Aprelimast Safe, Effective for Recalcitrant Cutaneous Dermatomyositis

Patients with stubborn cutaneous dermatomyositis may find a safe and effective treatment option with Aprelimast (Otezla; Amgen).¹ According to a recently published study in JAMA Dermatology, datashowed it could have the potential to be add-on therapy in patients with the disease.

Apremilast is a phosphodiesterase 4 (PDE-4) inhibitor approved for the treatment of plaque psoriasis, psoriatic arthritis, and oral ulcers associated with Behçet disease. Off-label use of apremilast includes hidradenitis suppurativa, atopic dermatitis, lupus erythematosus, as well as use in alopecia areata has been reported in small studies.

Dermatomyositis (DM) is an idiopathic myopathy that affects the skin, proximal muscles, and other internal organs. Cutaneous DM can present with or without muscle disease. Currently, cutaneous disease in DM has no standardized treatment approach and presents a challenge to medical professionals and patients. Typical treatment includes systemic steroids and steroid-sparing agents (hydroxychloroquine, methotrexate, mycophenolate mofetil, intravenous immunoglobulin, rituximab, calcineurin inhibitors, cyclophosphamide, and azathioprine) as well as Janus kinase (JAK) inhibitors and interleukin 6 (IL-6) inhibitors.

The phase 2a, open-label, single-arm, nonrandomized, controlled trial, enrolled 8 patients with cutaneous dermatomyositis, defined by a cutaneous disease activity severity index (CDASI) score of greater than 5 after treatments with steroids, steroid-sparing agents, or both. The patients were given apremilast 30 mg orally twice daily in addition to their regular ongoing therapies.

The results included:

• 7 patients (ORR, 87.5%) achieved the primary outcome of a decrease in CDASI score of at least 4 points at 3 months after apremilast treatment
• The mean (SD) decrease in CDASI was 12.9 (6.3) points at 3 months (P<.001).
• The patients found apremilast to be a tolerable treatment, with no adverse reaction grade 3 or higher. The most frequent adverse effects included transient headache, nausea, and diarrhea.

In the 6-month, nonrandomized, controlled trial, 2 patients were able to taper their steroid use and 1 patient was able to taper off their steroid-sparing agent, but the use of apremilast as a monotherapy was not achieved. The study suggests that a subset of patients could potentially be taken off steroids or use a lower dose, which would minimize long-term toxic effects associated with steroids.

The authors said this is believed to be the first study to investigate the role of apremilast in DM. They noted limitations to the study, including a small sample size, lack of a control group, and use of stable concomitant therapy. They also said the patients were all white and predominately women, which could limit the generalizability of their results.

References:

1. Bitar C, Ninh T, Brag K, et al. Apremilast in Recalcitrant Cutaneous Dermatomyositis: A Nonrandomized Controlled Trial. JAMA Dermatol. Published online October 5, 2022. doi:10.1001./jamadermatol.2022.3917