DESTIN, Fla. — Although the strongest predictor of malignancy in dermatomyositis is age, all adult patients should undergo cancer screening, according to a speaker at the Congress of Clinical Rheumatology East annual meeting.
“We have to think about why you cannot just do age-appropriate screening,” Ruth Ann Vleugels, MD, MPH, vice chair for academic affairs at Brigham and Women’s Hospital, and program director of the dermatology-rheumatology fellowship program at Harvard Medical School, told attendees.
“We do not just stop at age-appropriate cancer screening in dermatomyositis — we do a full cancer screening,” Ruth Ann Vleugels, MD, MPH, told attendees. Image: Jason Laday | Healio
To underline her point, Vleugels highlighted a case in which a patient in her 30s demonstrated “quite extensive” breast cancer.
“We do not just stop at age-appropriate cancer screening in dermatomyositis — we do a full cancer screening,” she said.
Rheumatologists are encouraged to work closely with oncology colleagues to determine which immunotherapy regimen could be best suited for these patients. Moreover, guidelines are now available to help manage this population.
“Red flags include older age, refractory disease, extensive disease, cutaneous ulcers, certain antibodies and dysphasia,” Vleugels said.
The next case — in a wide-ranging talk on derm-rheum clinical pearls — described a patient with anti-melanoma differentiation-associated protein 5 (MDA5) dermatomyositis. This condition is marked by inflammatory joint pain primarily in the hands. Painful erythematous macules and gottron’s papules are present in the palms.
“These are MDA5 palms,” Vleugels said. “You have got to flip those palms over and look at them.”
These patients also often demonstrate extensive non-scarring alopecia, according to Vleugels.
“Here is the kicker: they do not have muscle disease to clue you in,” she said. “The skin here really matters, because if we miss the skin, we miss the lung disease.”
Ruth Ann Vleugels
Patients with MDA5 dermatomyositis often have a poor survival curve — often worse than patients with cancer — and it is due to complications from interstitial lung disease (ILD), according to Vleugels.
“If we can just catch these patients early, and treat them early, we can modify their risk,” she said.
Wound-healing medications are essential for patients with MDA5 dermatomyositis. In addition, botulinum toxin injected near the neurovascular bundle in the hand can be effective at managing the hallmark palm involvement.
The future of MDA5 treatment is likely to involve Janus kinase (JAK) inhibition, according to Vleugels. After initial studies of this drug class in patients with dermatomyositis in 2016, tofacitinib (Xeljanz, Pfizer) has become useful for patients with refractory skin disease, she added.
“They are doing beautifully on JAK inhibition,” Vleugels said.
Looking ahead, a phase 3 trial of the novel monoclonal antibody dazukibart (Pfizer) is currently enrolling patients with MDA5 dermatomyositis.
“Why do I need new therapies for dermatomyositis?” Vleugels said.
To answer, she described a woman with refractory disease who has cycled through medications ranging from hydroxychloroquine to JAK inhibition.
“These patients are tough to treat,” Vleugels said. “Even with best therapies available I have not put her in remission yet.”
Sources/Disclosures
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Vleugels RA. Pearls from the Dermatology-Rheumatology Clinic. Presented at The Congress of Clinical Rheumatology East; May 9-12, 2024; Destin, Florida (hybrid meeting).
Disclosures:
Vleugels reports associations with Argenx, Pfizer and Priovant.