Hydroxychloroquine no longer best treatment for systemic dermatomyositis
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DESTIN, Fla. — Hydroxychloroquine may no longer be the optimal treatment option for systemic dermatomyositis, according to data presented at the Congress of Clinical Rheumatology East.
In her presentation, Avery H. LaChance, MD, MPH, director of the Connective Tissue Diseases Clinic at Brigham and Women’s Hospital, and assistant professor of dermatology at Harvard Medical School, said she has moved on from hydroxychloroquine as a first-line systemic therapy for systemic dermatomyositis.
“The only time I am reaching for hydroxychloroquine is in patients with super mild disease,” she said.
Rather, patients with systemic disease may benefit from intravenous immunoglobulin, Janus kinase (JAK) inhibition or gabapentin to treat itch or pain. Rituximab (Rituxan, Genentech) can be effective in the muscles and lungs, while lenabasum is an emerging option, LaChance added.
“The treatment of skin disease in dermatomyositis really requires a multimodal approach,” she said.
According to LaChance, about 80% of patients with dermatomyositis are so-called “classic patients.”
“This means there is muscle involvement,” she said, adding that the remaining 20% are clinically amyopathic.
More women than men are impacted, with double-peak incidences occurring in both juveniles and then middle-aged individuals.
“There is a much higher concern for cancer in the middle-aged population,” LaChance said.
“Pathogenesis includes genetic factors and then environmental factors that turn patients over into this inflammatory response,” she added.
Unfortunately, diagnostic delays are common, particularly in amyopathic dermatomyositis, according to LaChance. These patients are marked by cuticular dystrophy and periungual erythema.
Gottron’s papules, mid-facial erythema and heliotrope rashes can be found in dermatomyositis patients, according to LaChance.
“To distinguish the Malar rash of cutaneous lupus from mid-facial erythema, the mid-facial erythema is going to cross over that nasal-labial fold a lot of times,” she said. “It is going to hug over the upper eyelids.”
A workup of these patients involves similar labs as cutaneous lupus, along with creatine kinase, lactate dehydrogenase and aldolase. Lungs should also be checked using a pulmonary function test with diffusing capacity of the lungs for carbon monoxide. Malignancy screening is also recommended.
Regarding treatment, LaChance recommended starting with sun protection, topical steroids and calcineurin inhibitors, followed by methotrexate and mycophenolate.
Offering additional dermatology tips to rheumatologists, LaChance said it is important to note that cutaneous lupus may or may not be associated with lupus.
“Cutaneous lupus is two to three times more common than SLE,” she said.
She added that there is a multifactorial pathogenesis that includes genetics, environment, the innate or adaptive immune response, along with demographic factors.
“We see this more in women, and certainly there is a higher prevalence in our Black population,” LaChance said.
There are three major subtypes of cutaneous lupus defined by location and the depth of the pathology, she added. They are acute, subacute and chronic.
“Acute cutaneous lupus erythematosus can wax and wane within hours to weeks,” LaChance said. “Subacute cutaneous lupus lasts longer.”
Chronic subtype cutaneous lupus can manifest as discoid, lupus panniculitis, Chilblains lupus and timid lupus.
For rheumatologists treating a patient with suspected cutaneous lupus, in addition to a review of symptoms and a biopsy, the workup should include anti-nuclear antibodies (ANA), dsDNA, anti-smith, anti-Ro and anti-La antibodies, a complete blood count and a blood area nitrogen test. Creatinine, urinalysis and liver function tests also should be performed.
Regarding treatment, first-line approaches for cutaneous lupus include behavioral modifications like sun protection and smoking cessation. Corticosteroids are next in line, followed by calcineurin inhibitors and then antimalarials such as hydroxychloroquine.
“If you get someone who is not fully clear and who needs a little more love, you may switch them from hydroxychloroquine to chloroquine,” LaChance said.
For more widespread or serious cutaneous lupus, methotrexate and mycophenolate mofetil are next in line. Thalidomide or lenalidomide (Revlimid, Bristol Myers Squibb) can be effective, while azathioprine or dapsone are recommended in pregnant patients.
Meanwhile, oral retinoids, rituximab (Rituxan, Genentech) and belimumab (Benlysta, GlaxoSmithKline) may be used in patients with systemic disease. Emerging therapies in this population include JAK inhibitors and anifrolumab (Saphnelo, AstraZeneca), she added.
“This is the framework of what I use in cutaneous lupus,” LaChance said.
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LaChance A. Dermatology for the rheumatologist: Lupus and dermatomyositis. Presented at: The Congress of Clinical Rheumatology East; May 12-15, 2022; Destin, Fla.
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