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Objective:To evaluate the changes and significance of bone turnover makers in patients with SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome.Methods:Thirty-two patients with SAPHO syndrome who were treated in the department of rheumatology and immunology of Beijing Jishuitan Hospital were collected as the study group, and 28 healthy subjects were taken as the control group. The clinical data and bone turnover markers were compared between the two groups, and the correlation between the bone turnover markers and disease-related indicators were analyzed. Two independent samples were comp-ared by using t test (in line with normal distribution) and rank sum test (not in line with normal distribution). The results of more than two independent samples were compared by one-way analysis of variance, and the correlation between two variables was analyzed by Spearman. Results:Compared with the clinical data of the two groups, hemoglobin (Hb) of the study group [128(122, 140) g/L] was significantly lower than that of the control group [139(125, 154) g/L]( U=306.5, P<0.05), but alkaline phosphatase (ALP) [75.00(66.00, 85.75) mmol/L] was significantly higher than that of the control group [56.00(48.50, 63.25) mmol/L] ( U=153, P<0.01). The comparison of bone turnover markers showed that serum total procollagen type 1 amino-terminal propeptide (tP1NP)[52.51(41.72, 86.11) ng/ml], Beta C-terminal cross-linked telopeptides of type Ⅰ collagen (β-CTX) [0.57(0.39, 0.72) ng/ml] and OC [(20±8) ng/ml] levels in the study group were significantly higher than those in the control group [34.91(28.97, 42.80) ng/ml, 0.34(0.27, 0.49) ng/ml, (15±4) ng/ml] ( U=183, P<0.01; U=223, P<0.01; t=3.180, P<0.01). There was no significant difference in 25-(OH)VD 3 between the two groups [14.73(12.25, 19.23) ng/ml, 16.72(11.74, 20.92) ng/ml] ( P>0.05). The serum biochemical markers of bone turnover were not related to spine, bone and joints involvement. The grouping results of the number of joints involved showed that there were significant differences in serum osteocalcin (OC) levels of patients ( F=3.684, P<0.05), and the more the number of joints involved, the lower the OC value. Correlation analysis showed that serum tP1NP ( r=0.805), β-CTX ( r=0.460) and OC levels were positively correlated with each other(all P<0.01). Levels of tP1NP, β-CTX, OC, and 25-(OH)VD 3 in the study group were not related to age, course of disease, and neutrophil granulocyte (all P>0.05). However, β-CTX was positively correlated with C-reactive protein (CRP) ( r=0.392, P<0.05), and OC was positively correlated with erythrocyte sedimentation rate (ESR) ( r=0.475, P<0.05). Conclusion:The levels of tP1NP, β-CTX and OC in patients with SAPHO syndrome are significantly increased. Levels of β-CTX and OC can reflect the severity of patients' inflammation. The level of OC is related to the number of joint involvement of the patient.
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Objective To improve the recognition of synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome by investigating its clinical features and imaging manifestations. Methods The data of 14 patients with SAPHO syndrome (8 males, 6 females, age range: 18-61 years) between January 2014 and January 2017 were retrospectively analyzed. The clinical features, the results of laboratory (erythrocyte sedimentation rate (ESR), C reaction protein (CRP), complement component 3 (C3) and component 4 (C4), human leukocyte antigen (HLA)-B27) tests, pathology and imaging examinations (CT and whole-body bone im-aging) were collected. Results SAPHO syndrome was mainly characterized by skin damage and bone joint damage. The main manifestations of skin lesions were pustules, desquamation and erythema. Those of bone and joint injury were sternocostoclavicular hyperostosis, stiff spinal hyperostosis, peripheral arthritis, and sacroiliitis. There were 5 with fasting ESR, 5 with abnormal CRP, 3 with abnormal C3, and 1 with abnormal C4 among 14 patients. HLA-B27 was negative in all patients. Whole-body bone imaging revealed that 14 pa-tients had abnormal radioactivity, mainly involving the anterior chest wall, spine, pelvis and long bone. Chest CT manifestations of the patients were the destruction of joint bone, the fusion of the hypertrophy and the narrowing of the joint space. Symmetric lesions accounted for the majority. Pathology examination showed non-specific inflammation in 9 patients. Conclusions SAPHO syndrome is a disease characterized by skin, bone and joint abnormalities. Whole-body bone imaging can find systemic bone damage in early stage, and CT can detect bone changes and soft tissue lesions.
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Objective To explore the initial symptoms of synovitis,acne,pustulosis,hyperostosis,osteitis (SAPHO) syndrome,and to analyze the clinical and laboratory characteristics of these patients.Methods We retrospectively analyzed the initial symptoms of 164 patients diagnosed with SAPHO syndrome presented at Peking Union Medical College Hospital from 2004 to 2015,and their clinical,laboratory,and radiological data were collected.The t test,Mann-Whitney U test and chi-square test were used to compare the clinical differences between the SAPHO patients with different initial symptoms.Results Among the 164 patients recruited,84(51.2％) had skin lesions before osteoarticular symptoms,whereas 29(17.7％) after and 42(25.6％) simultaneously.Nine (5.5％) patients had no skin lesions.The time interval between onset of skin and osteoarticular lesions was less than 2 years in 133 (81.1％) patients,but up to 35 years at most.Interestingly,a significantly higher age at onset was observed in patients with osteoarticular symptoms prior to skin lesions than those after [(41 ±10) years vs (36±11) years,t=-2.174,P=0.032].Moreover,positive HLA-B27 was more frequently detected in patients having osteoarticular symptoms as the initial presentations (10.3％ vs 0,P=0.016).Although treated more aggressively before baseline,patients presented with osteoarticular symptoms prior to skin lesions had significantly higher level of hs-CRP at baseline compared with those after [5.42 (1.88,12.70) mg/L vs 11.60 (3.76,22.08) mg/L,Z=-2.096,P=0.036].Conclusion Skin lesions tend to appear prior to osteoarticular symptoms in most SAPHO syndrome patients.The percentage of patients who developed skin lesions after osteoarticular symptoms increase with age at onset.
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Objective To investigate the distribution of age at onset and its influence on clinical characteristics in synovitis,acne,pustulosis,hyperostosis,and osteitis (SAPHO) syndrome.Methods We recruited 164 patients with SAPHO syndrome who presented to Peking Union Medical College Hospital from Jan 2004 to Mar 2015.All the patients were assessed for medical history,laboratory tests and imaging presentations.The distribution of age at onset was analyzed using Shapiro-Wilknormality test and Kolmogorov-Smimov test for mixed normal distribution.The influence of age at onset on clinical features was analyzed using Mann-Whitney U test and x2 test.Results A double-peak mixed normal distribution of age at onset of skin lesions was found in female patients with SAPHO syndrome,with means and standard deviations of (30±6) years (early-onset) and (51 ±7) years (late-onset) for each mixed normal distribution.The cut-off point was determined to be 42 years old.Nonetheless,a typical single-peak normal distribution of age at onset of skin lesions was observed in male patients.A significantly higher frequency of thoracic region pain [14/36 (38.9％) vs 6/70 (8.6％),x2=14.28,P＜0.01,spinal lesions revealed by bone scintigraphy [23/35 (65.7％) vs 23/66(34.8％),x2=8.79,P=0.003],and peripheral skeletal lesions revealed by bone scintigraphy [17/35 (48.6％) vs 17/66(25.8％),x2=5.33,P=0.021] were found in late-onset female patients compared with early-onset ones.Moreover,female patients with late onset had significantly higher hs-CRP level [(12±12) mg/L vs (9±11) mg/L;U=911.5,P=-0.042)],pain VAS (4.8±1.8 vs 4.0±2.1;U=948,P=0.036),and BASFI (3.0±2.2 vs 1.8±2.0;U=822.5,P=0.003) at baseline than those with early onset.Conclusion Female patients with SAPHO syndrome have a double-peak distribution of age at onset of skin lesions.Female patients with early and late onset of skin lesions exhibit distinct clinical characteristics.
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Objective To use cluster analysis to explore the clinical phenotypes of Synovitis-Acne-Pustulosis-Hyperostosis-Osteitis (SAPHO) syndrome.Methods One hundred and sixty-four patients fulfilled the Kahn and Khan's criteria for SAPHO syndrome were recruited in Peking Union Medical College Hospital from 2004 to 2015.For all patients,demographics,clinical,laboratory and imaging data were collected.Cluster analysis was performed using an iterative partitioning K-means method including 11 variables that was most characteristic in patients with SAPHO syndrome.The optimal number of clusters was determined by the elbow method and Silhouettes coefficient in combination with clinical significance.Results An optimal of two phenotypes with distinct clinical features were identified.Cluster 1 was characterized by axial skeletal involvement with older age at onset [(38±11) years] and lower prevalence of severe acne (11.2％);Cluster 2 had no axial involvement with younger age at onset [(33±8) years;U=1 800,P=0.010] and higher prevalence of severe acne (26.8％;x2=4.567,P=0.033).Cluster l patients had been treated more aggressively by baseline compared with Cluster 2 patients;and were more frequently prescribed TNF-α inhibitors (32.8％ vs 2.4％;x2=1 672.5,P＜0.01) and bisphosphonates (39.7％ vs 19.5％;x2=1962,P=0.032).Nonetheless,the disease activity indices were significantly higher at baseline in Cluster 1 than Cluster 2 patients [Bath ankylosing spondylitis disease activity index (BASDAI) (3.5±1.8) vs (2.8±2.0);U=1 800,P=0.010] [Bath ankylosing spondylitis functional Index(BASDFI) (2.4±2.3) vs (1.5±1.7);U=1 791,P=0.009).Moreover,Cluster 1 patients had significantly increased inflammatory markers at baseline compared with Cluster 2 patients [erythrocyte sedi-ment-ation rate(ESR) (34.9±2.9) mm/1 h vs (19.0±14.6) mm/1 h;U=1 204.5,P＜0.01] [high-sensitivity C-reactive protein (hs-CRP) (16±19) mg/L vs (8±11) mg/L;U=1 628,P=0.01].Conclusion Char-acterized by the presence or absence of axial skeletal involvement,two disease subtypes exist in SAPHO syndrome,which exhibit distinct features in age at onset,the prevalence of severe acne,and disease severity.
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ABSTRACT Autoinflammatory disorders are immune-mediated diseases with increased production of inflammatory cytokines and absence of detectable autoantibodies. They course with recurrent episodes of systemic inflammation and fever is the most common symptom. Cutaneous manifestations are prevalent and important to diagnosis and early treatment of the syndromes. The purpose of this review is to emphasize to dermatologists the skin symptoms present in these syndromes in order to provide their early diagnosis.
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Humans , Skin Diseases/etiology , Autoimmune Diseases/complications , Autoimmune Diseases/diagnosis , Inflammation/complications , Inflammation/diagnosis , Skin Diseases/immunology , Inflammation/immunologyABSTRACT
Objective To investigate whether the proportions of Th17/Treg balance were impaired in the peripheral blood of patients in different phases of synovitis acne pustulosis hyperostosis osteitis (SAPHO) syndrome.Methods We studied 22 cases diagnosed as SAPHO syndrome and 11 healthy controls.According to the scores of VAS pain,BASDAI and BASFA,the 22 patients were divided into active group and stable group.By means of flow-cytometry,the frequencies of total and different subsets of Th17 and Treg cells in peripheral blood mononuclear cell of different groups of SAPHO syndrome and healthy controls were studied.The values of Th17/Treg balance were analyzed.The relationship was analyzed with Kruskal-Wallis test,Mann-Whitney test and Pearman's test.Results The mean percentage of Th17 cells was markedly higher in the active group [(2.74±0.25)％] than in the stable group [(1.16±0.09)％] (U=0.000,P＜0.01) and healthy controls [(1.13±0.11)％] (U=0.000,P＜0.01).No differences were found among active group [(2.10±0.20)％],stable group [(2.51±0.20)％] and control group [(2.44±0.22)％] (x2=2.16,P=0.339 4).The ratio of Th17 cells to Treg cells was markedly higher in active group [(1.48±0.25)％] than in the other two groups [(0.47±0.03)％] (U=0.000,P＜0.01).We also found the positive correlation of the ratios of Th17/Treg cells with the values of VAS in SAPHO syndrome patients (r=0.752 7,P＜0.01).Conclusion The results demonstrate that the development of SAPHO syndrome is closely related to the imbalance of systemic Th17/Treg cells,Increased ratio of Th17/Treg cells may be the main factor that cause disease recurrence,and then,lead to the manifestations of high levels of inflammation and joint pain.
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SAPHO syndrome, characterized by synovitis, acne, pustulosis, hyperostosis, and osteitis is rare compared to other spondyloarthropathies. It is also difficult to diagnose, and treatment methods have not yet been fully identified. Approximately 72% of patients are diagnosed with at least one other disease before a final diagnosis of SAPHO syndrome. In addition, SAPHO syndrome is subject to a delayed diagnosis period of 4.5 to 9.1 years. Medications such as non-steroidal anti-inflammatory drugs, disease-modifying anti-rheumatic drugs, and tumor necrosis factor inhibitors are used in treatment of SAPHO syndrome. Bisphosphonate is also used for refractory SAPHO syndrome; however, most reports on this relate to intravenous injection of medication. The authors experienced and subsequently reported on a case involving a patient with SAPHO syndrome accompanied by fracture and infection of the left second finger who was treated with the oral biphosphonate, alendronate.
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Humans , Acne Vulgaris , Acquired Hyperostosis Syndrome , Alendronate , Antirheumatic Agents , Delayed Diagnosis , Diagnosis , Fingers , Hyperostosis , Injections, Intravenous , Osteitis , Spondylarthropathies , Synovitis , Tumor Necrosis Factor-alphaABSTRACT
SAPHO syndrome is a rare inflammatory, pseudoinfectious disease. Initially it was an acronym for Syndrome Acne Pustulosis Hyperostosis Osteitis, and the meaning of S was later changed to synovitis. It occurs predominantly in children and adults and is not common over 60 years. The most common clinical presentation is osteoarticular involvement at the anterior chest wall and skin manifestations may be evident, but it could occur years earlier or develop later. We report on two cases of mother and daughter. A 51-year-old female was diagnosed with SAPHO syndrome with costochondritis and palmoplantar pustulosis. Five years later, her 31-year-old daughter presented with similar skin manifestations of the hand and foot.
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Adult , Child , Female , Humans , Middle Aged , Acne Vulgaris , Acquired Hyperostosis Syndrome , Foot , Hand , Hyperostosis , Mothers , Nuclear Family , Osteitis , Psoriasis , Skin Manifestations , Synovitis , Thoracic WallABSTRACT
El síndrome Sapho (sinovitis, acné, pustulosis, hiperostosis, osteítis) es una condición crónica e inflamatoria con manifestaciones cutáneas y osteoarticulares. Su etiología no es clara, aunque se ha considerado una posible asociación con las espondiloartritis. Diferentes tratamientos con esteroides y medicamentos antiinflamatorios son utilizados en esta patología, con respuesta variable. Los bisfosfonatos son nuevas modalidades en el tratamiento del síndrome de Sapho, gracias a sus propiedades antiosteoclásticas y antiinflamatorias. Se reporta el caso de un paciente con Sapho exitosamente tratado con ácido zoledrónico.
SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis) is a chronic and inflammatory condition, with cutaneous and osteoarticular manifestations. Its etiology is still unclear, although a possible association has been considered between this syndrome and spondylarthropathies. Several types of treatment with steroids and anti-inflammatory drugs are currently used for this condition, obtaining different results. Biphosphonates are a new treatment option for SAPHO syndrome due to their anti-osteoclastic and anti-inflammatory properties. We report the case of a male patient with SAPHO syndrome, who was successfully treated with zoledronic acid.
A síndrome SAPHO (sinovitis, acne, pustuloses, hiperosteoses, osteítes) é uma condição crônica e inflamatória com manifestações cutâneas e ósteo-articulares. Seu etiologia não é clara, ainda que se considerou uma possível associação com as espondiloartritis. Diferentes tratamentos com esteroides e medicamentos antiinflamatórios são utilizados nesta patologia, com resposta variável. Os bisfosfonatos são uma das novas modalidades no tratamento da síndrome de SAPHO, graças a suas propriedades anti-osteoclásticas e anti-inflamatórias. Reporta-se o caso de um paciente com SAPHO tratado com ácido zoledrónico com sucesso
Subject(s)
Humans , Diphosphonates , Spondylarthropathies , Acquired Hyperostosis SyndromeABSTRACT
The SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) includes a group of findings characterized by bone lesions usually located on the anterior chest wall, often associated with skin lesions. We report the case of a 47 years old patient, with osteochondritis at costoesternal and manubrium-sternal joints, besides of palmar-plantar pustulosis. The diagnosis is predominantly clinical and there are several treatment options described in the literature.
A síndrome SAPHO (sinovite, acne, pustulose, hiperostose e osteíte) inclui um grupo de achados caracterizado por lesões ósseas localizadas geralmente na parede torácica anterior, frequentemente associadas a lesões cutâneas. Relata-se o caso de uma paciente de 47 anos, com quadro clínico composto por osteocondrite de articulação costoesternal e manúbrio-esternal, além de pustulose palmo-plantar. O diagnóstico é predominantemente clínico e há diversas opções de tratamento descritas na literatura.
Subject(s)
Female , Humans , Middle Aged , Acquired Hyperostosis Syndrome/diagnosis , Acquired Hyperostosis Syndrome/drug therapy , Alendronate/therapeutic use , Dermatologic Agents/therapeutic use , Methotrexate/therapeutic useABSTRACT
The clinical data of 16 patients with synovitis,acne,pustulosis,hypemstosis,osteitis (SAPHO) syndrome were retrospectively analyzed.There were 7 men and 9 women with a mean age of (38 ± 11) years at onset and middle-aged females predominated.The most common clinical manifestations were palmoplantar pustulosis and osteoarticular disease involving anterior chest wall,spondylarthrilides and shoulder joint.ECT was the sensitive imaging examination for SAPHO syndrome.Combined treatment can alleviate symptoms of the disease.
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Objective To describe the clinical and imaging manifestations of patients with synovitis acne pustulosis hyperostosis osteomyelitis (SAPHO) syndrome,and to analyze the diagnostic importance of different clinical and imaging manifestations for SAPHO syndrome. Methods Seventeen patients (7 males and 10 females) with SAPHO syndrome were recruited in this study.Age ranged from 36 to 67 years with a mean age of (48 ± 8) years. All patients fulfilled the diagnostic criteria of Benhamou. Serum HLA B27 antigen records were reviewed for all patients. Imaging data of the abnormal bone sites were collected by conventional radiograph in all patients,CT in 13 patients as well as MR in 3 patients.Average time to take for a definite diagnosis of the syndrome was 3.7 years (ranged from O.5 to 13 years).Results Serum HLA B27 antigen was positive in all patients. Both skin and bone abnornalities were found in all patients.Ten patients had skin palmoplantar pustulosis and two patients had acne. Involving sites of bone and joints include sacroiliac joints,anterior chest and limbs.Sacroiliac joints were asymmetrically involved with imaging features in all patients.Eight patients exhibited anterior chest wall involvement. Five patients had osteomyelitis at limbs. For all images of 17 patients,CT was superior to conventional radiography in detecting abnormal changes of bone erosion and soft tissue swelling.MR imaging was able to depict edema changes that was not detectable by CT and radiography.Conclusion SAPHO syndrome is a rare disease,but for patients with skin and bone-joint abnormalities,especially with skin palmoplantar pustulosis,acne as well as with imaging features at the sacroiliac joint and anterior chest wall,SAPHO syndrome should be taken into a diagnostic consideration.
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Objective To investigate the clinical features of SAPHO syndrome.Methods Clinical data of 22 cases of SAPHO syndrome were analysed.Results There were 7 males and 15 females among the 22 patients.The average age at onset of cutaneous and osteoarticular lesions was 45 years and 44 years, respectively.Of the 22 patients,21 had palmoplantar pustulosis and 1 had acne fulminans.Anterior chest wall (ACW) was involved in 19 patients,peripheral joints in 4 patients and sacroiliac joints in 2 patients.Osteoarticular manifestations occurred prior to the onset of skin lesions in 10 cases.after that in 9 cases,and simultaneously in 3 cases.The mean interval between the onset of cntaneous and osteoarticular lesions was 2.7 years and the longest interval was 20 years.Conclusions Middle-aged females predominate in patients with SAPHO syndrome seen in dermatological clinics.Palmoplantar pustulosis and ACW involvement are the most common clinical manifestations of SAPHO syndrome.