Objective To investigate the clinical characteristics of Takayasu′s arteritis (TA) and the difference between male and female patients. Method The clinical features of 159 patients with TA were retrospectively analyzed. Results In our series of patients, the ratio of male and female was 1:3.1. The average age of male and female patients at onset of first symptom was (32?13) years and (24?9) years respectively (P

Objective To improve the understanding of progressive pseudorheumatoid dysplasia (PPD). Methods The clinical and roentgeno graphic features of two patients with PPD diagnosed in our department were analysed, and the related literature was reviewed. Results The patients first experienced the osseous swelling in phalangeal joints of both hands in childhood, and progressively almost all joints were involved. The spine was also involved. By analyzing the clinical information of 53 cases, it was found that PPD involved both male and female similarly. The ages at onset of first symptoms, were from 1 to 10 years, and in seventy seven percent of patients the ages were 3 years to 5 years. Clinical features included progressive involvement of the major joints, including small joints of the hands, hips, knees, ankles, wrists and shoulders. Premature osteoarthritis developed in early adult life, and it was the major reason of disability. 38% of patients were short in stature. The roentgenographic features consisted of generalized platyspondyla with irregular delineation of the endplates of the vertebral bodies, varying degrees of epiphyseal involvement with enlargement of the large joints, metacarpal heads and phalanges, secondary degenerative arthritis with periarticular osteoporosis. The symptoms of PPD were similar to those of rheumatoid arthritis (RA), but differed from it by the Absence of synovitis and other inflammatory changes, and radiographically by the Absence of destructive changes and the presence of dysplastic bone changes. There was no specific treatment for cure. Conclusion PPD is a rare autosomal recessive skeletal disorder associated with WISP3 gene mutations. Its clinical features and typical roentgenographic features are helpful to the diagnosis.

Objective:To study the effects of compatibility circumstances on guizhi's effect in dispelling cold and relieving exterior in complex prescriptions. Methods:202 formulae that effected on Sanhanjiebiao were collected from 1425 formulae with guizhi,and the frequency of herbs combining with guizhi in 202 formulae was ranked from high to low. Results:According to the compatibility frequency from high to low,herbs are:Radix Ledebouriellae(60.89%) ,Herba Ephedrae(36.63%) ,Notopterygium Incisum Ting ex H.T.Chang(27.23%) ,Radix Angelicae Dahuricae(14.85%) ,Rhizoma Zingiberis Recens(11.39%) ,Herba Schizonepetae(5.94%) ,Radix Ledebouriellae and Herba Schizonepetae(4.46%) successively. Conclusion:It showed that guizhi should combine with corresponding herbs to form compatibility circumstances to exert the effect of Sanhanjiebiao in formula,and the function exertion will be effective.

Objective To study the imaging features of sacroiliac joints(SIJ) in patients who were misdiagnosed as spondyloarthritis(SpA).Methods A total of 34 patients with chief complaint of back pain and misdiagnosed as SpA from January 2007 to April 2013 in Department of Rheumatology Chinese PLA General Hospital were enrolled.The imaging,clinical manifestations,laboratory examinations data were analyzed.Result The main reason for misdiagnosis as SpA was because of sacroiliitis presenting on imaging.The final diagnoses included 24 patients as SIJ infection,4 patients as neoplastic diseases,2 patients as metabolic bone diseases,2 patients as sacroiliac joint degeneration,1 patient as gout of sacroiliac joint,1 patient as diffuse idiopathic bone hypertrophy.For patients with infection,there were 10 patients receiving X-ray and 22 patients receiving CT of SIJ.However,5 and 7 patients had negative results respectively.These patients with infection had abnormalities in MRI including all with bone marrow edema,21 patients with erosion of bone and joint,22 patients with muscle involved.As to the patients with malignancies,SIJ CT scan appeared normal.Bone marrow edema and erosion in MRI were found in all neoplasm patients expect one as ependymoma.Adjacent muscles were involved in the patient with Ewing's sarcoma.Either X-ray or CT in other patients demonstrated obvious abnormalities,but only mild erosion of bone was found in MRI.Conclusion Bone marrow edema of SIJ in MRI represented not only in patients with SpA.Rheumatologists should analyze the clinical manifestations and laboratory examinations comprehensively in order to avoid the misdiagnoses.

Objective To determine whether thalidomide can maintain remission after discontinuing the treatment of etanercept in ankylosing spondylitis (AS). Methods One hundred and five patients with active AS treated with etanercept 50 rag/week for 12 weeks and attained an ASAS20 response at week 12. They were randomly assigned to receive thalidomide 150mg/night, sulfasalazine (SASP) 2.0 g/d, or non-steroidal anti-inflammatory durgs (NSAIDs) only. All patients were followed monthly for BASDAI, BASFI, PGA and spinal pain VAS. A Kaplan-Meier survival analysis was used to calculate the probability of a relapse. Results One hundred patients completed the follow-up. Thirty patients were treated with thalidomide, 33 patients with SASP and 37 patients with NSAIDs only. The mean follow-up time was 5.1 months and the longest time was 12 months. At the end of the follow-up, the percentage of patients who maintained remission in the thalidomide group was 40%, much higher than SASP group (15%) and NSAIDs group 11% (P=0.0265 and 0.0053 respectively). No difference was found between the remission rate of SASP and NSAIDs only group (P=0.5881). Conclusion Thalidomide can successfully maintain remission of AS after discontinue etanercept treatment.

ObjectiveTo explore the diagnostic and differential diagnostic points in a patient with rheumatoid arthritis (RA) complicated with fever and pulmonary diseases. MethodsFull clinical analysis was performed for a 55-year old patient with arthritis, fever, and wheeze. ResultsThe clinical pictures of the patient were consistent with those of RA. Computed tomography(CT) revealed interstitial lung diseases and intrapulmonary cavities. Antifungal agents were given experimentally, and the intrapulmonary cavities disappeared finally, thus the patient was diagnosed as RA, concurrently complicated with interstitial lung disease and pulmonary fungal infection. ConclusionThe possibility of pulmonary fungal infection should be considered in RA patients who presented with fever and intrapulmonary cavities.

Objective To understand the features of immunoglobulin G4-related diseases and to improve its diagnosis and treatment.Methods The clinical data of 20 cases discharged from our hospital were analyzed retrospectively.Results Twenty cases with IgG4 related diseases were reviewed retrospectively.IgG4-related diseasesis more prevalent in male than in female (14∶6),and the mean age was 58 years.Pancreas and bile ducts were the most commonly affected organs,2 and more than 2 organs were involved in 17 patients.All patients had significantly elevated serum immunoglobulin G4.Histopathological findings included diffuse tissue fibrosis with extensive lymphoplasmacytes infiltration.On immunohistochemical staining,the ratio of IgG4-positive plasma cells to IgG-positive plasma cells was higher than 40％.One patient deceased and one patient was left untreated,other patients responded well to the treatment.Conclusion There is no characteristic clinical symptoms for IgG4-related diseases,and serum immunoglobulin G4 is the essential diagnostic method in suspected cases.IgG4-related diseases can be well controlled with prompt use of glucocorticosteroid.

Objective The purpose of this paper is to study the variations of the bone conduction hearing in the patients with chronic suppurative otitis media complicated by bone conduction deterioration after tympanoplasty and to analyze the relative factors. Methods In 54 cases (61 ears) of decreased bone conduction thresholds associat-ed with chronic suppurative otitis media, reviewed retrospectively were the data before the operation and during the follow-ups from 3 to 27 months. The bone conduction thresholds at 0. 5,1, 2 and 4 kHz were studied in relation to the courses of disease, the complications of cholesteatoma, the operation procedures and the reconstructions of os-sicular chains before and after surgeries. Results Before operation the most obvious hearing loss of bone conduction was found at 4 kHz in 61 ears, while after operation the average bone conduction hearing at the 4 frequencies was improved at different degrees with the most at 2 k Hz. Thirty-two of 61 cases (52.50%) were found to have their hearing improved at least by more than 10 dE at two frequencies without any deterioration at the 4 frequencies. Con-clusion The factors of the duration of disease and complications of cholesteatoma are obviously not related to the improvement of hearing after operation. A patient with reconstruction of ossicular chain is markedly superior to one without the reconstruction in improvement of hearing. When the auditory ossicular chains and functions of the oval and/or round windows are damaged, the bone conduction hearing can be affected accordingly. However the hearing of some cases will be improved after tympanoplasty. Also, the possible improvement is also related to different sur-geries in treating patients with middle ear infections.

Objective To evaluate the efficacy profile of loading dose of anti-tumour necrosis factor (TNF)-α antibody infliximab in the treatment of ankylosing spondylitis (AS) and investigate the parameters that could be used as predicting factors for clinical response to infliximab in AS.Methods This was an open-labeled.mutticenter trial.Subiects eligible for this study were adults with a diagnosis of definite AS.Active disease was a Bath AS disease activity index(BASDAI)≥4 and spinal pain VAS≥4.Infliximab 5 md/kg was infused at weeks 0,2,6.All patients were followed for up to 10 weeks.Logistic regression likelihood ratio tests (univariate and multivariate) were performed to investigate which of the following parameters assessed before treatment were associated with ASAS 20,ASAS 40 and BASDAI 50 response:sex.age.disease duration,HLA-B27,BASDAI,spinal inflammatory (questions 5 and 6 of the BASDAI),spinal pain,night pain,patient global assessment,Bath AS functional index (BASFI),BASMI,enthesis index (EI),presence of peripheral arthritis,chest expansion,ESR,CRP,Bath AS radiology index (BASRI).Results Sixty-three patients (79% were males.90% were HLA-B27 postivie with a median age of 32 years and the median disease duration was 10 years) completed the treatment.The proportion of ASAS 20 responders.ASAS 40 responders and BASDAI 50 at week 10 was 84%,75%,70%,respectively.Univariate and multivariate Logistic regression analysis showed none of the 17 parameters evaluated at week 0 including ESR/CRP,disease duration were predictive for improvement.Conclnsion In this study,a loading regimen of infliximab has demonstrated consistent efficacy and none of the demographic parameters and baseline AS activity parameters are predictors for maior clinical response to infliximab in active AS.

Objective To evaluate the efficacy and safety profile of a recombinant human tumor necrosis factor receptor: Fc fusion protein in ankylosing spondylitis (AS). Methods This was a multicenter,randomized, double-blind, placebo-controlled trial in the first 6 weeks and then followed by an open-labeled trial in the next 6 weeks. One hundred and forty-three patients of active AS were randomly assigned to receive 25 mg twice-weekly subcutaneous injections of rhTNFR:Fc or placebo for 6 weeks. The primary endpoint was proportion of ASAS20 responders at week 6. The secondary endpoints were the proportion of subjects achieving a BASDAI 20%, BASDAI 50% and BASDAI 70% improvement at week 6. Other secondary endpoints, related to reducing signs and symptoms of AS and improving range of motion and physical function, were evaluated.Results Treatment with rhTNFR:Fc resulted in significant improvement. At 6 weeks, 68% of the 71 patients in the rhTNFR: Fc group had a treatment response, as compared with 28% of those in the placebo group(P＜0.01). Improvements over base-line values for other measures of disease activity were significantly greater in the rhTNFR:Fc group, rhTNFR:Fc was well tolerated, The most frequently treatment related adverse event was injection site reaction. Conclusion rhTNFR:Fc has demonstrated consistent evidence of efficacy and is well tolerated in the treatment of active AS.