Progression From Cutaneous to Systemic Lupus Erythematosus May Not Involve Systemic Symptoms

By Will Boggs MD

February 07, 2014

NEW YORK (Reuters Health) - Most patients who progress from cutaneous lupus erythematosus (CLE) to systemic lupus erythematosus (SLE) do not develop significant systemic manifestations, according to a longitudinal study.

"There is a risk of progression to SLE when patients start out with CLE alone," coauthor Dr. Victoria P. Werth from the Hospital of the University of Pennsylvania in Philadelphia told Reuters Health in an email.

"In fact, there are sufficient skin criteria for SLE so that patients with just skin disease can meet criteria for SLE," she explained. "In our population of CLE patients, most do not go on to get SLE, and those who do more than half the time do not have serious enough disease to require medium to large doses of steroids or immunosuppressive drugs."

Till now there have been mixed reports on the rate of progression of CLE to SLE, and there are minimal data on the severity of systemic symptoms developed by patients who transition from CLE to SLE.

Dr. Werth and colleagues sought to characterize the types and severity of systemic symptoms in a longitudinal study of 77 patients with CLE.

Thirteen patients (17%) went on to meet criteria for SLE, with an average of 8.03 years (and all more than six months) between their CLE diagnosis and their SLE diagnosis, according to the January 29 JAMA Dermatology online report.

Of these 13 patients, nine developed one additional criterion and four developed two additional criteria to go on to be called SLE.

Additional criteria included hematologic involvement in four, arthritis in three, malar rash in three, renal involvement in two, photosensitivity in two, oral ulcers in two, and discoid rash in one. No patient had cardiopulmonary or neurologic involvement.

Four patients diagnosed with SLE went on to develop new musculoskeletal involvement during follow-up and four went on to develop new renal disease.

All 64 patients who remained with CLE had only mild new systemic involvement, whereas 62% of those who developed SLE had mild additional systemic involvement and 38% of those with SLE developed moderate to severe additional systemic disease.

Baseline antinuclear antibody (ANA) titer was the only characteristic that differentiated those who still had CLE from those who went on to develop SLE. People with CLE only more often had a negative ANA titer at baseline.

"Patients with CLE do need to be monitored regularly to assess whether they have new signs, symptoms, or lab findings such as new-onset low complement levels, high dsDNA levels, or proteinuria that is new or increasing in amount," Dr. Werth said. "However, progression to a diagnosis of SLE does not automatically indicate that the patient will have moderate or severe disease. Many patients meet criteria for SLE because of their skin findings and laboratory findings that do not require treatment."

"Overall, it is important to reassure patients that those with predominantly skin disease do not automatically progress to moderate to severe systemic lupus," Dr. Werth concluded. "That is often a major concern for patients, and the message is to monitor patients but communicate that even if they meet criteria for SLE that there is a huge spectrum for the degree of severity that is observed, with the majority having mild SLE."

Dr. Benjamin F. Chong from University of Texas Southwestern Medical Center in Dallas wrote an invited commentary to accompany this report. He told Reuters Health via email, "Most CLE patients who do progress usually have complaints that are primarily musculoskeletal in nature, and do not experience severe complications of lupus. Thus, in most cases, co-management of the patient's lupus with a rheumatologist is sufficient."

"Thorough review of systems with periodic laboratory tests needs to be performed with each CLE patient to check for signs of progression," Dr. Chong said.

In his commentary, Dr. Chong added, "The Cutaneous Lupus Registry at University of Texas Southwestern Medical Center is prospectively studying patients with CLE who do and do not develop SLE to determine both clinical features and biomarkers that generate a thorough characterization of these high-risk patients. This form of 'personalized' medicine can revolutionize treatment of patients with CLE because those at high risk for progression to SLE could be promptly started on antimalarial therapy, which can delay the onset of SLE."

Dr. Chong told Reuters Health, "CLE patients could be started on antimalarials if they are 1) refractory to topical and intralesional steroid treatment, 2) have several lesions present that makes topical treatment too cumbersome to apply regularly, and/or 3) they have other systemic symptoms (e.g., arthritis) that appear to be consistent with lupus."


JAMA Dermatol 2014.


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