or in conjunction with hemolytic anemia. Iron overload syndromes (Option C), such as hemochromatosis, are not a morbidity of SLE unless associated with increased transfusion requirements.
Some patients with SLE or overlap syndromes can have interstitial lung disease that would warrant additional testing with pulmonary function testing or chest CT. However, this patient is asymptomatic, and assessment for pulmonary disease (Option D) is not warranted.
Key Points
- Patients with systemic lupus erythematosus have a high risk for cardiovascular disease compared with the general population, including a greater risk for myocardial infarction and ischemic stroke, even in younger patients.
- Control of the underlying systemic lupus erythematosus may be the most important way to minimize cardiovascular risk, but patients should also be screened for other modifiable risk factors.