Le syndrome SAPHO

SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome is a rare entity characterized by the association of heterogeneous osteoarticular and cutaneous manifestations that have for common denominator an aseptic inflammatory process. The etiopathogeny of this disease is still a matter of debate. Although it has been related to the spondylarthritis family, an infectious origin is suggested. Diagnosis is based on the presence of at least one of the three diagnostic criteria proposed by Kahn. The treatment includes NSAIDs, antibiotics, corticosteroids, methotrexate and more recently the bisphosphonates and the TNFα inhibitors.     

Pamidronate for SAPHO syndrome

SIR, Marshall et al. [1]     

SAPHO syndrome and pamidronate revisited

SIR, We read with interest the paper of Amital et al. [1] andwould like to comment on it. Since our unit was set up (1984)we have diagnosed 40 patients with the     

An infrequent presentation of SAPHO syndrome

We describe a case of SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) with an unusual presentation in a 14 year-old girl with low grade fever which lasts 8 months, left low back pain and elevated erythrocyte sedimentation rates and protein C-reactive with chronic anaemia. A radiograhy of the lower limbs showed a lytic image with osteitis and hyperostose in the right fibula, as a casual finding. This information, in addition to the acne, pustulosis, sternoclavicular arthritis and the studies got from the magnetic resonance image (MR) of spine, pointed out the diagnosis of SAPHO.     

Pustulotic arthro-ostitis (SAPHO syndrome)

The sterile arthro-osteitis, especially in anterior chest wall, and also in spine and peripheral joints were observed in patients with pustulosis palmaris et plamtaris. These conditions were named pustulotic arthro-osteitis. Furthermore, the SAPHO syndrome, which stands for synovitis, acne, pustulosis, hyperostosis and osteitis, has been proposed in order to describe more varieties of bone, joints and skin lesions.     

Frontal bone involvement in SAPHO syndrome

Clinical Rheumatology -     

SAPHO syndrome or psoriatic arthritis? A familial case study.

OBJECTIVE: To discuss the relationships between SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome and the group of spondylarthropathies. METHODS: Few reports of familial SAPHO have been published. We describe three children, two sisters and one brother, whose clinical and radiological presentation was in accordance with SAPHO syndrome. RESULTS: Two children developed psoriasis, and one child palmoplantar pustulosis. Both sacroiliac and sternoclavicular joints were involved in these three cases. Some features in our observations are also common to psoriatic arthritis. No association was found with HLA antigens, but a history of trauma preceding the onset of symptoms was present in all three children. CONCLUSIONS: We can consider that SAPHO is nosologically related to spondylarthropathies. Psoriatic arthritis could be the missing link between SAPHO and spondylarthropathies. It is likely that both genetic and environmental factors are involved.     

Osteitis as a symptom of SAPHO syndrome

SAPHO syndrome is an acronym given to a spectrum of diseases with the following features: synovitis, acne, pustulosis, hyperostosis and osteitis. The main problem of the differential diagnosis in 3 cases presented in our paper was the aseptic osteitis.     

Mucosal lesions may be a minor complication of SAPHO syndrome: a study of 11 Japanese patients with SAPHO syndrome

Since the term synovitis–acne–pustulosis–hyperostosis–osteitis (SAPHO) syndrome was proposed by Chamot et al. (Rev Rhum Mal Osteoartic 54:187–196, 1987), clinical reviews concerning this syndrome have been mainly reported from Europe. We carried out a retrospective analysis of 11 Japanese patients with SAPHO syndrome, and reviewed the clinical features of our series in comparison with those in a European large case study. In this study the major features of SAPHO syndrome were chronic osteitis of the anterior chest wall and pustulotic arthro-ostitis with middle age onset, and mucosal lesions seemed to be a minor complication of SAPHO syndrome. The non-erosive peripheral large joints arthritis and the particular HLA types (HLA-B51, B52, or A26), which had been reported to be increased in Behcet’s disease, were frequently seen in SAPHO syndrome with mucosal lesions. This study also suggests that SAPHO syndrome with mucosal lesions may be part of a broader disease spectrum including Behcet’s disease.     

Entheseal ultrasound abnormalities in patients with SAPHO syndrome

This study was conducted to investigate the presence and characteristics of the ultrasound lesions that may be found in the entheses of patients with SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome. This cross-sectional study included 15 patients with SAPHO syndrome and 30 healthy controls matched for age, sex and body mass index. Subjects with regular sport activities as well as those with other rheumatic conditions were excluded from the study. Ultrasonography was used in both groups to study 14 entheses of the upper and lower extremities. Different elementary lesions representative of enthesis damage were defined. A total of 210 entheses in the study group and 420 in the control group were evaluated. Only one patient presented clinical enthesitis. In the study group, seven of the 15 patients (47%) showed morpho-structural entheseal alterations, versus only four of the 30 controls (13.3%; p?相似文献   

Methotrexate to treat SAPHO syndrome with keloidal scars

SAPHO syndrome is an uncommon clinical entity, recently described in literature, which usually affects children, young adults and middle-aged people. It is defined by the association of skin lesions (severe acne, palmo-plantar pustulosis, supurative hidradenitis), synovitis, hiperosthosis and osteitis; however, not all manifestations are required for correct diagnosis. We report a currently forty-two year-old man who initiated follow-up in 1992 for severe acne. His diagnosis changed along the years and has been treated with antibiotics many times to control pustule formation and hidrosadenitis, which evolved to keloidal scars, requiring neck zethaplasty due to limitation of mobility. In 1996 isotretinoin was started, with good response to treatment but recurrence after its completion. Ten years later, isotretinoin was being used again due to worsening clinical picture when the patient developed polyarthritis, lost 11kg in 3 months and was found to have an elevated erythrocyte sedimentation rate. SAPHO syndrome was then diagnosed by the Rheumatology clinic, which started methotrexate at 10mg per week to a good clinical response. Several cutaneous and articular flares have occurred since, however he is now clinically stable with methotrexate 20mg per week and finasteride 5mg per day. SAPHO syndrome is a rare disease and its diagnosis is often late and difficult. In this case, skin lesions preceded arthritis in several years and have been resistant to other common treatments. The patient had a better improve after initiation of methotrexate. Although the skin disease has evolved to keloids, the patient has had excellent treatment response.     

Successful treatment of SAPHO syndrome with zoledronic acid

The SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis) is a chronic, relapsing, inflammatory condition with skin and osteoarticular manifestations. Its etiology remains unclear, and various treatment regimens with steroids and nonsteroidal antiinflammatory drugs frequently fail to control the disease, while exposing patients to the side effects of these drugs. Because the SAPHO syndrome manifests as a destructive inflammatory bone disease, use of bisphosphonates that possess antiosteoclastic and probably antiinflammatory properties has been suggested to be helpful. To our knowledge, this is the first reported case of successful treatment with zoledronic acid of SAPHO syndrome that was resistant to conventional treatment.