Synovitis, acne, pustulosis, hyperostosis and osteitis syndrome: a single centre study of a cohort of 164 patients

Li, Chen; Zuo, Yuzhi; Wu, Nan; Li, Li; Li, Feng; Zhang, Weihong; Xu, Wenrui; Zhao, Xue Qiao; Jing, Hongli; Pan, Qingqing; Zhou, Weixun; Shi, Xiaohua; Fan, Yu; Wang, Jianyi; Liu, Sen; Liu, Zhenlei; Zhang, Fengchun; Zeng, Xiaofeng; Chen, Hui; Zhang, Siya; Liu, Jinhe; Qiu, Guixing; Wu, Zhihong; Dong, Zhenhua; Zhang, Wen

doi:10.1093/rheumatology/kew015

Cited by 68 publications

(91 citation statements)

References 26 publications

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“…However, 1.2% of the patients were RF positive in our SAPHO cohort with 164 patients. [1] In another study, anti-CCP antibody was negative in all the 69 patients tested. [14,15] Only 1 similar case who concurrently fulfilled the diagnostic criteria for both RA and SAPHO syndrome was previously reported.…”

Section: Discussionmentioning

confidence: 99%

“…[1] The osteoarthritic disorders mainly affect the anterior chest wall (ACW, including sternoclavicular, manubriosternal, and costosternal joints), the spine and sacroiliac joint. [2] However, the appendicular skeleton, especially the small joint is a less frequently affected site in SAPHO.…”

Section: Introductionmentioning

confidence: 99%

“…[2] However, the appendicular skeleton, especially the small joint is a less frequently affected site in SAPHO. [1,2] Howver, rheumatoid arthritis (RA) is a chronic inflammatory disease mainly affecting the synovial tissue in joint, which can result in joint damage. [3] It is seropositive for rheumatoid factor (RF) and anti-cyclic citrullinated peptide (anti-CCP) antibody, with a predilection on small joints of the hand and wrist.…”

Section: Introductionmentioning

confidence: 99%

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The coexistence of SAPHO syndrome and rheumatoid arthritis

Zhang

2017

Medicine

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Rational:SAPHO (Synovitis-Acne-Pustulosis-Hyperstosis-Osteitis) syndrome is a rare disease featured by its dermatological and osteoarthritic disorders, the latter of which mainly affecting the anterior chest wall, spine, and sacroiliac joint. However, rheumatoid arthritis (RA) is a chronic autoimmune disease, mainly affecting the synovial tissue of small joints in hands and feet. Here, we present an extremely rare case diagnosed with both SAPHO syndrome and RA, with an onset interval of 10 years. So far, only 1 similar case has been reported in the English literature.Patient Concerns:In Sep 2015, a 59-year-old female patient presented to our hospital, complaining of refractory low back pain, left sternoclavicular joint pain, and palmoplanar pustulosis (PPP). In addition, RA had been diagnosed 10 years earlier in the patient, manifested as pain and swelling in bilateral hands and wrists, accompanied by morning stiffness, as well as positive serologic tests.Interventions:In our hospital, laboratory tests revealed elevated inflammatory markers, and imaging examinations of relevant sites showed specific osteoarthritic lesions for SAPHO syndrome.Diagnoses:These findings lead us to make an easy diagnosis of the coexistence of SAPHO syndrome and RA in this petient.Outcomes:Treatment with tripterygium wilfordii polyglycosidium and prednisone was introduced. Both dermatological and osteoarthritic symptoms improved during a 3-month follow-up. Symptoms of RA were successfully controlled with prednisone and leflunomide since 2005.Lessons:We present an extremely rare case diagnosed with both SAPHO syndrome and RA, with an onset interval of 10 years. With this case report, we want to draw attention to the diverse features of SAPHO syndrome.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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The coexistence of SAPHO syndrome and rheumatoid arthritis

Zhang

2017

Medicine

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“…Our research team previously reported the largest cohort study of SAPHO syndrome in the world, including one hundred and sixty-four patients [21]. In this study, psychiatric evaluation and MRI scans were performed on twenty-eight patients of these SAPHO patients, and fourteen SAPHO patients were diagnosed with depression.…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, it is important to identify depression in SAPHO patients as it may have similar effect on AS/psoriatic patients. Our research team is involved in the largest cohort study of SAPHO syndrome in the world [21]. When assessing the clinical, laboratory and radiological features of SAPHO syndrome, we also tried to explore the episode of depressive symptoms in SAPHO patients and revealed neurobiological basis of depression symptoms in these patients using rs-fMRI.…”

Section: Introductionmentioning

confidence: 99%

Depression in patients with SAPHO syndrome and its relationship with brain activity and connectivity

Duan

Zuo

et al. 2017

Orphanet J Rare Dis

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BackgroundSynovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome is a rare disease and there is no related literature concerning psychiatric symptoms in SAPHO patients. Thus, we believe that this will be the first paper to explore the episode and the neurobiological basis of depression symptoms in SAPHO patients using resting state functional magnetic resonance imaging (rs-fMRI). Twenty-eight SAPHO patients and fifteen age- and gender- matched normal controls (NC) were consecutively submitted to psychiatric evaluation and rs-fMRI scanning.Results46.2% (13/28) of SAPHO patients were diagnosed as depression. The local spontaneous activity study showed that depressed SAPHO (D-SAPHO) patients had decreased amplitude of low-frequency fluctuation (ALFF) in the bilateral ventrolateral prefrontal cortex (VLPFC, attributed to the anatomical structures of Brodmann’s area 47, 45 and 44) and right dorsolateral prefrontal cortex (DLPFC, attributed to the anatomical structures of Brodmann’s area 8, 9 and 46), increased ALFF in the bilateral middle temporal gyrus, when compared to non-depressed SAPHO (ND-SAPHO) patients. The functional connectivity (FC) study disclosed that D-SAPHO patients had an increased FC in the anterior portions of default mode network (DMN) (the bilateral inferior frontal cortex, anterior cingulate cortex and insula cortex), and a decreased FC in the posterior areas of DMN (left middle occipital cortex), when compared to ND-SAPHO patients. Furthermore, correlation analysis revealed that both ALFF and FC values were significantly correlated with depression scores of SAPHO patients.ConclusionThese results prompt us to understand the underlying pathophysiological mechanism of depression in SAPHO syndrome, and demonstrate that abnormal brain functional areas may serve as effective biological indicators to monitor depression in the future.Electronic supplementary materialThe online version of this article (doi:10.1186/s13023-017-0658-5) contains supplementary material, which is available to authorized users.

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Tofacitinib for the Treatment of Nail Lesions and Palmoplantar Pustulosis in Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis Syndrome

Cao

et al. 2021

JAMA Dermatol

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IMPORTANCENail involvement is common in synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome, which has a strong association with quality of life in patients with SAPHO. Tofacitinib is an oral Janus kinase inhibitor that has been previously shown to be effective for nail psoriasis.OBJECTIVE To assess the efficacy and safety of tofacitinib for the treatment of nail involvement in SAPHO syndrome. INTERVENTIONS Participants received tofacitinib, 5 mg, twice daily, for 12 weeks. DESIGN, SETTING, AND PARTICIPANTS This open-label, single-arm, prospective pilot study included 13 patients with SAPHO syndrome accompanied by nail lesions and active palmoplantar pustulosis who were recruited from Peking Union Medical College Hospital from September 2019 to December 2019. Follow-up was completed in March 2020. Analysis began March 2020. MAIN OUTCOMES AND MEASURESThe primary end point was the percentage of the change from baseline in Nail Psoriasis Severity Index scores at week 12. Secondary end points included the percentage of the change from baseline in Palmoplantar Psoriasis Area and Severity Index scores, change from baseline in Visual Analogue Scale scores in global osteoarticular pain, Dermatology Life Quality Index scores, and inflammatory markers. Adverse events were recorded throughout the study. RESULTSThirteen female Asian patients (means [SD] age, 39.7 [12.3] years) were included, all of whom completed the study. At week 12, significant improvements were observed in Nail Psoriasis Severity Index scores (median, −67% [interquartile range (IQR), −56% to −77%]; P < .001) and Palmoplantar Psoriasis Area and Severity Index scores (median, −71% [IQR, −58% to −78%]; P < .001). Significant improvement was also noted in Dermatology Life Quality Index scores (median, −12 [IQR, −8.5 to −15]; P < .001) at week 12. A significant decrease in Visual Analogue Scale scores in global osteoarticular pain was observed at week 8 (median, −4 [IQR, 0 to −5]; P = .02) but was not significant at week 12. Inflammatory marker levels were decreased, as indicated by erythrocyte sedimentation rate (median, −8 mm/h [IQR, −4 mm/h to −11 mm/h]; P < .001) and high-sensitivity C-reactive protein levels (median, −1.6 [IQR, −0.3 to −4.1]; P = .01). No severe adverse events were observed. CONCLUSIONS AND RELEVANCEIn this pilot study, tofacitinib yielded significant remission of nail lesions and palmoplantar psoriasis accompanied by an improvement in quality of life in patients with SAPHO syndrome. Additional follow-up studies to evaluate the long-term efficacy and safety of tofacitinib for nail involvement in SAPHO syndrome are warranted.

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Synovitis, acne, pustulosis, hyperostosis and osteitis syndrome: a single centre study of a cohort of 164 patients

Abstract: SAPHO syndrome is predominant in middle-age women, characterized by dermatological and osteoarticular manifestations with unknown aetiology. CT scan and bone scintigraphy are useful for diagnosis. There is still no standard treatment to control the disease.

Cited by 68 publications

References 26 publications

The coexistence of SAPHO syndrome and rheumatoid arthritis

The coexistence of SAPHO syndrome and rheumatoid arthritis

Depression in patients with SAPHO syndrome and its relationship with brain activity and connectivity

Tofacitinib for the Treatment of Nail Lesions and Palmoplantar Pustulosis in Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis Syndrome

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