‘A little bit unique’: Consider every SAPHO manifestation when examining patients
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SCOTTSDALE, Ariz. — SAPHO is complex, but knowing its potential manifestations can help clinicians identify and help patients, according to a speaker at the Basic and Clinical Immunology for the Busy Clinician meeting.
SAPHO, or Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis, is defined by the skin and joint manifestations in its namesake.
“In rheumatology, we talk about synovitis a lot, but in SAPHO syndrome, it is a little bit unique,” Adam Brown, MD, of the Cleveland Clinic, told attendees during the hybrid meeting. “It primarily loves the chest, and it causes swelling, stiffness and pain.”
In SAPHO, there is not typically a lot of synovitis involvement among the periphery, according to Brown. Additionally, it is common for skin manifestations to present before any joint manifestations, he added.
“The next thing to talk about is the skin manifestations, which often, importantly, precede the joint manifestations,” Brown said. “And again, the skin manifestations can just be by themselves and not related to SAPHO syndrome.”
Following synovitis, physicians should assess patients for acne.
“The acne is usually pretty severe, often in the face and often presenting for years prior to the diagnosis,” Brown said. “Again, acne is not uncommon, but the acne in SAPHO tends to be pretty severe.”
Meanwhile, contrary to the synovitis presentation, when clinicians are looking for pustulosis, they should look toward the periphery and examine hands of the patients, he added.
“The palmar pustulosis can be pretty awful,” Brown said. “The patients can have these sterile lumps and masses on their hands that can be very painful. They can also exhibit the Koebner’s phenomenon, where if they touch or grab something, that can worsen the symptoms and cause more pain.”
Finally, Brown urged providers to pay attention to the final two letters of the acronym: hyperostosis and osteitis.
“Osteitis is inflammation of the bones,” Brown said. “Osteitis, again, is not unique to SAPHO syndrome — a lot of different inflammatory conditions can cause osteitis.”
Hyperostosis, meanwhile, is “a little bit different,” he added. A reactive bone growth, this sign is typically pretty pronounced, according to Brown.
“Importantly, what is not often recognized is that hyperostosis often takes a while to develop,” he said. “Sometimes a patient can have a sort of swelling and pain in one of the joints in the chests, and when an X-ray is done, it is normal.”
When examining a patient and considering SAPHO syndrome, Brown said it is important to recognize that not all manifestations of these signs points to SAPHO syndrome.
“All the manifestations and all of the letters of the acronym can kind of be seen by themselves,” he added. “They do not necessarily need to be seen in SAPHO syndrome.”
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Brown A. Managing SAPHO: Is it just a PsA variant? Presented at Basic and Clinical Immunology for the Busy Clinician, Feb. 25-26, 2023; Scottsdale, Arizona (hybrid meeting).
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