Study validates standard of care therapies in morphea

Standard of care treatments for morphea yielded reasonable success over long-term follow-up, according to a study.

“Prospective studies of the disease course in patients with morphea are lacking, particularly those comparing adults and children,” Jack C. O’Brien, MD, of the department of dermatology at the University of Texas Southwestern Medical Center, Dallas, and colleagues wrote.

In the prospective cohort study, the researchers aimed to assess morphea disease course in 130 adults and children who had been treated with standard of care treatment protocols using validated disease outcome tools.

Eligible participants were culled from the Morphea in Adults and Children cohort, had at least 2 years of clinical follow-up and had Localized Scleroderma Cutaneous Assessment Tool scores recorded at each study visit. Patients seen at UT Southwestern Medical Center between Nov. 1, 2008, and April 1, 2016 — totaling 663 visits in all — underwent analysis.

The cohort included patients with a mean age of 34.4 years; patients were 78% women and 74% white. Participants were followed for a mean of 4.3 years.

A linear subtype of morphea was reported in 55% of the patients, while 31% had a generalized subtype.

Recurrence of disease activity occurred in 43% of 30 patients with generalized subtype and 21% of 66 patients with linear subtype (HR = 3.28; 95% CI, 1.38-7.79).

Following initial resolution of disease activity, the median time to first recurrence was 1.1 years (interquartile range, 0.8-1.9 years) for patients with generalized disease and 2.3 years (IQR, 1.0-3.3 years) for the linear group.

Among 50 patients who had at least 5 years of follow-up data available for analysis, 36% experienced recurrence of disease activity.

Other findings showed that 67% of patients treated with methotrexate and 47% of those undergoing UV-A1 phototherapy demonstrated a full response as assessed by modified Localized Scleroderma Skin Severity Index at 1 year of follow-up.

The researchers noted that sclerosis improved more slowly over a period of 2 to 5 years following discontinuation of treatment. An additional observation was that atrophy increased while sclerosis subsided.

“Standard of care therapy appears to improve disease activity, which allows sclerosis to improve, and provides relative stability of other features of disease damage,” the researchers said. “Patients with morphea should be monitored for recurrent disease activity over extended periods.”

Carla N. Cruz-Diaz, MD, and Anna K. Haemel, MD, both of the department of dermatology at the University of California, San Francisco, suggested in an accompanying editorial

that specific demographic and clinical variables may have altered treatment response in this cohort.

“For example, patients with linear morphea were more likely to achieve disease control at 1 year vs. those with generalized morphea,” they wrote. “Sclerosis within lesions of the upper extremities also improved more quickly than in lesions of the trunk and lower extremities.”

Of particular note was that flares occurred in more than one-third of patients over 5 years of follow-up.

“In addition, treatment with UV-A1 phototherapy was associated with a shorter time to recurrence after initial disease control than treatment with methotrexate,” they wrote. “Although this result may be partially explained by the longer active treatment phase for methotrexate vs. UV-A1 phototherapy, these findings suggest that additional studies of a more extended UV-A1 phototherapy course are warranted.”

Other important findings suggest that O’Brien and colleagues were able to map the anatomic location of recurrent lesions, according to Cruz-Diaz and Haemel. Results showed that most of these occurred at the site of prior lesions. “This finding may help focus targeted examination for active disease in the clinic,” they wrote.

Regarding the phenomenon of improved sclerosis and worsening atrophy, Cruz-Diaz and Haemel suggested that this information may help patients understand the lesion healing process.

“Overall, the study by O’Brien et al is reassuring in that patients largely responded to standard treatments for morphea,” they wrote. “However, the tendency of more than one-third of patients to relapse over time highlights the need to clarify an optimal approach to long-term management.” – by Rob Volansky

Disclosures: O’Brien and Cruz-Diaz report no relevant financial disclosures. Please see the reports for all other authors’ relevant financial disclosures.