Cutaneous Signs of Connective Tissue Disease

"Recognizing early signs of connective tissue disease is crucial for aggressive treatment and considering novel therapies," said Dr Vleugels during her presentation on “Hot Topics from the Connective Tissue Disease Clinic” at Dermatology Week 2023. "We need to be able to treat patients with extensive disease who have failed traditional therapies, and anifrolumab may be a viable option."

Ruth Ann Vleugels, MD, MPH, MBA, is the vice chair for academic affairs of the Brigham and Women’s Hospital department of dermatology in Boston, MA. She serves as the director of the autoimmune skin disease program at Brigham and Women’s Hospital and is codirector of the rheumatology-dermatology program at Boston Children’s Hospital.

"Anifrolumab has shown promising results in patients with mild to moderate skin disease activity," Dr Vleugels indicated. "Our experience with 10 patients with treatment-refractory cutaneous lupus has shown a mean decrease in their CLASI [Cutaneous Lupus Erythematosus Disease Area and Severity Index] score of 17."

"It is exciting to see such dramatic improvement in patients who have failed multiple therapies," she remarked. "Anifrolumab may be a game changer for patients with treatment-refractory discoid lupus."

Dr Vleugels stated, "MDA-5 dermatomyositis is a dermato-pulmonary syndrome, and patients tend to lack muscle disease but almost always have interstitial lung disease. It is really critical to diagnose this early and try to prevent deaths in these patients."

"Hydroxychloroquine is not particularly effective in our dermatomyositis patients. If you have a patient who walks in with moderate to severe dermatomyositis, I want you to think about starting another agent," she emphasized. "Intravenous immunoglobulin led to the FDA approval of IVIG for dermatomyositis last year.”

Dr Vleugels shared these clinical pearls for Janus kinase (JAK) inhibitors:

• Many skin diseases warrant an increased dose but consider negative side effects.
• They are safe to combine with methotrexate.
• Consider the recombinant zoster vaccine and varicella titers.

Referring to a patient who showed dramatic improvement after being treated with JAK inhibitors, she noted, “I have been able to use this in younger patients."

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Reference
Vleugels RA. Hot topics from the connective tissue disease clinic. Presented at: Dermatology Week 2023; May 3-6, 2023; Virtual.