Rheumatic diseases are characterized by immunological dysfunction.T-cell medicated immune response plays a very important role in the development and persistence of rheumatic disease.CTLA-4 is an important co-stimulatory molecules expressed on T cells.It has inhibitory effect on T cell-mediated immune response.Plenty of evidences have demonstrated abnormalities in CTLA-4 alleles and proteins in patients with rheumatic diseases.Therefore,CTLA-4 has become an target in treating rheumatic diseases.CTLA-4Ig has been developed as an fusion protein which is composed of extrA-cellular domain of human CTLA-4 and the Fc fragment of human IgG1.So far,only data about CTLA-4Ig in treating rheumatoid arthritis are available.These data showed that CTLA-4Ig could relieve the symptoms of rheumatoid arthritis and retard the development of the disease.It is safe and effective.In addition,effectiveness was demonstrated even in patients who failed to response to anti-TNF monoclonal antibodies.Therefore,CTLA-4Ig is an encouraging therapy for rheumatic diseases.However,because of very short history,long-term study is needed to fully understand its role in the treatment of rheumatoid arthritis.

Objective To explore the clinical characteristics, treatment and outcomes of patients with panniculitis. Methods Clinical data of patients with panniculitis admitted to our hospital were analyzed and followed up. The disease was classified into two sub-types: cutaneous type and systemic type. The clinical profile and treatment as well as the outcomes were compared. Results The mean follow up time of 61 in-patients with panniculitis was 49.2 months (range: 2～216 months). Remission could be achieved in 17 patients, recurrent episodes happened in 26 patients. Eighteen patients turued out to be diffuse connective tissue disease (CTD), hematological disorders, malignant tumors and tuberculosis during follow-up. The age at sympton onset was 33±17 years old in 43 patients with primarily panniculitis which eutaneoua sub-tye was 12 patients and systemic sub-tye was 31 patients. The male: female ratio was 1:15. Patients with systemic sub-type were associated with liver (25 patients), spleen (23 patients), respiratory system (11 patients), kidney (12 patients), and circulatory (3 patients) system involvement. Nine patients with systemic sub-type presen-ted white blood cell decrease while none of in the cutaneous sub-type. The difference was significant (P=0.044). Biopsy of the lesions had shown nuclear dust in 16.3% of primarily panniculitis patients. Conclusion Pannieulitis can associate with systemic rheumatic diseases, malignant tumors, hematological disorders and tuberculosis. The main systemic involvements with systemic sub-type are liver and spleen, and may have whiteblood cell decreasing. Some patients could achieve remission when treated with corticosteroids and/or immunosuppressive drugs. However,the majority of patients will have recurrences when corticosteroids is tapered.

Objective To investigate the difference and treatment strategy of malignancy-associated dermatomyositis and other para-neoplastic neurological syndromes (PNS).Methods The clinical characteristics of a patient with malignancy-associated dermatomyositis and poly radiculoneuropathy was reported and the relevant literature was reviewed.Results Patients with dermatomyositis had increased risk of malignancies,and should routinely screened.Dermatomyositis and polyradiculoneuropathy was clinically similar,but could rarely be seen in the same malignant patient.Malignancy -associated dermatomyositis and PNS had similar pathogenesis.The treatment strategy of both was similar.Malignancy specific treatment should be initiated and immune suppressive agents should be prescribed concurrently.Conclusion Rheumatolgists should aware the association between dermatomyositis and potential underlying malignancies.Multiple para-neoplastic syndromes could be seen in the same patient,but the diagnosis should be considered as one.

Objective To analyze the peripartum complications,frequency of neonatal abnormalities and the associated factors in patients with primary Sj?grenˊs syndrome (SS). Methods The chart of 39 patients with primary SS who were admitted to the hospital for delivery were retrospectively reviewed. The clinical data, obstetrical outcome and the frequency of neonatal abnormalities as well as the possible associated factors were analyzed. Results There were 76 pregnancies and 41 deliveries among these 39 patients. Two patients (5%) had pregnancy-related hypertension, 1 (3%) had gestational diabetes and 1 (3%) had eclampsia. Twenty-eight(72%) patients had at least one episode of complication. In which, 27(68%) were induced abortion, 2 (5%) were premature birth, 6 (21%) were amniotic fluid volume abnormalities, 2 (5%) were post-partum bleeding, 5 (13%) were premature rupture of membrane, 6 (21%) were intra-uterine distress and 3 (8%) had intra-uterine growth retardation. Fetal abnormality was detected in 7(18%) patients, in which 3 fetus (8%) died before delivery, 2 fetus (5%) were small than gestational age and 2 (5%) had fetal deformity. Compared to the reports in the literature, the rate of abortion and fetal death was higher, but the rate of growth retardation , induced abortion was lower. Conclusion The frequency of peri-partum complication and fetal abnormality is increased in patients with primary SS. Pregnancies in patients with primary SS should beclosely monitored by rheumatologists.

Objective To estimate the frequency of heart valvular involvement in Chinese patients with Takayasu arteritis (TAK) and its correlation with other clinical features. Methods Ninety-one patients with TAK were studied and their clinical manifestations, laboratory test, vascular images and echocardio - gra-phic findings were retrospectively reviewed. The data were compared between patients who had heart valvular involvement and those who had not. Mann-Whitney U-test or Student's t-test, Chi-squared test or Fisher's exact test were used for analysis. Results Aortic regurgitation and mitral regurgitation were both confirmed in 20 (22%) patients. Tricuspid regurgitation and pulmonary valve regurgitation were observed in 10 (11%) and 2 (2%) patients, respectively. Older age at onset, longer disease duration, hypertension and involvement of the ascending aorta were all significantly more frequent in TAK patients with aortic regurgitation (t=2.903, P<0.01; Z=-2.759, P<0.01; χ2=7.918, P<0.01; Z=-4.454, P<0.01) than those without. However, the constitutional symptoms and acute phase reactants [erythrocyte sedimentation rate (ESR) and C reactive protein (CRP)] were not statistically different between patients with and without valvular regurgitation (P>0.05). Aortic valvular pathology examination of the three TAK patients who had underwent aortic valve replacement showed no inflammatory cell infiltration. Conclusion This study has demonstrated that heart valve involvement is common in patients with TAK, mainly in the form of regurgitation, and which may be secondary to hypertension and other cardiovascular complications. We should pay more attention to the screening and treatment of heart valvular involvement in TAK patients.

Objective To evaluate the clinical diagnostic contribution of 18 F-FDG PET/CT in giant cell arteritis with initial presentation as fever of unknown origin (FUO).Methods Eight cases with initial presentation as FUO diagnosed with the contribution of PET/CT were retrospectively studied in Peking Union Medical College Hospital.The radiologic manifestations of PET/CT were analyzed.Results Eight patients (4 men and 4 women) with average (63 ± 7) years (range from 55-75 years) were included in our study based on the criteria.Non-specific clinical symptoms were common in these patients,including fatigue,night sweat and weight loss.They all suffered from anemia of chronic disease.Not surprisingly,the inflammatory parameters were elevated significantly in all patients.The medical history,physical examination and routine lab and radiologic examinations couldn't reveal the causes of fever.PET/CT was performed in all of them,which demonstrated intense 18F-FDG uptake in the area of aorta and its major branches with maximal standard uptake value (SUVmax) 2.1-4.6 (3.6 ± 0.9).Immunosuppressive agents were effective to control the inflammation activity.The SUV decreased significantly after treatment in the follow-up PET/CT.Conclusions PET/CT has demonstrated high yield of diagnostic contribution in giant cell arteritis with initial presentation as fever of unknown origin.As to elderly FUO patients presenting with prominent inflammatory reaction,PET/CT may provide potential value to differentiate diagnosis from maligancies.

Objective To improve the understanding of drug-induced lupus (DIL) and the differences from systemic lupus erythematosus (SLE).Methods Clinical manifestation and treatment of patients with definite DIL were retrospectively analyzed.Results Six patients with DIL were enrolled in this study,including 4 females and 2 males.Two patients were diagnosed after receiving interferon,one after soluble tumor necrosis factor receptor fusion protein,one after propylthiouracil,one after penicillamine,and one after levofloxacin.High titer of antinuclear antibody was identified in all six patients,including 3 with positive anti-dsDNA antibody.One patient had positive anti-Sm antibody.One patient had positive anti-RNP antibody.One patient had anti-nucleosome antibody.One patient had anti-histone antibody.One patient had antimitochondrial antibodies-M2,and one patient had anticardiolipin antibodies.Conclusion Patients with DIL are not as severe as those with SLE.After cessation of suspected drugs and administration of standard treatment,the clinical outcome of DIL is satisfying.

With the recognition of antineutrophil cytoplasmic antibodies (ANCA)-related vasculitis and widespread vaccination against viral hepatitis B, the prevalence of polyarteritis nodosa (PAN) varied considerably. In our study, patients diagnosed as polyarteritis nodasa (PAN)based on the 1990 American College of Rheumatology(ACR) criteria were reclassified using 2007 European Medicines Agency(EMA) algorithm modified by 2012 Chapel Hill Consensus Conference(CHCC) definitions, aiming to evaluate the new classification criteria for the diagnosis of PAN. A total of 113 PAN patients admitted to Peking Union Medical College Hospital from January 2002 to December 2018 were retrospectively analyzed, who were classified into three subtypes including 9 patients with cutaneous, 80 with classic and 24 Hepatitis B virus (HBV) associated PAN. All patients were reclassified according to 2007 EMA algorithm using CHCC 2012 definitions. As a result, 7 patients were diagnosed as microscopic polyangiitis(MPA) and 19 patients with unclassified vasculitis based on the new classification criteria. The diagnostic rate of PAN was gradually declined as the classification criteria of vasculitides was update. However, there are quite a few PAN patients in China, whom rheumatologists should pay attention to the early diagnosis and treatment.

One 51 years old man was admitted to the rheumatology department with a history of prominent eyes, headache and blurred vision for half year. The main manifestations included retrobulbar inflammatory pseudotumor and retroperitoneal fibrosis. He was initially diagnosed as granulomatosis with polyangiitis. Prednisone and cyclophosphamide were administrated and effective. New mass of dura mater and osteosclerosis presented during follow up. Finally Erdheim Chester disease(ECD) was diagnosed by biopsy and pathological examination. Vemurafenib, a v-raf murine sarcoma viral oncogenes homolog B1 (BRAF) inhibitor, 480 mg was given twice a day. The patient′s condition is stable and still in follow-up. Although ECD is a rare histiocytosis, clinicians should pay attention to its manifestations and differential diagnoses.

Objective To investigate the clinical efficacy of Dahuang Xiaozhi Suppository in treating chronic abacterial prostatitis/chronic pelvic pain syndrome (CABP/CPPS) of moist heat and blood stasis syndrome. Methods Two hundred patients of CABP/CPPS were randomly divided into control group and observation group, 100 cases in each group. 2 groups were disabled anti-infective meidicne and other preparations, and received diet and life intervention. The control group received the treatment of Qianliean Suppository, and the observation group received the treatment of Dahuang Xiaozhi Suppository, one capsule each time, once a day, into the anus depth of about 3–4 cm. Ten days were a treatment course with two day interval between each course of treatment. The treatment lasted for three courses. The clinical efficacy of the two groups was observed and compared. The indexes including the scores of NIH-CPSI, leukocyte count and lecithin corpuscles in prostatic fluid, and urinary flow rate were evaluated. Results The total effective rate was 100% (100/100) in the observation group and 78% (78/100) in the control group, and the observation group was much higher than control group (P<0.05). The scores of NIH-CPSI and leukocyte count in both groups were significantly lower after treatment (P<0.01), and those in observation group weresignificantly lower than those in control group (P<0.01). The cases with lecithin corpuscles++++in both groups were significantly more after treatment (P<0.01), and those in observation group were significantly more than those in control group (P<0.01). The peak flow rate and mean flow rate in both groups were higher after treatment (P<0.01), and it was higher in observation group (P<0.01). Conclusion The efficacy of Dahuang Xiaozhi Suppository in treating CABP/CPPS of moist heat and blood stasis syndrome is remarkable, and it is better than Qianliean Suppository.