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We aimed to convert the English version of the Evaluation of Ankylosing Spondylitis Quality of life (EASi-QoL) into a Chinese version, and to test the reliability and validity of the Chinese version of EASi-QoL.The EASi-QoL was translated into Chinese. A total of 127 patients with ankylosing spondylitis (AS) were evaluated using the Chinese version of EASi-QoL to test its validity and reliability. Internal and test-retest reliability was assessed. In addition, correlation and exploratory factor analyses evaluating the structural, convergent, and criterion-related validities of this measure were performed.The total Cronbach's α coefficient of the Chinese version of EASi-QoL was 0.911. The Kaiser-Meyer-Olkin value of the scale in this study was 0.917, and the Bartlett's test value was 2403.499. The convergent validity of the related domains was analyzed using confirmatory factor analysis; and the average variance extracted values corresponding to the four dimensions were all 0.5, and the composite reliability values were all 0.7. Discriminative validity analysis showed that the correlation between the four domains of EASi-QoL and their related domains of the depression/anxiety screening scale and the Medical Outcomes study Short-Form 36 were moderate to high.The Chinese version of EASi-QoL has high reliability and validity, and can reflect the impact of AS on the quality of life. Key Points • The Chinese version of EASi-QoL can accurately measure the impact of AS on the quality of life from the perspective of a patient. It is applicable to the evaluation of AS patients in China • The Chinese version of EASi-QoL is a scale with good reliability and validity.

BackgroundImmune-mediated necrotizing myopathy (IMNM) is characterized by markedly elevated creatinine kinase and histologically scattered necrotic muscle fibers and generally associated with autoantibodies against signal recognition particle (SRP) or 3-hydroxy-3-methylglutaryl-coA-reductase (HMGCR). Poor clinical response to conventional therapies and relapses commonly occur in severe cases. Anti-B-cell therapies have been used in refractory/relapsing cases.MethodsThe characteristics of a patient with IMNM associated with anti-SRP antibodies including physical examination, laboratory tests, and disease activity assessment were evaluated. Conventional therapy, belimumab treatment schedule, and follow-up data were recorded. Medical records of IMNM patients treated in our department from September 2014 to June 2021 were reviewed to evaluate the efficacy and safety of anti-B-cell therapy for anti-SRP IMNM. A literature review of patients with anti-SRP IMNM treated with anti-B-cell therapies was performed.ResultsWe describe a case of a 47-year-old woman with IMNM associated with anti-SRP antibodies who relapsed twice after conventional therapy but showed good response and tolerance to belimumab at 28 weeks follow-up. In this review, three patients from our department were treated with rituximab. Two of the three patients rapidly improved after treatment. Twenty patients and five retrospective studies were included in the literature review. All patients were administered rituximab as an anti-B-cell drug.ConclusionDespite a lack of rigorous clinical trials, considerable experience demonstrated that anti-B-cell therapy might be effective for patients with IMNM associated with anti-SRP antibodies. Belimumab in association with steroids might be an encouraging option for refractory/relapsing cases.

Objective: Inflammatory bowel disease is an immune-mediated chronic inflammatory disease of the gastrointestinal tract for which curative drugs are currently not available. This study was performed to assess the therapeutic effects of cinacalcet on dextran sulfate sodium (DSS)-induced colitis.Methods: Primary macrophages obtained from bone marrow and the macrophage cell line RAW264.7 were used to examine the inhibitory effect of cinacalcet on cytokine production, the PKCδ/ERK/P65 signaling pathway, and NF-κB P65 translocation. Colitis was induced using DSS to assess the treatment effect of cinacalcet. Bioinformatics approaches were adopted to predict potential targets of cinacalcet, and a drug affinity responsive target stability (DARTs) assay was performed to confirm binding between cinacalcet and potential target.Results:In vivo analysis showed that cinacalcet reduced the disease activity score, prevented shortening of the colon, diminished inflammatory cell infiltration, and protected the structural integrity of the intestinal wall. Cinacalcet also reduced production of the inflammatory cytokines TNFα, IL-1β, and IL-6 in the colon and sera of mice with DSS-induced colitis. In vitro studies revealed that cinacalcet suppressed the translocation of P65 and inhibited production of the inflammatory cytokines IL-1β and IL-6. Mechanistic studies revealed that the target of cinacalcet was neurokinin-1 receptor (NK1R) and their binding was confirmed by a DARTs assay. Furthermore, the inhibition of NK-κB P65 activation was found to occur via the suppression of PKCδ/ERK/P65 signaling mediated by cinacalcet.Conclusion: Cinacalcet inhibits the activation of NF-κB and reduces the production of inflammatory cytokines by suppressing the PKCδ/ERK/P65 signaling pathway via targeting NK1R, suggesting that it can be used to treat inflammatory diseases, particularly colitis.

To summarize the clinicopathological characteristics, diagnosis and treatment of IgG4-related disease (IgG4-RD).The clinical data of 43 cases with IgG4-RD diagnosed from January 2013 to December 2017 were retrospectively analyzed. The clinical data of the patients including clinical characteristics, accessory examinations, diagnosis, and treatment were collected.Among the 43 patients with IgG4-RD, the ratio of male to female was 3∶1, the mean age was (51.3±15.9) years. Eleven patients had gastrointestinal symptoms, including 5 cases of IgG4-related cholangitis with the feature of dilation of the biliary system and narrowing of the lumen in the abdominal enhanced CT scans, and 6 cases of IgG4-related autoimmune pancreatitis with the feature of pancreatic enlargement or soft tissue density shadow in the abdominal enhanced CT scans. There were 10 cases (23.3%) with periorbital involvement, with the feature of intraorbital soft tissue nodule in the CT scan. Besides, 9 cases (20.9%) had lymphadenopathy, 6 cases (14.0%) had fever. The results of autoimmune antibody tests showed that 14 of 42 patients had increased antinuclear antibody (ANA). Among 40 patients who underwent immunoglobulin tests, 25 cases had elevated IgG, 12 cases had increased IgA, and 29 cases had increased IgE. Coombs test were performed for 6 cases and 4 patients were positive. Serum immunoglobulin G4 subtypes showed that the IgG4 levels of 35 patients were higher than 1 350 mg/L. The immunohistochemistry showed that IgG4 (+) cells (3-500/HPF) were infiltrated, with the CD20 (+), CD3ε (+) or CD138 (+). Among the 43 patients, 5 patients underwent operations due to misdiagnosis. All patients were treated with steroid and immunosuppressive agents after diagnosis, and their clinical symptoms were improved.The clinical symptoms of IgG4-RD are various, involving multiple organs. Therefore, the standardized diagnosis and treatment of IgG4-RD should be strengthened.

Rheumatoid arthritis (RA) is a chronic autoimmune disease that affects the joints and other organs for which there is currently no effective treatment. Mesenchymal stem cells (MSCs) have therapeutic potential due to their immunomodulatory and differentiation effects. While extensive experimental studies and clinical trials have demonstrated the effects of MSCs in various diseases, MSCs have been found to cause abnormal differentiation and tumor formation. Therefore, extracellular vesicles derived from MSCs (MSC-EVs) are more effective, less toxic, and more stable than the parental cells. MSC-EVs transfer various nucleic acids, proteins, and lipids from parent cells to recipient cells, and thus participate in chronic inflammatory and immune processes. In this review, we summarize the properties and biological functions of MSCs and MSC-EVs in RA. Improvement in our understanding of the mechanisms underlying MSC and MSC-EVs in RA provides an insight into potential biomarkers and therapeutic strategies for RA.

Cardiac dysfunction is commonly noted in patients with idiopathic inflammatory myopathies (IIMs). This study aimed to investigate the characteristics of cardiac dysfunction using cardiac magnetic resonance (CMR) in polymyositis (PM), dermatomyositis (DM) and necrotising myositis (NM). Fifty-one patients with IIMs and 20 matched healthy controls (HCs) were assessed using CMR examination. The clinical data, cardiac serum markers and autoimmune antibodies were determined for all patients. Cardiac involvement was identified by myocardial native T1, extracellular volume (ECV), late gadolinium enhancement (LGE) and left ventricular ejection fraction (LVEF). Different subtypes of IIMs showed different patterns of LGE and varying degrees of myocardial damage. The PM subgroup showed higher native T1 (p = 0.010) and ECV (p = 0.000) than the HCs. The prevalence of LGE was comparable between the PM and DM subgroups (40.0% vs. 31.6%, p = 0.741); however, it was higher in the PM subgroup than in the NM subgroup (40% vs. 0.0%, p = 0.014). Patients with positive LGE in the PM subgroup showed a higher proportion of positive LGE (p = 0.018) and lower LVEF (p = 0.024) than those with positive LGE in the DM subgroup. In multivariate analysis, the presence of LGE could be predicted by increased NT-proBNP (p = 0.036, OR = 1.001) and anti-MDA-5 antibody positivity (p = 0.011, OR = 12.4). The risk factors associated with native T1 were NT-proBNP (p = 0.016, β = 0.353) and body mass index (BMI) (p = 0.024, β = − 0.331). Distinct cardiac involvements in different subtypes of IIMs were identified using CMR. Elevated NT-proBNP and a low BMI were the risk factors associated with LGE and elevated native T1. • The characteristics of cardiac involvement in different subtypes of IIMs could be identified with cardiac magnetic resonance. • The NT-proBNP levels could reflect focal and diffuse myocardial damage in patients with IIMs.

Sjögren’s syndrome (SS) is a chronic autoimmune disorder of the exocrine glands mediated by lymphocytic infiltrates damaging the body tissues and affecting the life quality of patients. Although traditional methods of diagnosis and treatment for SS are effective, in the time of personalized medicine, new biomarkers, and novel approaches are required for the detection and treatment of SS. Exosomes represent an emerging field in the discovery of biomarkers and the management of SS. Exosomes, a subtype of extracellular vesicles, are secreted by various cell types and can be found in most bodily fluids. Exosomes are packed with cytokines and other proteins, bioactive lipids, and nucleic acids (mRNA, circular RNA, non-coding RNA, tRNA, microRNA, genomic DNA, and ssDNA), and transport such cargo between cells. Evidence has indicated that exosomes may play roles in processes such as the modulation of the immune response and activation of inflammation. Moreover, due to features such as stability, low immunogenicity and toxicity, long half-life, and the capacity to penetrate the blood-brain barrier, exosomes have also emerged as therapeutic tools for SS. In this review, we summarize existing literature regarding the biogenesis, isolation, and function of exosomes, specifically focusing on exosomes as novel biomarkers and their potential therapeutic uses in SS.

Spondyloarthritis Research Consortium Canada (SPARCC) score is an effective magnetic resonance imaging (MRI) evaluation method for inflammation in axial spondyloarthritis. Previously published meta-analyses have shown tumor necrosis factor α inhibitors (TNFi) had great effectiveness on improving disease activity and function in axial spondyloarthritis. However, there still has no one that concentrates on the impact of TNFi on MRI inflammation. We conduct a meta-analysis to summarize the impact of TNFi on MRI inflammation in axial spondyloarthritis using SPARCC score. Comprehensive search was conducted in the databases of OVID Medline, OVID EMBASE, and Cochrane library on November 14, 2020. We investigated the differences in SPARCC score of sacroiliac joint and spine, before and after TNFi treatment in patients with axial spondyloarthritis. SPARCC score was further compared in the subgroup by diagnostic category and TNFi types. In addition, clinical assessment indicators including ankylosing spondylitis disease activity score, bath ankylosing spondylitis disease activity index, bath ankylosing spondylitis functional index, c-reactive protein were also analyzed. Data were pooled by mean differences (MD) with 95% confidence intervals (CI) and publication bias was assessed by Egger’s test. Jadad scale was applied to assess the quality of included trials. Compared with control group, TNFi significantly improved SPARCC score of sacroiliac joints (n = 11, MD = 2.86, 95% CI 2.50, 3.23) and spine (n = 5, MD = 1.87,95%CI 1.27, 2.46). This effect was consistent among subgroups by different diagnostic category (ankylosing spondylitis, non-radiographic axial spondyloarthritis) and TNFi types (adalimumab, certolizumab pegol). Analysis of clinical assessment indicators also confirmed the therapeutic effect on axial spondyloarthritis. Egger’s test suggested no possibility of publication bias. This meta-analysis shows that TNFi are effective to improve MRI inflammation in patients with axial spondyloarthritis and the treatment effectiveness is not affected by diagnostic category and TNFi types.

Background Anti-melanoma differentiation-associated gene 5 (MDA5) dermatomyositis (DM) is a distinctive subtype of DM that carries a significant risk of interstitial lung disease (ILD). The mechanisms remain elusive. Objectives To explore the role of the type I interferon (IFN) system in the pathogenesis of anti-MDA5 DM. Methods Twenty patients with anti-MDA5 DM were studied and compared with patients with anti-aminoacyl-tRNA synthetase (ARS) DM (n = 10) and autoantibody-negative patients with DM (n = 20). The levels of inflammatory cytokines, B-cell-activating factor (BAFF) and Krebs von den Lungen (KL)-6 in blood were tested by enzyme-linked immunosorbent assay and multiplex assays. Expressions of transcripts for IFN-associated sensors and type I IFN-inducible genes in peripheral blood mononuclear cells (PBMCs) were detected by real-time polymerase chain reaction. Expressions of the signal transducer and activator of transcription (STAT)1, interferon-stimulated gene (ISG)15 and MxA proteins in skin lesions were analysed by immunohistochemistry. Results Plasma IFN-α levels were significantly increased in patients with anti-MDA5 DM. PBMCs from patients with anti-MDA5 DM showed significant upregulation of the TLR3, TLR7, IFIH1 and DDX58 genes, as well as serial IFN-inducible genes. Skin biopsies from patients with anti-MDA5 DM were characterized by strong expression of the STAT1, ISG15 and MxA proteins. In the patients with anti-MDA5 DM and ILD with high IFN-α production, there was a positive quantitative correlation between IFN-α and BAFF (rs = 0·63, P = 0·044). In addition, the higher levels of BAFF paralleled the higher concentrations of KL-6 (rs = 0·86, P = 0·0012). Conclusions Our data confirm the aberrant activation of the type I IFN system in anti-MDA5 DM. Overproduction of IFN-α linked with BAFF may be implicated in the development of ILD.

Autoimmune diseases (ADs) are a class of chronic disease conditions with impaired tolerance to autoantigens. Currently, there is no effective treatment for ADs, and the existing medications have limitations due to non-specific targets and side effects. Accumulating evidence has shown that mesenchymal stem cells (MSCs) play a role in ADs treatment. These beneficial effects mainly rely on cell-to-cell communication through the secretion of extracellular vesicles (EVs) and soluble factors. MSC-derived EVs (MSC-EVs) could modulate adjacent and distinct cells by transferring various DNA, mRNA, non-coding RNAs, proteins, and lipids from parent cells to recipient cells. MicroRNAs (miRNAs) are small non-coding RNAs that negatively regulate multiple target genes at the post-transcriptional level and are involved in chronic inflammatory and immune processes. Compared to fluid, MSC-EVs delivery can protect miRNAs from the degradation of ribonucleases, ensuring that miRNAs are able to perform their respective crucial roles in AD recipient cells. In this review, we discussed the therapeutic prospects and challenges of miRNAs secreted by MSC-EVs (MSC-EV-miRNAs) by reviewing the experimentally verified therapeutic outcomes of MSC-EV-miRNAs for several ADs, including rheumatoid arthritis (RA), autoimmune hepatitis (AIH), asthma, colitis, systemic sclerosis (SSc) and graft-versus-host disease (GVHD).

Aseptic meningitis is an extremely rare neurologic complication of relapsing polychondritis (RP). We reported a case of a 58-year-old Chinese female with intractable headache, puffy ears, pleocytosis, and cranial magnetic resonance imaging (MRI) showing thickened and enhanced meninges. She was finally diagnosed of aseptic meningitis due to RP after full exclusion of infectious causes. She gradually developed neurosensory hearing loss, vertigo, and saddle nose while glucocorticosteroid therapy and combined cyclophosphamide could not control her headache. Ultimately, cyclosporin A was tried showing a good response. Only 18 previous cases were found in the literature and the clinical manifestation, cerebrospinal fluid (CSF) characteristics, imaging features, and therapy considerations of RP-related aseptic meningitis were summarized by reviewing the literature. Aseptic meningitis due to RP is a rare condition of undetermined pathoetiology. Its diagnosis is primarily based on clinical manifestations combined with CSF and MRI examinations plus adequate exclusion of possible infections. Corticosteroid is the basic therapy but choice of protocol should be individualized.

Polymyositis (PM) is a chronic disease characterized by muscle pain, weakness, and increase in muscle-related enzymes, accompanied with inflammations in lymphocytes. However, it is not well understood how the molecular alternations in lymphocytes contribute to the development of polymyositis. The mechanistic target of rapamycin (mTOR) signaling is the central regulator of metabolism and inflammation in mammalian cells. Based on previous studies, we proposed that mTOR signaling may control inflammatory reactionsvialipid metabolism. In this study, we aim to figure out the role of mTOR signaling in the development of polymyositis and identify novel biomarkers for the detection and therapy of polymyositis. After screening and validation, we found that palmitoleic acid, a monounsaturated fatty acid, is highly regulated by mTOR signaling. Inhibition of mTORC1 activity decreases palmitoleic acid level. Moreover, mTORC1 regulates the level of palmitoleic acid by controlling its de novo synthesis. Importantly, increased palmitoleic acid has been proven to be a marker of polymyositis. Our work identifies palmitoleic acid in peripheral blood mononuclear cells (PBMC) as a biomarker of polymyositis and offers new targets to the clinical therapy.

Chimeric antigen receptor T (CAR-T) cells are T cells engineered to express specific synthetic antigen receptors that can recognize antigens expressed by tumor cells, which after the binding of these antigens to the receptors are eliminated, and have been adopted to treat several kinds of malignancies. Autoimmune diseases (AIDs), a class of chronic disease conditions, can be broadly separated into autoantibody-mediated and T cell-mediated diseases. Treatments for AIDs are focused on restoring immune tolerance. However, current treatments have little effect on immune tolerance inverse; even the molecular target biologics like anti-TNFα inhibitors can only mildly restore immune balance. By using the idea of CAR-T cell treatment in tumors, CAR-T cell-derived immunotherapies, chimeric autoantibody receptor T (CAAR-T) cells, and CAR regulatory T (CAR-T) cells bring new hope of treatment choice for AIDs.

Background Treatment effect of tumor necrosis factor α inhibitors (TNFi) in patients with spondyloarthritis (SpA) had been proved by plenty of studies that could remarkably eliminate symptoms, signs, and laboratory inflammatory manifestations [1,2]. Simultaneously, the treatment effect of TNFi on delaying radiographic progression in SpA was often reported [3,4], including magnetic resonance imaging (MRI) which has a high sensitivity on detecting the earliest inflammation [5]. Objectives A meta-analysis was performed to summarize the treatment effect of TNFi on MRI progression reflected by SPARCC score in SpA patients Methods Comprehensive search was conducted in the electronic databases of OVID Medline, OVID EMBASE and Cochrane library on Nov.27, 2018. All randomized controlled trials (RCTs) focused on MRI progression and disease activity in SpA patients treated with TNFi were included. Primary outcome was the Spondyloarthritis Research Consortium Canad (SPARCC) MRI scoring system, accompanied with or without other outcomes, including ankylosing Spondylitis Disease activity Score (ASDAS), Bath ankylosing Spondylitis disease activity index (BASDAI), Bath ankylosing Spondylitis functional index (BASFI), C-reactive protein (CRP). Data were pooled by mean differences (MD) with 95% confidence intervals (CI) and publication bias was assessed by funnel plot. Sensitivity analysis was performed to test the result robustness. Jadad scale was applied to assess the methodology quality of included trials. Two reviewers independently selected studies, extracted data, and assessed study quality. Results Totally 10 RCTs enrolled 1006 patients with high methodology were included. Compared with control group, TNFi significantly improved SPARCC score at sacroiliac joint (SIJ) (MD=2.84, 95% CI 2.43-3.25), SPARCC score of spine (MD=1.87, 95% CI 1.27-2.46), aSDAS (MD=0.96, 95% CI 0.71-1.20), BASDAI (MD=1.15, 95% CI 0.51-1.78), BASFI (MD=0.95, 95% CI 0.51-1.40), and CRP (MD=4.64, 95% CI 1.06- 8.23) in double-blind phase. Subgroup analyses by disease subgroup and individual TNFi showed treatment effect of TNFi on delaying MRI progression was not affected by disease subgroup and individual TNFi. Sensitivity analysis showed the treatment effects of TNFi versus placebo were consistent with TNFi versus controls, suggesting the results of the study were robust. The funnel plot of SPARCC score at SIJ based on TNFi versus control in double-blind phase was asymmetric, suggesting there might have potential publication bias. Conclusion TNFi are effective to treat SpA patients and delay MRI progression which are assessed by SPARCC score and treatment effect is consistent among disease subgroups and individual TNFi. References [1] Baraliakos X, Listing J, Fritz C, et al. Persistent clinical efficacy and safety of infliximab in ankylosing spondylitis after 8 years–early clinical response predicts long-term outcome. Rheumatology (Oxford). 2011; 50(9):1690–9. [2] Sfikakis PP. The first decade of biologic TNF antagonists in clinical practice: lessons learned, unresolved issues and future directions. Curr Dir autoimmun. 2010;11:180–210. [3] Wanders a, van der Heijde D, Landewe R, et al. Nonsteroidal antiinflammatory drugs reduce radiographic progression in patients with ankylosing spondylitis: a randomized clinical trial. Arthritis Rheum. 2005;52(6):1756-65. [4] Poddubnyy D, Rudwaleit M, Haibel H, et al.Effect of nonsteroidal antiinflammatory drugs on radiographic spinal progression in patients with axial spondyloarthritis: results from the German Spondyloarthritis inception Cohort. Ann Rheum Dis. 2012;71:1616-22. [5] Puhakka KB, Jurik aG, Schiottz-Christensen B, et al. Magnetic resonance imaging of sacroiliitis in early seronegative spondylarthropathy. Abnormalities correlated to clinical and laboratory findings. Rheumatology (Oxford). 2004;43(2):234–7. Disclosure of interests None declared

Background Interleukin-2 inducible T-cell kinase (ITK) is expressed in T cells, and plays an important role in autoimmune inflammatory diseases through regulating the balance of Th17/Treg. However, its role in human systemic lupus erythematosus (SLE) remains unclear. The present study aims to measure the activation status of ITK in T cells from SLE patients and healthy controls, and identify its possible correlation to disease severity. We also discuss the serum levels of Th17, Treg related cytokines including IL-17, IL-21, IL-22, IL-10, analyzing correlation between ITK and Th17/Treg related cytokines. Methods Peripheral blood samples were drawn from 42 patients with SLE and 43 healthy blood donors, and the phosphorylation of ITK protein was studied in T cells using flow cytometry. In addition, serum levels of Th17/Treg related cytokines were studied with enzyme-linked immunosorbent assay (ELISA). Results Percentages of CD4 + pITK + T cells, CD8 + pITK + T cells were higher in SLE patients compared with controls, and were positively related to disease activity, some clinical and laboratory parameters. Percentages of CD4 + pITK + T cells, CD8 + pITK + T cells were more prominent in active SLE patients compared with less active patients. Serum levels of Th17 and Treg related cytokines were higher in patients compared with controls. CD4 + pITK + T cells were related to levels of IL-17, IL-21. Conclusion These data indicate that increased ITK expression could act as a disease activity marker and as a risk factor for involvement in SLE, but it still needs further study to confirm.

Rationale Some diseases contribute to hypopituitarism without clinical manifestations and the glucocorticoid therapy may unveil central diabetes insipidus. The condition is rare and usually causes problems for clinical physicians. Patient concerns A 59-year-old woman presented to our hospital due to facial numbness and persistent eyelid heaviness. Diagnosis Physical examination and cerebrospinal fluid examination supported a diagnosis of Guillain-Barre[Combining Acute Accent] syndrome. Magnetic resonance imaging showed an empty sella. Hormone test indicated hypopituitarism. Interventions The patient received intravenous immunoglobulin and glucocorticoid. Central diabetes insipidus appeared after 20 days. Subsequently, the patient was prescribed 1-desamino-8-D-arginine vasopressin and prednisone. Outcomes During 6 months' follow-up, the patient's urine output was gradually reduced to normal level. Lessons This case indicated that hypopituitarism may be caused by an empty sella and be masked by adrenal insufficiency. Central diabetes insipidus may present after glucocorticoid therapy.

Background Because of the lack of effective treatment of systemic sclerosis (SSc), cellular therapy is considered as a salvage option. 1–4 Mesenchymal stem cells (MSCs) are gaining attentions in the field of cell therapy of SSc. However, current isolation methods of MSCs are all invasive and challenging. 5 Some studies have reported the isolation from urine recently, and definition of urine-derived stem cells (USCs). 6 The collection of USCs is noninvasive that could be a desired resource of MSC. So far, the information of therapeutic effect on SSc is limited. Objectives We aimed to explore the biological characterizations of USCs and investigate the therapeutic effect on murine SSc model. Methods USCs were isolated and cultivated from sterile urine samples of healthy adult individuals. The related cell markers were examined by flow cytometry. The differentiation potentials were observed in adipogenic, osteogenic and chondrogenic medium, respectively. SSc murine models were conducted by daily intradermal injections of bleomycin and were further divided as treated and untreated group. Also, the healthy control group was conducted by daily injection of phosphate buffered saline (PBS). Treated group received an infusion of 2.5 × 10 5 USCs in the tail vein twice in one week after the 3 week modelling. Skin samples were obtained one week after the treatment. Hematoxylin-eosin and Masson staining were accomplished to observe the skin thickness and the hydroxyproline content was detected by hydroxyproline kit. The relative expression of collagen type I alpha 1 chain (Col1-a1), alpha-smooth muscle actin (α-SMA), and fibronectin 1(Fn-1) were detected by real-time quantitative PCR. Results The morphology of USCs was spindle-shaped. They express CD73, CD90, and CD105 but CD34, CD45, CD19, CD11b, or HLA-DR. USCs possessed the abilities to differentiate into adipocytes, osteoblasts, and chondrocytes. In USCs treated group, the skin thickness(p=0.031) (Figure1A), the deposition of collagen in HE and Masson trichrome-staining (p=0.007) (Figure1B), and Col1-a1 gene expression(p=0.010) (Figure1C) were significantly reduced in comparison with untreated group and were close to healthy controls. Conclusions USCs can be isolated from adult sterile urine. They possess the multipotent differentiation abilities and displays a therapeutic effect on bleomycin-induced skin fibrosis and collagen synthesis. References [1] Gabrielli A, Avvedimento EV, Krieg T. Scleroderma. N Engl J Med2009;360:1989–2003. [2] Verrecchia F, Laboureau J, Verola O, et al. Skin involvement in scleroderma-where histological and clinical scores meet. Rheumatology2007;46:833–841. [3] Fleming JN, Nash RA, McLeod DO, et al. Capillary regeneration in scleroderma: Stem cell therapy reverses phenotype?PLoS One2008;3:e1452. [4] Aschwanden M, Daikeler T, Jaeger KA, et al. Rapid improvement of nailfold capillaroscopy after intense immunosuppression for systemic sclerosis and mixed connective tissue disease. Ann Rheum Dis2008;67:1057–1059. [5] Le Blanc K, Pittenger M. Mesenchymal stem cells: progress toward promise. Cytotherapy2005;7:36–45. [6] Zhang Y, McNeill E, Tian H, et al. Urine derived cells are a potential source for urological tissue reconstruction [J]. J Urol2008;180:2226–2233. Disclosure of Interest None declared

To analyze the clinical features of interstitial pneumonia with autoimmune features (IPAF) and the correlation between them.We respectively analyzed the patients with interstitial lung disease (ILD) admitted in our hospital from January 2014 to January 2017. The patients who met all priori requirements and at least one feature of one domain were recuited, and the clinical features and autoimmune diseases related prognosis were analyzed.There were 90 patients recruited,including 38 patients completely met IPAF classification criteria and 52 patients who incompletely met. The average age was (62.34±14.98) yr.. The pneumonia pattern of complete IPAF patients was non-specific interstitial pneumonia (NSIP), while it was usually interstitial pneumonia (UIP) in the incomplete IPAF patients. During follow-up,11 patients were diagnosed with autoimmune diseases (4 with complete IPAF,and 7 with incomplete IPAF) . According to Cox regression analysis,completely meet the criteria of IPAF was related to the relapse of disease.There exist relation between IPAF and autoimmune diseases. The patients with IPAFmay finally develop into autoimmune diseases. The IPAF classification criteria provide basic structure for this disease,but the limitation of the criteria call for revising by more clinical trials.

Comorbidity of autoimmune diseases is a very important issue but easily ignored in the clinical practice. The treatment of comorbidity of autoimmune diseases needs cooperation of multiple disciplines,which is totally different from traditional clinical disciplines division and treatment mode. Based on the clinical features of the disease,we will comprehensively look through genetic,environmental,and immune factors involving in molecular and immunological compatibility pathogenesis,and also generalize common pathological features,such as immune complex deposition and accumulation of lymphocytes. We will also investigate the association and differences between the diseases with comorbidity,and explore the outcome and prognosis of comorbidity of autoimmune diseases. With clarify of the concept of autoimmune comorbidities,we hope bring more and more attention on this aspect,so as to improve the diagnosis,treatments as well as the prognosis of these diseases.

To investigate the correlation of disease activity and thyroid indicators ,immunologic markers of system lupus erythematosus (SLE) in SLE with Hashimoto's thyroiditis (HT).The clinical data of 63 cases of SLE with HT were collected. According to Systemic lupus erythematosus disease activity index 2000 (SLEDAI-2000),we classified the patients into four groups,which were remission group (5 cases),low (19 cases),moderate (12 cases) and high (27 cases) disease activity group. Each patient received the measurement of thyroid function indicators and autoantibodies,SLE immunologic indicators,serum complement (C3,C4),C-reactive protein (CRP),erythrocyte sedimentation rate (ESR), and routine blood test. The correlation of thyroid indicators,immunologic markers and disease activity were analyzed.The difference of free triiodothyronine (FT3) level in the four groups was statistically significant (The pathogenesis of HT is associated with the disease activity in the patients of SLE with HT.

To investigate the relationship between tumor related markers and the presence of interstitial lung disease (ILD) in dermatomyositis (DM) patients as well as potential serum markers for accompanied ILD.Fifty-nine DM patients were included,including 30 patents with ILD. Serum level of anti-transcription intermediary factor1-γ (TIF1-γ) and tumor-associated antigens (TAAs) were detected to analyze the correlation of these markers with ILD. Meanwhile,the diagnostic value of these markers was evaluated by receive operating characteristic (ROC) curve analysis.We found that there were 5 patients bearing malignancies,independent from the presence of ILD (Presence of malignance and positive anti-TIF1γ could be the protective factors for ILD in DM. Elevated serum levels of CA125,CA153,CYFRA21-1 could indicate the accompanying ILD.

Objectives: This study aims to demonstrate the utilization of serum interleukin-18 (IL-18) level as a biomarker for disease activity and organ damage in patients with dermatomyositis (DM) or polymyositis (PM) using the Myositis Disease Activity Assessment Tool. Patients and methods: Forty-nine patients (13 males, 36 females) with idiopathic inflammatory myopathies were enrolled in the study. Patients were diagnosed with DM (n=33) or PM (n=16). Twenty age-and sex-matched healthy volunteers (9 males, 11 females) who were determined to be free of autoimmune disease were included as a control group. Disease activity and organ damage were assessed with Myositis Disease Activity Assessment Tool. Serum IL-18 level was measured with enzyme-linked immunosorbent assay and the differences between patient groups were determined. Results: The mean ages of DM or PM patients were 44 and 45, respectively. Interstitial lung disease (ILD) was found in 24 patients (48.98%), including 18 patients with DM (54.55%) and six patients with PM (37.5%). There was no significant difference of disease duration between DM patients (17.5 +/- 22.3 months) and PM patients (14.9 +/- 28.7 months). Serum IL-18 level in DM (669.2 +/- 528.1 pg/mL) was significantly higher than that in the control group (388.3 +/- 139.4 pg/mL, p=0.017), but no significant difference was observed between the PM and control group. IL-18 was significantly higher in DM patients with ILD (890.5 +/- 618.8 pg/mL) than in patients without ILD (403.8 +/- 84.8 pg/mL, p=0.003). It was significantly elevated in patients with severe disease activity (1001.7 +/- 694.2 pg/mL) than in those with moderate (485.3 +/- 102.2 pg/mL) or mild disease activity (421.0 +/- 270.2 pg/mL) (p=0.039). In DM patients, IL-18 was significantly correlated with global disease activity and disease activity in the skin, lung, and heart. Conclusion: Serum IL-18 level was higher in patients with DM and this was associated with disease activity and ILD complication. Thus, it can be used as a valuable serological marker for DM disease activity and ILD complication.

Mammalian cells adapt to different environmental conditions and alter cellular metabolic pathways to meet the energy demand for survival. Thus, the metabolic regulation of cells under special conditions, such as hypoxia, should be precisely regulated. During the metabolic regulation, mammalian target of rapamycin (mTOR) plays a vital role in the sensing of extracellular stimulations and regulating intracellular adaptations. Here, we report that mTOR complex 1 (mTORC1) signalling is a central regulator of lipid homoeostasis in lymphocytes. In hypoxia, mTORC1 activity is reduced and shifts lipid synthesis to lipid oxidation. Moreover, knockdown tuberous sclerosis complex 1 (TSC1) constitutively activates mTORC1 activity and impairs the hypoxia-induced metabolic shift. Therefore, TSC1 knockdown enhances hypoxia-induced cell death. Re-inactivation of mTORC1 activity via rapamycin may resist hypoxia-induced cell death in TSC1 knockdown lymphocytes. Our findings provide a deep insight into mTORC1 in the metabolic balance of lipid synthesis and oxidation, and imply that mTORC1 activity should be precisely regulated for the lipid homoeostasis in lymphocytes.

Rationale: Behçet's disease (BD) is a chronic immune-mediated inflammatory disorder involving multiple organ systems. In BD, intestinal ulcers can present as a refractory lesion capable of perforation, which makes the choice of treatment difficult. Patient Concerns: A 34-year-old male who was diagnosed with intestinal BD and suffered with an ileocecal perforation. He underwent surgery for an ileostomy and was given corticosteroids as treatment. However, the ulcerative lesion remained resistant to the therapy that was provided which delayed the closure operation. Diagnosis: Intestinal BD with severe post-operative complication. Interventions: A course of adalimumab (ADa) therapy was started. Subsequently surgery was performed. And ADa and thalidomide were used as a maintenance therapy. Outcomes: In this case, a course of ADa therapy was given which healed the intestinal ulcers and allowed us to successfully perform the closure operation. Lessons: This case indicates that ADa may be an effective treatment option in future cases, minimizing complications and allowing the closure operation to be performed successfully.

Gαq, the alpha subunit of Gq, a member of the Gq/11 sub-family, was reported to inhibit phosphatidylinositol-3-Kinase (PI3K) activation and prevent the activation of Akt. Previous studies demonstrated that mice losing Gαq in their immune system could spontaneously develop inflammatory arthritis. In this study, we showed that the Gαq expressions at mRNA and protein levels in the peripheral blood lymphocytes (PBLs) from patients with rheumatoid arthritis (RA) were significantly decreased in comparison of which in healthy individuals. The expression levels of Gαq mRNA in PBLs from patients with RA were correlated with RA disease activity (DAS28), anti-cyclic citrullinated protein antibodies, C-reactive protein and rheumatoid factor. We also demonstrated that Gαq controlled the apoptosis of RA PBLs through regulating the activity of Mcl-1 and caspase-3. These data suggested that Gαq might be involved in the pathogenesis of RA by regulating PBLs apoptosis.

Studies investigating the association between Interleukin-23 receptor (IL-23R) gene polymorphisms and Crohn’s disease (CD) report conflicting results. Thus, a meta-analysis was carried out to assess the association between the IL-23R polymorphisms and CD. A systematic literature search was conducted to identify all relevant studies. Pooled odds ratio (ORs) with 95% confidence interval (CIs) was used to estimate the strength of association. Finally, a total of 60 case-control studies in 56 articles, involving 22,820 CD patients and 27,401 healthy controls, were included in the meta-analysis. Overall, a significant association was found between all CD and the rs7517847 polymorphism (OR = 0.699, 95% CI = 0.659 ~ 0.741, P P

To evaluate the efficacy and safety of compound Decumbent Corydalis Rhizome (DCR) in treating patients with knee osteoarthritis (OA). Totally 79 patients with knee osteoarthritis were selected from out-patient and inpatient departments of West China Hospital and randomly divided into the test group and the control group. The test group (n = 41) was given Compound DCR with the dosage of 1.8 g · d(-1), while the control group (n = 38) was administered with diclofenac sodium with the dosage of 75 mg · d(-1). After 12 weeks of treatment, the total efficacy rates based on patients/physicians evaluation for experimental and control groups were 68.29%, 63.41% and 71.05%, 63.16%, respectively, without significant difference between the two groups. Both of the two groups showed significant improvements in the main efficacy indexes (pain on walking 20 m) and minor indexes (tenderness on palpation, Western Ontario and McMaster Universities OA index (WOMAC) and Short-Form Health Survey (SF-36 ), but without significant difference in efficacy between them. The incidence of related adverse events was 24.39% in the test group and 47.37% in the control group, respectively, with significant differences between the two groups (P0.05). In the controlled study, compound DCR is as efficient as diclofenac sodium but more tolerable, with a good clinical application prospect.

To evaluate the clinical value of the mutidimensional health assessment questionnaire (MDHAQ) for disease activity assessment in patients with rheumatoid arthritis (RA).A total of 100 RA patients were recruited in this study and completed the MDHAQ. Clinical data and involved joint function assessment were also collected. Routine assessment of patient index data 3 (RAPID3) was calculated using the MDHAQ. The correlations between RAPID3 and disease activity score 28 (DAS28), simplified disease activity index (CDAI), simplified disease activity index (SDAI) and clinical characteristics were analyzed.RAPID3 was correlated with erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), swollen joints count, tender joints count and overall physician rating (P0.05). The MDHAQ had a Cronbach's alpha coefficient of 0.821 and a KMO of 0.576. Bartlett's test of sphericity showed P0.001. RAPID3 was also correlated with DAS28, CDAI and SDAI, with a coefficient of 0.743, 0.697 and 0.640 (P0.001), respectively.MDHAQ is a reliable instrument for disease activity assessment in patients with RA. It is easy to administer in busy clinical settings.

To compare the disease activity and therapeutic strategy of late-onset rheumatoid arthritis (LORA) with young-onset rheumatoid arthritis (YORA).Medical records of 259 patients with rheumatoid arthritis (RA) were reviewed retrospectively. The cut-off between LORA and YORA was operationally set at 60 years of age at disease onset. Gender ratio, disease duration, feature of involved joint, extra-articular features, laboratory features, disease activity score and treatment strategy were compared between LORA and YORA.The LORA patients had a gender ratio (male/female) of 1/1.88, which was approaching 1/1 in the older age group. It took longer for LORA to be diagnosed than YORA (P0.001). LORA had more frequent involvement of shoulders (P0.001), while elbow, wrist, metacarpophalangeal joint ( MCP), proximal interphalangeal joint (PIP) and ankle joints were more common in YORA (P0.001). LORA patients were more likely to have anemia than YORA patients (P0.05). No significant differences were found between LORA and YORA in specific serologic index, including rheumatoid factor and anticyclic citrullinated peptide antibody, and disease activity score 28-C-reactive protein (DAS28-CRP) and clinical disease activity index (CDAI). But LORA had a higher simplified disease activity index (SDAI) (P=0.002). Glucorcoticoid was used in 67.4% LORA patients, compared with 29.3% in YORA patients (P0.001). In contrast, disease-modifying anti-rheumatic drugs (DMARDs) were used in 73.7% LORA patients, compared with 97.6% in YORA patients (P0.001).Delayed diagnose of LORA is common due to atypical presentation at disease onset. RA should be considered in elderly patients with large joints for differential diagnosis. LORA is more likely to have anemia than YORA, albeit no significant differences in serological index and extraarticular presentations. LORA patients should be treated with DMARDs as aggressively as YORA patients, if their comorbidities allow to do so.

To investigate the clinical characteristic of rheumatoid arthritis associated with cold pattern knee joint pain.The study enrolled 60 patients of RA with the complaint of knee pain, 30 cases with cold pattern and 30 cases without cold pattern. The clinical symptoms, DAS28 score, cold knee score were collected, and serum hypoxia inducible factor-1alpha (HIF-1alpha) erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and thermal texture maps (TTM) of knee temperature were measured. The difference between two groups was compared to analyze the clinical characteristics of RA with cold pattern knee pain.There were differences between the two groups in the course (t=5.932, P=0.000), DAS28 score (t=2.716, P=0.007), knee (delta TTM) (t=7.731, P=0.000), absolute difference of thermal texture maps between popliteal fossia and front district in knee (ATTM) (t=14.295, P=0.000), CRP (t=5.684, P=0.000), ESR (t=4.506, P=0.000), HIF-1alpha (t=4.817, P=0.000).RA patients with cold pattern knee pain show the clinical characteristics with longer course of disease, lower level of local inflammation, lower disease activity.

To investigate the correlation of the receptor activator of nuclear factor kappa-B ligand (RANKL)/serum osteoprotegerin (OPG) system, Dickkopf-1 (DKK-1) and bone marrow edema (BME) in rheumatoid arthritis (RA) with the complaint of knee pain.The clinical data of 50 cases of RA with the complaint of knee pain were collected. According to MRI finding, half of them (25 cases) had bone marrow edema (BME). Each patient received the measurement of serum OPG, RANKL, DKK-1, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), anti cyclic citrullinated peptide antibody (CCP), rheumatoid factort(RF). The clinical symptoms, disease activity score 28 (DAS28), were compared between BME and non-BME griups, and the correlation between RANKL/OPG system, DKK-1 and BME of RA was analyzed.Compared with non BME group, BME group had shorter course (P=0.000), higher DAS28 score (P=0.009), CRP (P=0:000), RF (P=0.033) and CCP (P=0.012). lower level of serum OPG (P=0.000), higher level of RANKL (P=0.000), RANKL/OPG (P=0.000), and DKK-1 (P=0.001). The severity of bone marrow edema was correlated with the serum RANKL (volume r(s)=0.31, P=0.027; degree r(s)=0.33, P=0.022), RANKL/OPG (volume r(s)=0.29, P=0.039; degree r(s)=0.28, P=0.043), DKK-1 (volume r(s)=0.33, P=0.021; degree r(s)=0.34, P=0.019).BME is one of the early signs of bone erosion in RA, there were more active inflammation, autoantibodies, and osteocasts in RA patients with BME.

To compare of the efficacy of four assessment criteria for evaluating disease activity in rheumatoid arthritis.Clinical data were collected from 172 patients ofrheumatoid arthritis. Disease activity was evaluated by four assessment criteria, which are disease activity score 28-C-reactive protein (DAS28-CRP), disease activity score 28-erythrocyte sedimentation rate (DAS28-ESR), simplified disease activity index (SDAI) and clinical disease activity index (CDAI). The patients were divided into four groups which were remission, low, middle and high disease activity. The correlations and consistencies among four methods were compared.Disease activities evaluated by four methods in 172 RA patients were significantly positive correlated (P0.01). It was significantly consistent between SDAI and CDAI, also between DAS28-ESR and DAS28-CRP, the Kappa values were 0.949 and 0.862 respectively. The differences of the four methods in remission and high disease activity groups were no statistical significance. The proportion of the patients in low disease activity and middle disease activity groups evaluated by DAS28-ESR and DAS28-CRP were statistical different from that evaluated by CDAI and SDAI (P0.05).Strong correlation was observed between the four methods. CDAI could be used to evaluate whether RA is remission or activity. For disease activity assessment, DSA28-ESR and DAS28-CRP seems superior to CDAI and SDAI.

To explore the function and mechanism of CCL19 in the pathogenesis of rheumatoid arthritis.Synovial fibroblasts were collected from 5 cases of rheumatoid arthritis. Peripheral blood mononuclear cells (PBMCs) were obtained from 5 healthy people by Ficoll-Hypaque density gradien centrifugation. The cells were stimulated with IL-1beta, TNF-alpha, IL-17 and other cytokines, and then the expression of CCL19 was detected by RT-PCR. The cells also were treated with different concentration of CCL19, then the expressions of IL-1beta, TNF-alpha were detected by RT-PCR, the expressions of p-ERK, p-p38 were detected by western blot.IL-1beta promoted the CCL19/CCR7 expression in both synovial fibroblasts and PBMCs. CCL19 upregulated the expression of IL-10 in both synovial fibroblasts and PBMCs. The stimulation of CCL19 also increased its receptor CCR7 expression. CCL19 activated p-ERK and p-p38 in PBMCs.The positive feedback loop between CCL19 and IL-1 participate in the development of rheumatoid arthritis.

The objective of this study is to examine and evaluate whether serum 25(OH)D is associated with disease activity in patients with rheumatoid arthritis (RA). Our results suggested that serum 25(OH)D in RA groups has significant lower level (35.99±12.59 nmol/L) than that in the normal groups (54.35±8.20 nmol/L,P<0.05). Based on the DAS28, patients with RA were divided into four subgroups, and no differences were found in the four groups (P>0.05). The 25(OH)D levels in complete remission, low disease activity, middle disease activity, and high disease activity group were32.86±12.26,33.97±13.28,38.41±10.64, and38.94±13.35 nmol/L, respectively. Based on the serum 25(OH)D levels, patients with RA were divided into inadequate group and normal group, and there were no significant differences in baseline characteristics and disease activity in the two groups. Our results showed that serum 25(OH)D levels in the inadequate group are significantly lower than those in the normal group. However, no correlations were found between 25(OH)D levels and disease activity among 116 patients with RA. The present findings will help to understand the association between 25(OH)D and disease activity of RA.

Rheumatoid arthritis is a systemic autoimmune disease characterized by chronic inflammation of multiple joints, with disruption of joint cartilage. The proliferation of synovial fibroblasts in response to multiple inflammation factors is central to the pathogenesis of rheumatoid arthritis. Our previous studies showed that 4-HNE may induce synovial intrinsic inflammations by activating NF-κB pathways and lead to cell apoptosis. However, the molecular mechanisms of how synovial NF-κB activation is modulated are not fully understood. Here, the present findings demonstrated that 4-HNE may induce synovial intrinsic inflammations by mTORC1 inactivation. While ectopic activation of mTORC1 pathway by the overexpression of Pim-2 may disrupt the initiation of inflammatory reactions and maintain synovial homeostasis, our findings will help to uncover novel signaling pathways between inflammations and oxidative stress in rheumatoid arthritis development and imply that Pim-2/mTORC1 pathway may be critical for the initiation of inflammatory reactions in human rheumatoid arthritis synovial cells.

To the Editor: SAPHO syndrome is a heterogeneous disorder characterized by synovitis, acne, pustulosis, hyperostosis, and osteitis. Its diagnosis can be difficult if there are no typical skin manifestations. We reported a case of SAPHO syndrome which also fulfills the current diagnostic criteria, for ankylosing spondylitis (AS). A 62-year-old Chinese female patient was evaluated for her bilaterally swelling sternoclavicular joints in our center. She had a 30-year history of nonprogressive lower back pain and spinal stiffness without debilitating motion limitation of the spine and was once diagnosed of AS in our center according to the documented human leukocyte antigen B27 (HLA-B27) positivity. Ten years ago, she began to develop episodic pain in her sternoclavicular area and bilateral shoulders. Localized reddening and tenderness were noticed over her sternoclavicular joints and the medial clavicular areas during those episodes. Her pain could also be mitigated by oral meloxicam, and her daily activity was not affected. Physical examination showed enlarged sternocostoclavicular joints and deformed shoulders. Motion range of her hip joint, spine, and chest wall appeared normal. No acne or pustulosis-like rashes were noted. Laboratory assays revealed slightly elevated C-reactive protein at 20 ng/ml; erythrocyte sediment rate rose to 50 mm/h; spinal X-ray demonstrated wide-spread osteosclerosis, vertebral erosion, and multilevel bony bridging formation and marginal syndesmophytes [Figure 1a]. Chest computerized tomography (CT) revealed fusing destruction of her sternocostoclavicular joints with obvious hyperostosis [Figure 1b]. Pelvic CT showed obvious joint space narrowing, osteosclerosis and erosion of bilateral sacroiliac joints [Figure 1c]. Her scintigraphic bone scanning showed increased uptake in the sternoclavicular joint, sternum, and bilateral clavicles forming “bullhead sign”. Finally, this patient was treated by oral meloxicam and thalidomide along with yearly infusion of zoledronate. No relapse of joint pain was reported in 6 months follow-up. Figure 1 (a) Ankylosing change on the spinal X-ray with ivory vertebrae and syndesmophytes on multiple levels. Cortical erosion and hyperostosis of the vertebral bodies with bony bridging were also noted. In addition, sternocostoclavicular hyperostosis with obviously ... Diagnosis of SAPHO syndrome depends on the presence of at least one of three diagnostic criteria including osteoarticular manifestations of severe acne or palmoplantar pustulosis; hyperostosis of the anterior chest wall, limb, or spine; or chronic recurrent multifocal osteomyelitis (CRMO).[1] Although sternoclavicular joint is the most frequent site of the disease in adults, sacroiliac joint lesion and the spinal lesion are not rare. Paravertebral ossifications in SAPHO may simulate syndesmophyte in AS. Erosion of sacroiliac joint accompanied by florid sclerosis and hyperostosis are characteristic of SAPHO spectrum.[2] Nevertheless, in adults as high as 30% of SAPHO patients showed HLA-B27 positivity. Many SAPHO cases also fulfilled the diagnostic criteria of AS or spondyloarthropathies (SpA).[3,4,5] Interestingly, the spinal lesion and sacroiliitis, in this case, occurred 20 years previous to the sternocostoclavicular hyperostosis, and no skin lesion was found all through her 30 year's history. However, unlike typical AS or psoriatic arthritis, SAPHO is a benign disorder. Its spinal lesions are slow in progression and not, particularly debilitating.[1] Thus, the largely spared joints mobility and benign prognosis in this patient's spinal lesions after 30 years evolution favored the diagnosis of SAPHO syndrome. In conclusion, the axial skeletal lesions in SAPHO syndrome appear to be a gray zone overriding the current classification criteria for SAPHO syndrome and SpA. Hyperostosis and CRMO as SAPHO's most specific manifestations may help to distinguish it from classic SpA. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.