Source / Disclosures
Seo P. vasculitis: pearls for practice. Presented at the Eastern Clinical Rheumatology Conference; mayo. 12-15, 2022 (Mixed meeting).
Disclosures: The SEO does not report any relevant financial disclosures.
Treatments for rare complications of vasculitis can range from high-dose prednisone to nasal lavage, according to a spokesperson for the Eastern Clinical Rheumatology Congress.
Philip Siu, MD, Massachusetts, director of the Johns Hopkins Center for Vasculitis, offered no bones about the challenges in managing vasculitis.
“A lot of patients who end up in our clinic end up there because no one knows what to do with them,” he said. “But we don’t know what to do with them either.”
Siu covered four complications that may appear in patients – aphthous ulcers, leukocytic vasculitis, hypereosinophilia, and chronic sinusitis.
Aphthous ulcers in herpetiform can occur at 1 mm to 3 mm thick and can be found in clusters on the tongue and palate. However, they often heal within 1 to 4 weeks. Smaller versions of these ulcers can measure 5 mm to 10 mm in size, affect the buccal or oral mucosa and heal within one week without scarring, according to Seo.
“These are the lesions that you won’t see in the clinic,” he said. “It’s small, relatively asymptomatic and heals quickly.”
In the meantime, the main ulcers are larger than 10 mm, affect the mucous membrane, soft palate, pharynx or tongue, heal in more than 4 weeks with scarring.
“Some sores hurt so bad that people don’t eat,” Seo said.
He added that it is important for clinicians to be vigilant about misdiagnosing patients with aphthous ulcers with the more serious Behçet’s disease.
“Bahgat’s diagnosis is a burden,” he said. In fact, what they have is an inflammatory skin condition. Therefore, when they are more serious, I call them complex castles until something appears to them as my delight.”
For primary idiopathic thrush, the treatment regimen is clear: prednisone 60 mg per day for 3 to 5 days.
“About that much prednisone — wait, I can explain to you,” Seo said. He noted that treatment with methotrexate, azathioprine and other strategies “did not work.”
However, prednisone treatment should be used in a “medication in the pocket” strategy, given as soon as symptoms appear. Sometimes patients want to wait until the ulcer becomes more severe, but by then it is too late, according to Seo.
On the topic of leukocytic vasculitis, Siu suggested that it tends to appear in the lower extremities due to pressure and trauma. Although rest and elevation are recommended, compression stockings may also help prevent this skin condition.
The drug treatment of cutaneous vasculitis is dapsone at a dose of 100 mg orally every day. Seo noted that dapsone can be found in 25 mg tablets, which means that if patients are unable to tolerate all 100 mg at a time, they can be divided.
“The 25mg dose is really helpful when it comes to titrating the dose down to 75mg as well,” Seo said.
If dapsone is ineffective or contraindicated, colchicine may work with or without dapsone. Hydroxychloroquine may be effective in this group of patients if they are “on the way to lupus,” Seo said.
Turning to hypereosinophilic and idiopathic hypereosinophilic syndromes, Seo said the myeloproliferative syndromes are characterized by a translocation of FIP1L1-PDGFRA, along with B12 and tryptase. For lymphoproliferative manifestations, note the TCR clones.
Importantly, hypereosinophilia is a “clinical diagnosis” and may not be associated with genetic abnormalities, according to Seo.
With regard to treatment, interleukin-5 inhibition may be effective in most acidic conditions.
Seo said the approach to treating chronic sinusitis is much less than drug therapy. It has been suggested that cleaning the sinuses with a neti pot could ultimately be more effective than ongoing medical interventions.
However, using a Neti pot can be a challenge in itself.
“If you don’t succeed the first time, leave the pot and try again tomorrow,” Seo said. “It may take a few days before you can successfully irrigate your sinuses.”
In the end, routine sinus washing is “much better than drugs,” Seo says. “Using a neti pot is really the only way to control these patients.”