Dr. Li Chen: Clinical characteristics, diagnosis, imaging characteristics and treatment of SAPHO syndrome.

SAPHO syndrome is a rare disease, its symptoms are complex, difficult to diagnose, I hope that all rheumatologists through these issues of the article can pay attention to this rare disease, early identification, early diagnosis, early treatment.
Li Chen of Beijing Concord Hospital made a comprehensive introduction to the clinical manifestations and diagnostic standards, imaging evaluation and treatment of SAPHO syndrome.
, SAPHO syndrome clinical characteristics and diagnosis 1, what is SAPHO syndrome? SAPHO syndrome is a special group of clinical syndromes involving bone and skin injuries, which are clinically rare.
SAPHO syndrome are Synovitis (slippery membraneitis), Acne (acne), Pustulosis (pustules), Hyperostosis (bone fat), Osteomyelitis (myelitis) 5 English alphabet abbreviations.
sapho syndrome was first proposed by French scholars in 1987, when S represented the syndrome, not glioartitis.
many doctors will diagnose the disease against these five letters, but in fact this diagnosis is wrong, and do not understand the core of the disease.
core content of SAPHO syndrome is osteoarthritis and myelitis caused by bone sclerosis, can be accompanied by skin performance, the original concept was also put together 2 skin performance and 2 bone performance.
because the concept of the disease is not well understood, the disease is underestimated and is often missed by us.
typical clinical manifestations of 2 and SAPHO syndromes? Let's take a look at a queue study we initiated.
Of the 164 patients we reported, more than 90% had skin manifestations, mainly psoriasis (PPP), which accounted for about 70%, while the risk of severe acne was only 16%, and could be accompanied by psoriasis-like rashes and even possible nail changes.
skin manifestations are not necessary for the diagnosis of SAPHO syndrome, as rashes can occur at any stage of bone damage and can even be missing.
common to SAPHO syndrome: Figures A and B are typical PPP skin loss, but many PPP skin loss is not so typical due to treatment or disease development.
C is a manifestation of merging A changes; Figure D is severe acne.
the proportion of men with SAPHO syndrome is about 1:2, women have good hair, on average around the age of 36 symptoms, the average diagnosis takes 3.6 years.
Men and women also have different morbidity times, women usually have 2 peaks, one in the 30s (usually after the birth), the other peak in the 50s (usually accompanied by severe mid-axis fatigue);
164 patients with baseline information on the age of onset of the characteristics of the statistical chart patients appear clinical bone pain mainly manifested in the front chest pain, easy to misdiagnose as rib cartilitis, etc. , usually this pain is more hidden or suddenly aggravated, the location is more diffuse, not fixed, can be accompanied by shoulder pain, coughing or sneezing aggravation, weight aggravation, some patients can spontaneously relieve or oral non-steroidal anti-inflammatory drugs (NASID) relief.
in addition to the pain in the front chest wall, patients will also appear cervical vertebrae, thoracic vertebrae, lumbar vertebrae pain, usually easily misdiagnosed as cervical vertebral disease, lumbar disc protrusion and so on.
If the spinal cord is affected by lesions patients pain will be very severe, peripheral joints can also be affected but mostly non-aggressive changes, mostly shoulder joints, shin joints are tired, it is easy to misdiagnose as strong spina blinitis.
pathogenesis of SAPHO syndrome is not yet clear.
Currently we believe that it belongs to the category of its own inflammatory syndrome, involving a disorder of the immune system, possibly due to the Th17 cell/regulatory T cell balance is broken, causing the expression of inflammatory factors, and then activating the activity of bone-breaking cells, causing changes in the clinical bone system.
clinical tests, we can see an increase in red blood cell sedation rate (ESR) and C-reactive protein (CRP) during the acute phase.
patients can see abnormal bone metabolic indicators, including elevated β-I. collagen C-end peptides (β-CTX) and decreased osteocalcin levels.
tests of anti-nuclear antibodies (ANA), rheumatoid factors (RF), and HLA-B27 in patients were generally negative.
if the results are positive, consider whether there are other diseases.
diagnosis of SAPHO syndrome with 3, SAPHO syndrome is a huge challenge for many clinicians.
because SAPHO syndrome is a disease spectrum that covers more than 50 disease names like a large umbrella.
SAPHO syndrome umbrella map in 1988, a clinical-based diagnosis (1) bone joint performance and polymer acne and explosive acne or purulent sweat adenitis; (3) bone fat (upper chest wall, limb end bone, spine) accompanied or not accompanied by skin damage, (4) chronic polysexual relapsed myelitis (CMRO) contains the central axis or exosome bone, accompanied or not accompanied by skin damage.
can diagnose SAPHO syndrome if one of the above 4 conditions is met.
But in 1994 Kahn MF and Khan MA proposed three diagnostic criteria for SAPHO syndrome: (1) myelitis with or without multiple lesions;
3 standards above meet 1 and can be diagnosed.
can be seen that there is a great emphasis on pathological diagnosis.
But soon realizing that the disease did not require many invasing tests, Kahn MF made the following revisions at the 2003 annual meeting of the American Rheumatology Society (ACR), re-emphasizing clinical diagnosis to avoid unnecessary invasions: (1) bone and/or joint disease accompanied by palms Osteosters; (2) bone and/or joint diseases are accompanied by severe constables; (3) adult isolated sterile bone fat or osteopathy (except acne bacillus acne) ;(4) chronic relapsed polyseptic myelitis in children.
can be diagnosed by complying with any of the following 4 articles.
15 years have passed and there are no new diagnostic revisions to SAPHO syndrome.
more than 600 patients currently diagnosed through our clinics, we hope to use our experience to come up with new diagnostics.
at this stage it seems likely that giving the disease a score may be a better way to diagnose it.
II, SAPHO syndrome imaging characteristics 1, SAPHO syndrome X-ray flat imaging characteristics We first say the traditional flat, that is, we say X-rays, can only see late lesions, can not do early diagnosis, but the traditional flat tablets for some late lesions and monitoring the progress of the disease is a relatively good choice, such as spinal joint disease, we recommend patients every 2 years, do a full spinal X-ray assessment.
We recommend that if you clinically suspect SAPHO syndrome, the most important thing to do is a full body bone scan, because we are looking at bone lesions, whole body bone scan can find the whole body bone tired.
characteristics can be manifested as abnormally radioactive thick stove on the upper chest wall, the typical image is "bull head signs", if the emergence of "bullhead signs" can be diagnosed, but most patients are not typical bovine signs, clinically only about 20% of patients will appear "bullhead signs."
, the type of bone scan lesions is of great value to the disease.
In 2017, we first proposed at the annual meeting of the American Rheumatology Society (ACR) that bone scans can guide clinical division, and we divided them into chest-lock joints, ribs and spine types based on the results of bone scans, with different clinical characteristics for each type, the least clinical performance of rib types, and the strongest clinical manifestations of spine types.
so at the moment we recommend bone scans as a necessary test.
Figure 1: Different patient bone scans, we can see different degrees of abnormal thickness at the front chest wall, but also can see the cervical, spinal, long bone and outer joints of thick 2, SAPHO syndrome CT imaging characteristics many doctors are accustomed to using chest CT or chest lock joint CT Assessing whether there is bone fat or bone hardening at the chest lock joint, we also evaluated when we first diagnosed the disease, we found that if the bone scan to assess the chest lock joint abnormalities, most patients have chest lock joint CT abnormalities, so slowly we do not use the chest lock joint CT to evaluate bone fat and bone hardening.
we found problems with the patient's spine through bone scans, and pain in the spine is a major concern for many patients, so we reported for the first time around the world using whole spinal CT to assess SAPHO syndrome.
We can see bone damage and bone fat in the chest lock joint during CT evaluation, as well as whether the vertebrae are damaged, hardened and collapsed, and can also be scanned to the crucive joint during the scan, a CT assessment scan can sweep almost all of the mid-axis bone condition.
ct assessment of the whole spine, we see that the most commonly affected areas are the thoracic vertebrae, followed by the lumbar and cervical vertebrae.
study was also selected as a presentation at the North American Imaging Annual Meeting and was awarded the Outstanding Trainer Award.
Figure 2: The CT manifestation of the whole spine of SAPHO syndrome, we can see different degrees of hardening at the front chest wall, while in the spine, especially the thoracic vertebrae we can see collapse, lumbar vertebrae can be seen with damaged secondary bone sclerosis and bone bridge formation.
3: You can see the proportion of spinal injuries to the thoracic spine, followed by the lumbar and cervical vertebrae, in the thoracic and lumbar spines we all see "kiss-like" changes, we call kissing changes.
But what we see through CT examination is a change in results and we can't see where there is disease activity, so to make up for this regret, we have carried out a full spinal MRI, chest lock joint MRI and shin joint MRI assessment SAPHO syndrome.
through the evaluation of nuclear magnetism, we found that bone marrow edema is the performance of the active period and is associated with hypersensitive CRP (HS-CRP), which also improves as the treatment of inflammation improves.
we also determine the intensity and cycle of treatment based on the bone marrow edema observed by the MRI.
our core is to reverse bone marrow edema and avoid fat deposits (fat deposits can cause bone bridge formation, affecting vertebral function).
our nuclear magnetic research was also included in the presentation of the European Radiological Society.
4: The patient's full spinal MRI performance can be seen at different degrees of bone marrow edema and fat deposition at the arrow;
3, SAPHO syndrome PET / CT imaging characteristics finally we look at PET / CT examination, many patients are because of bone pain bone scan suspected tumor, in order to find the original lesions and do PET / CT to eliminate tumors, however, many patients can not find the original lesions, can only find multiple lesions in the bones, so many doctors are very confused about this, but if you think of SAPHO syndrome problem will be solved.
the current literature reports that PET/CT can describe local inflammatory damage, can also identify the activity of saPHO damaged bone damage areas, and can use standard absorption values (the standard uptake value, SUV) to identify the inflammatory process.
we also reviewed the PET/CT performance of 26 cases of SAPHO syndrome and did not find a good correlation between SUV values and inflammation, but found that the disease may have many other manifestations besides the skeletal system.
In particular, abnormalities occur in the lymphatic ring of the mouth and pharynx, individual patients can improve the condition after the removal of tonsils, but also can also show the appearance of lymph nodes, thyroid glands, gastrointestinal tract, lungs of different degrees of abnormal intake, but also suggest that SAPHO syndrome may be a systemic disease.
5: You can see different degrees of musosis lesions, which manifest as an increase in SUV values.
6: We can see that PET/CT can also indicate that the system is suffering, manifested in the oral and pharynx lymphatic ring, lymph nodes, thyroid gland, gastrointestinal tract, lungs to varying degrees of abnormal intake.
4, a brief conclusion to listen to today's introduction, is there a new understanding of SAPHO syndrome? Recommended bone scan when doing the examination, can guide clinical division, if the spine is tired, you can choose the whole spine 3D reconstruction CT to assess the severity, use MRI to assess the degree of activity, you can use X-rays to assess the progress of the spine, PET/CT can eliminate tumors, in addition to the detection of some systems affected performance.
, the imaging assessment of SAPHO syndrome needs to be considered comprehensively, and the appropriate examination method should be selected according to the stage of the patient's disease and the degree of progression of the disease.
3. There is no consensus on the treatment of SAPHO syndrome, most studies are case reports or case series studies, only a few clinical trials, but the sample size is small, there is no placebo control and so on.
Because of its serum rheumatoid factor negative, relatively high risk of central axis joints and palate joints suffering, some scholars believe that the disease can be summarized in the serum-negative spinal joint disease category, is between strong spina bifidos (AS) The disease between psoriasis arthritis (PsA) (Figure 1), so the treatment is based on a serum-negative spinal joint disease treatment plan, which I am currently treating clinically, but I prefer it to be more like a mid-axis PSA.
Figure 1: SaPHO syndrome is currently considered to be a group of diseases between AS and PSA1, SAPHO syndrome treatment principles for SAPHO syndrome first to improve clinical symptoms, including bone pain and rash;
The last time we saw a lot of patients in the imaging assessment, the vertebrae formed a bone bridge, these changes will affect function, bone marrow edema and fat deposits before the formation of bone bridge, as soon as possible to eliminate bone marrow edema, to avoid fat deposits is also important.
we say imaging can guide clinical treatment decisions.
2, drugs currently available for SAPHO syndrome . . . nonsteroidal anti-inflammatory drugs (NSAIDs) . . . hormone drugs . . . antibiotics . . traditional anti-rheumatism course improvement drugs (DMARDs) . . . venous bisphosphonates . . anti-tumor necrosis factor α (Anti-TN).