‘Pearls and myths’: Oral, cutaneous signs of rheumatic disease hold ‘clue’ to intervention
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Managing oral, ocular and cutaneous complications is critical to optimizing outcomes in conditions ranging from Sjögren’s syndrome to juvenile dermatomyositis, according to a presenter who offered a cross-section of “pearls and myths” at ACR Convergence 2021.
John H. Stone, MD, MPH, director of clinical rheumatology at Massachusetts General Hospital, gave a rapid-fire rundown of “pearls and myths” for attendees. Starting with the mouth, he noted that Sjögren’s disease can manifest as a “red and beefy” tongue. “This is another important clue to an intervention that will help the patient,” he said.
Another pearl is that oral candidiasis can occur in patients with Sjögren’s disease, according to Stone. “It is important to remember to ask patients about a burning sensation on the tongue,” he said. “This is candidiasis.”
A common myth in this patient population is that a white discoloration of the tongue is also candida. “This is hyposalivation,” Stone said.
Clinicians managing lupus should also keep oral lesions on the radar. “A myth is that most oral lesions in lupus are painful,” Stone said. “In fact, most oral lesions in lupus are painless.”
It is for this reason that rheumatologists should actually look inside the mouths of their patients with lupus. “There is a lacy, reticular pattern to these lesions,” Stone said.
Rheumatologists who encounter a strawberry-like overgrowth on the gums should consider one diagnosis: granulomatosis with polyangiitis (GPA), according to Stone. But these are not the only unusual complications of GPA. “If you suspect that the patient has GPA, look for Churg-Strauss granulomas,” he said, noting that these frequently occur on the elbows.
Moving from the elbows to the fingers, Stone said that a major myth is that patients with systemic sclerosis all have Raynaud’s phenomenon and are positive for antinuclear antibodies. This, however, is not the case.
Looking at pediatric populations, Stone covered some myths and pearls surrounding macrophage activation syndrome (MAS), a “feared complication” of juvenile idiopathic arthritis. “The ferritin to [erythrocyte sedimentation rate] ratio helps distinguish JIA flares from MAS,” Stone said. If this ratio reaches greater than 21.5, swift intervention is critical. “We should not be timid when treating cytokine storm syndrome with interleukin-1 targeting agents.”
Turning to juvenile dermatomyositis, Stone offered the important pearl that myositis-specific autoantibodies found in the juvenile form of the disease are different from those found in adults.
Also in juvenile dermatomyositis, it is critical to treat the skin disease as aggressively as possible. “Dilated capillaries in the eyelids are the last cutaneous manifestation to resolve,” Stone said. “You should not stop treatment until this is resolved.”
Moving back to adult populations, Stone dispelled the myth that Takayasu’s arteritis always requires immunosuppression. “You have time reduce flares with glucocorticoids or IL-6 targeting therapies,” he said. “Takayasu is a slow disease.”
An additional pearl about Takayasu’s arteritis is that a stent should only be administered after “careful thought and consideration,” according to Stone. He suggested that stenting is “irrelevant” most of the time.
The final Takayasu-related pearl pertains to bruits. “Listen for bruits on the back,” Stone said. “They could be coming from one of the pulmonary arteries. The descending aorta can also be a site of a Takayasu lesion that leads to a loud bruit over the back.”
Stone’s final pearls pertained to Erdheim-Chester disease. He noted that foamy histiocytes throughout the tissue embedded in other types of inflammation are the “giveaway” to this condition. Clinicians are encouraged to perform a PET scan to confirm this diagnosis, however, because while many patients have lesions in the long bones, it can be difficult to spot for one key reason. “Only about 50% are symptomatic,” he said.
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Stone J. Abstract #7S120. Presented at: ACR Convergence 2021; November 5-9, 2021 (virtual meeting).
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