DESTIN, FLORIDA—During the Congress of Clinical Rheumatology (CCR) East meeting in May, Michelle Petri, MD, MPH, MACR, professor of medicine in the Division of Rheumatology and director of the Johns Hopkins Lupus Center, Johns Hopkins School of Medicine, Baltimore, presented a talk titled 10 Things I Want You to Know about Lupus.
HCQ Use in SLE
The first pearl Dr. Petri offered is the benefit of hydroxychloroquine (HCQ) and how it should be used as a background therapy for all patients with systemic lupus erythematosus (SLE). She explained HCQ should not be tapered in a patient with quiescent lupus because multiple studies have demonstrated an improvement in survival and reduction in organ damage and cardiovascular risk in those taking the drug. She explained her disagreement with the ophthalmology and ACR weight-based guidelines for HCQ, although she agrees with the need for retinal toxicity monitoring.
Next, Dr. Petri discussed a second pearl on HCQ retinopathy. The risk of retinopathy is low in the first 10 years of use. Central serous retinopathy is an important mimic of HCQ retinopathy seen in patients taking high doses of glucocorticoids. She emphasized the importance of direct communication with ophthalmology when there is confusion regarding the etiology of an abnormal eye exam or inappropriate recommendations.
HCQ whole blood levels can also be used to stratify patients for retinopathy risk.
“If a patient has whole blood hydroxychloroquine levels above 1,200 ng/mL consistently, visit after visit, that patient has an increased risk [of HCQ retinopathy],” Dr. Petri said. She recommended maintaining HCQ whole blood levels “in the optimal range, 750–1,200 [ng/mL].”
Vitamin D in Lupus Pregnancy
Dr. Petri
Dr. Petri’s third pearl urged rheumatologists to understand the role of vitamin D in SLE. “We found up to a 25-hydroxy vitamin D level of 40 significantly reduces urine protein,” she said.
Maternal fetal medicine experts are also recommending vitamin D supplementation in pregnancy in the general population because data suggest it reduces adverse pregnancy outcomes, according to Dr. Petri. She studied vitamin D levels in pregnant patients with SLE, but found a U-shaped association between vitamin D level and adverse pregnancy outcomes.1
“If the woman’s vitamin D level is low, she is at greater risk of adverse pregnancy outcomes,” Dr. Petri said. “However, if the vitamin D level is too high, she is at greater risk of adverse outcomes, as well.”
She recommends measuring vitamin D levels every trimester in a lupus pregnancy and making sure 25-hydroxy vitamin D levels are “in the sweet spot,” between 40 and 59 ng/mL.
Antiphospholipid Antibodies & Thrombosis Risk
Dr. Petri’s fourth pearl regarded the fact that antiphospholipid (aPL) antibody presence is intermittent in SLE, but thrombosis risk remains. “Get in the habit of checking aPL antibodies more than once so you will actually know which of your patients are hypercoagulable,” Dr. Petri said.
Patients with SLE are positive intermittently for aPL antibodies, often less than 25% of the time when serially checked.
The fifth pearl: Thrombosis in SLE occurs for many reasons. In the Hopkins SLE cohort, researchers demonstrated PC4d (i.e., platelet-bound complement split product or marker of platelet activation) is associated with risk of thrombosis.
Dr. Petri also highlighted a recent “earth-shattering” discovery made by her colleagues at Johns Hopkins. “Sixty percent of people with catastrophic antiphospholipid antibody syndrome (CAPS) have mutated complement control proteins,” she said.
A significant reduction in surface CR1 expression, a complement receptor, also occurs in CAPS. “Given [that] CAPS is a complementopathy, similar to atypical HUS [hemolytic uremic syndrome], treatment [for CAPS] should be an anti-complement therapy, like eculizumab,” she said.
“The next time you have a patient with CAPS in your ICU, what are you going to do that actually matters?” Dr. Petri asked. The answer: “Anti-complement therapy.”
Comorbidities & Glucocorticoids
The sixth pearl: Recognize that cardiovascular events occur early in SLE, even in the first two years after diagnosis. Prednisone itself increases the risk of cardiovascular events, especially at doses of 10 mg or more per day, Dr. Petri explained.
Reducing prednisone was a central theme of the seventh take-home message. Dr. Petri said higher doses of prednisone are associated with an increased risk of organ damage in SLE, even after adjusting for confounding by indication.
“If you increase prednisone by 1 mg, organ damage goes up by 3%,” Dr. Petri said.
She noted the 2023 EULAR guidelines for SLE management recommend reducing glucocorticoids to a maintenance dose of less than, or equal to, 5 mg of prednisone per day and, when possible, withdrawing them.2
Final Points
The eighth pearl: Implement earlier introduction of immunosuppressive agents and/or biologics, particularly in patients unable to reduce glucocorticoid below doses acceptable for chronic use.
Mortality in SLE was the ninth point Dr. Petri wanted everyone to recognize. The average age of death in her Hopkins SLE cohort is 55 years, compared with an average life expectancy of 78.39 years in the general U.S. population.
Dr. Petri’s 10th and final pearl was to think about remission when treating SLE. Although Dr. Petri has avoided discussing remission in the past, she notes the evolution of novel biologic therapies in SLE, including belimumab and anifrolumab, have helped many patients achieve remission.
Bottom line: SLE treatment is rapidly evolving as new therapies are developed and approved.
Mithu Maheswaranathan, MD, is an assistant professor of medicine in the Division of Rheumatology at Duke University School of Medicine, Durham, N.C.
References
- Madanchi N, Fava A, Goldman DW, et al. Association between 25-hydroxyvitamin D levels and adverse pregnancy outcomes in systemic lupus erythematosus. Arthritis Care Res (Hoboken). 2025 Apr;77(4):432–439.
- Fanouriakis A, Kostopoulou M, Andersen J, et al. EULAR recommendations for the management of systemic lupus erythematosus: 2023 update. Ann Rheum Dis. 2024 Jan 2;83(1):15–29.
