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SAPHO (synovitis-acne-pustulosis-hyperostosis-osteomyelitis syndrome), short for glioartitis, acne, pustules, bone fat, myelitis syndrome.
, acne, pustules, bone fat, myelitis syndrome is an abbreviation for the following 5 English words, namely: synovitis( synovitis), acne (acne), pustules (pus) Hyperostosis and myelitis, pustules, bone fat, myelitis syndrome is a chronic disease that mainly affects the skin, bones and joints.
SAPHO mainly includes osteopathy and palm abstules.
age of onset is in youth and middle age.
age of onset is 10 years old and the maximum age is 59.
the ratio of men to women is reported differently.
patients often have bone joint swelling pain, the most often tired is the chest lock joint, chest rib joint, shoulder joint, tier bone, shame bone and so on.
current treatment is based on the treatment of symptoms, the preferred non-steroidal drug treatment.
first described a case of aseptic hypertrophobia in the collarbone with palm abscess in 1967, and more than 250 cases have been reported since then.
Charnot and so on after generalization, take the initials of each lesions, put forward the name of filmitis, acne, pustules, bone fat, myelitis syndrome, and the syndrome of osteoarthritis is divided into acne-related rheumatic manifestations (clumps, outbreaks, sweat adenitis, septic), palm, pustules, thoracic bone fat and chronic recurrence of myelitis.
all patients were tired of chest joints and caused sterile bone hypertrophomatitis.
In 1987, French physician Chalmot and others proposed that palm pustules, purulent sweat adenitis or severe acne accompanied by thick chest rib collarbone, chronic relapsed polysexual arthritis, palate arthritis and other damage collectively known as glioarthritis, acne, pustules, bone fat, myelitis syndrome.
common feature is that skin damage has sterile pustules at some point, and bone damage includes aseptic osteoarthritis, sterile myelitis and arthritis.
kawdi and others noted similar inflammatory cell immersion in the tissues of the skin, bone and membrane, all of which they believe have common causes.
more than a decade, although there have been more and more clinical reports, but the relevant reports on the cause and pathogenesis of research are almost blank.
SAPHO syndrome, in addition to glioarthritis, acne, pustules, bone fat, myelitis expression, is also common in nail injury, and this is closely related to the quality of life of SAPHO patients.
Li Chen of Concord Hospital has been studying SAPHO syndrome for a long time and has a series of research results.
previous clinical observations that Tofacitinib may contribute to the treatment of SAPHO syndrome.
is an oral Janus kinase inhibitor that has previously been shown to be effective against psoriasis nail lesions.
objective: to assess the efficacy and safety of tofatini to treat SAPHO syndrome nail injury.
: Participants received 5 mg tofatib twice a day for a total of 12 weeks.
design, location and participants: This open-label, one-arm, forward-looking pilot study included 13 patients with SAPHO syndrome associated with nail lesions and active palm plant pustules recruited from Beijing Concord Hospital from September 2019 to December 2019.
assessment was completed in March 2020.
analysis began in March 2020.
results and measures: The main endpoint is the percentage change in the nail psoriasis severity index score compared to the baseline at week 12.
end points include the percentage change in palm plant psoriasis area and severity score compared to the baseline.
visual simulation scale scores, overall bone and joint pain scores, dermatological quality of life index scores, and inflammatory markers.
to record adverse events throughout the study.
results: including 13 Asian female patients (average age of sD, 39.7 (12.3 years), all patients completed the study.
week, the nail psoriasis severity index score (median is -67% (IQR), -56% to -77%; P<.001 p mm h iqr> Conclusions and correlations: In this preliminary study, tofatinib significantly alleviated nail lesions and palm plant psoriasis and improved quality of life in patients with SAPHO syndrome.
to conduct other follow-up studies to assess the long-term efficacy and safety of tofatini to treat nails with SAPHO syndrome.
trial registration: China Clinical Trial Registration Number: ChiCTR1900025941.
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