Objective To substantiate the relationship between connective tissue diseases and lung bullae. Method In 10 years, 70 patients with lung bullae were admitted to internal medicine inpatient wards of Peking Union Medical College Hospital, among which 12 connective tissue diseases were diagnosed. Two cases were excluded because their lung bullae was secondary. Results Six cases were Sj?觟gren′s syndrome (SS), two cases were vasculitis [one Takayasu disease and one was polyarteritis nodosa (PAN)], one was rheumatoid arthritis (RA) and one was undifferentiated connective tissue disease (UCTD). Symptoms of respiratory system were present in the patients with pulmonary interstitial fibrosis (five cases) and pulmonary amyloidosis (one case). Pulmonary function tests showed restrictive ventilatory dysfunction and obstriction of small airway (two cases), diffusion dysfunction (one case), restrictive ventilatory dysfunction (one case). A trans-bronchial lung biopsy specimen showed thickening of alveolar septa, chronic inflammation, proliferation of fiberous connective tissue and inflammatory cell infiltration. Conclusion Connective tissue diseases may result in lung bullae because they cause narrow of small airways.It is definitive in the relationship between SS and lung bullae, but further study needs to be done to clarify the relationship between other connective tissue diseases and lung bullae.

Objective To investigate the efficacy of tumor necrosis factor alpha (TNF-α) antagonists therapy and the possible eauses of new onset or exacerbation of psoriatic skin lesion in patients with arthritides treated with TNF-α atagonists therapy. Methods One patient with definite psoriatie arthritis and one patient with definite ankylosing spondylitis, who were treated with TNF-α antagonist therapy developed an unexpected exacerbation or new onset of psoriatic skin lesion, were investigated in this study. Furthermore, the literatures associated with psoriasis induced by anti-TNF-α therapy were reviewed. Results The patient with psoriatic arthritis experienced exacerbation of psoriatic skin lesion and the skin lesions subsided after discontinuation ofetanereept therapy. The skin lesions recurred with re-introduction of etanereept, which improved after withd-rawal of etanercept therapy. The patient with ankylosing spondylitis unexpectedly developed psoriasis vulgaris after receiving etanercept therapy. The skin lesion waxed and waned followed the administration or discon-tinuation of etanercept therapy. The same settings were reported in patients with rheumatoid arthritis receiving different types of anti-TNF-α therapy. Conclusion Blockage of TNF-α is highly effective in arthritides. Ho-wever, some patients with arthritides can unexpectedly develop either a new onset or exacerbation of psoriatic skin lesions after initiation of TNF-α antagonist therapy. The skin lesions subside after discontinuation of the TNF-α antagonist therapy, but the causes remain unclear.

Objective To evaluate the value of serologic teats in patients with primary biliary cirrhosis by conducting a seroprevalence survey for anti-gp210 and anti-sp100 antibodies.Methods A total of 72 consecutive primary biliary cirrhosis (PBC) patients with or without Sj(o)gren's syndrome (SS) and 50 patho logic controls were studied.Antibodies were tested by ELISA assays with recombinant spl00 and purified gp210.Results The positive rates of anti-gp210 detected by ELISA was 31.1% and 45.5% in PBC patients with and without SS respectively.Among SS and virus hepatitis patients,none had anti-gp210 antibody (P＜0.01).The prevalence of anti-gp210 was similar in PBC patients with and without SS.The positive rates of an ti-sp100 detected by ELISA was 14.8% and 18.2% in PBC patients with and without SS respectively.Among the patients with SS,only 3.3% was positive for anti-sp100,but none had anti-sp100 reactivity in virus hep atitis patients.The prevalence of anti-sp100 was not significantly different between PBC and SS groups. Conclusion Anti-gp210 and anti-sp100 are highly specific for PBC.The sensitivity of anti-gp210 and anti sp100 is 31.1% and 14.8% respectively.They may be helpful in the diagnosis of PBC.

Objective To investigate the significance of anticentromere antibody(ACA)in patients with primary biliary cirrhosis(PBC).Method ACA was detected using indirect immunofluorescence(IIF)in 99 patients with PBC,and the difference was compared between patients with and without ACA.Results Fifty-three patients(53.5%)had ACA in serum.The average age of onset was elder in patients with ACA than in patients without ACA[(52.6?1.5)vs(46.2?2.0),P=0.012].There was no significant difference in sex ratio between the two groups.Incidence of gastrointestinal bleeding was higher in patients with than without ACA (13.2% vs 0,P=0.014).Difference was not significant in symptoms such as fatigue,pruritus,icterus,etc. Though it didn't reach statistically significant,the incidence of esophageal varices was higher in patients with ACA than without ACA(45.8% vs 10.0% respectively,P=0.061).There was no significant difference in diam- eter of portal vein,splenomegaly,ascites.Biochemically only serum total protein reached statistical significance between the two groups[(73.3+1.1)g/L vs(78.1+l.7)g/L respectively,P=0.017].Patients with ACA had low- er serum IgG than ACA negative patients[(15.1?0.6)g/L vs(18.4?1.0)g/L respectively,P=0.006].Nuclear envelope pattern(NE),one of the patterns of ANA,was rare in ACA positive group than ACA negative group (16.7% vs 50.0% respectively,P=0.002).Conclusion The prevalence of ACA is high in patients with PBC. Patients with ACA have high risk of oesophageal varices and gastrointestinal bleeding.Nuclear envelope pat- tern of ANA is rare in patients with ACA.

Objective · To investigate the expression of androgen receptor (AR) in human breast cancer and its prognostic significance. Methods · A total of 183 female patients with breast cancer were selected. The expression of AR in breast cancer tissues was detected by immunohistochemical staining. The relationship of AR expression with the clinicopathological characteristics was analyzed. Survival analysis of AR gene expression by an online database (Kmplot) was also performed. Results · The positive rate of AR expression was 47.5% and was significantly higher in grade 1 and grade 2 group than that in grade 3 group (57.6% vs 25.0%, P<0.05). Breast cancer patients with estrogen receptor, progesterone receptor and P53 positive expression had significantly increased AR levels (P<0.05). AR expression were significantly higher in patients with luminal type as compared withthose in patients with human epidermal growth factor receptor-2 (HER2) positive and patients with triple negative breast cancer (51.7% vs 31.6%,P<0.05). Kaplan-Meier curves identified that AR gene expression was positively correlated with relapse-free survival, overall survival and distant metastasis-free survivalof breast cancer patients (P<0.05). Conclusion · The breast cancer patients with AR positive expression have a better prognosis, which suggests that inhibitors of AR pathway may be a treatment option for breast cancer.

ObjectiveTo analyze the clinical characteristics of systemic lupus erythematosus (SLE) patients with cerebral tuberculosis.MethodsTen SLE patients with cerebral tuberculosis admitted to Peking Union Medical College Hospital(PUMCH) from January 1995 to October 2010 were retrospectively analyzed.ResultsThe median duration from onset of SLE to cerebral tuberculosis was 1.5 years(range from 0.6 to 30 years).All patients had received higher doses of steroid therapy (equal to prednisolone l mg· kg-1·d-1 ),and 3 of them had received methylprednisolone pulse therapy.Nine patients had taken immunosuppressant.When cerebral tuberculosis was diagnosed,the most common features were fever and headache,followed by nausea/vomiting and focal neurological signs.Nine patients were complicated with extra-cerebral infections.The cerebrospinal fluid(CSF) pressures was(247±66) cm H2O(range from 180 to 350 cm H2O),which was higher than normal.The mean CSF protein level was(1.4±0.7) g/L (0.15～0.45 g/L),which was normal or increased.Image examinations were helpful for the diagnosis of cerebral tuberculosis in SLE patients.Pointenhanced or ring-enhanced lesions were found in enhanced magnetic resonance imaging( MRI ) of brain in all patients who had donethis test (8 patients).After treated with targeted antibiotics and specific adjuvant therapies,the symptoms had relieved in 7 patients,however,I patient died,and 2 patients were lost during the follow-up.ConclusionWhen SLE patients with stable disease have unexplained fever,headache,and focal neurological signs,cerebral tuberculosis should be considered.Enhanced MRI of brain and chest X ray are helpful for the diagnosis of infectious cerebral lesions and should be performed promptly.Extracerebral tuberculosis infections can provide clues forthe identification of the causativeagents.Anti-tuberculosis treatment should be initiated as soon as the diagnosis is confirmed,and early treatment can markedly improve the outcomes.

Objective To explore the changes of blood fat in patients with cerebral infarction complicated by metabolic syndrome (MS) receiving community path intervention treatment.Methods A total of 116 cases of cerebral infarction complicated by MS were selected and given comprehensive intervention treatment after risk assessment.Patients were divided into intervention group (60 cases) and control group (56 cases) according to their difference in compliance.Results After intervention,the total cholesterol (TC),triglyceride (TG) and low density lipoprotein cholesterol (LDL-C) in two groups were significantly decreased,high-density lipoprotein cholesterol (HDL-C) was significantly increased.There were significant differences in intervention group before and after intervention (P＜ 0.05).Compared with those in control group,differences of all index in each time point in intervention group were statistically significant (P ＜0.05).There was no significant difference in carotid plaque integral before intervention between two groups (P＞ 0.05).The carotid plaque integral 12 and 24 months after intervention in intervention group was significantly lower than that before intervention (P ＜ 0.05).The carotid plaque integral 12 and 24 months after intervention in intervention group was significantly lower than that in control group [(3.20 ± 2.01) cm vs.(4.71 ±2.87) cm,(2.98 ±2.61) cm vs.(4.60 ±2.43) cm,P＜0.05].Twelve and 24 months after intervention in intervention group,TC and carotid plaque integral was significantly positive correlation (r =0.304 and 0.317,P ＜ 0.05),TG and carotid plaque integral was significantly positive correlation (r =0.229 and 0.128,P ＜ 0.05),LDL-C and carotid plaque integral was significantly positive correlation (r =0.654 and 0.518,P ＜ 0.05),and HDL-C and carotid plaque integral was significantly negative correlation (r =-0.495 and-0.528,P ＜ 0.05).Conclusion For patients of cerebral infarction complicated by MS,great importance should be attached to early prevention and control of their major components so as to reduce the incidence of acute cerebrovascular recurrence and mortality.

Objective To analyze the clinical characteristics and prognosis of ANCA associated microvasculitis patients with gastrointestinal tract hemorrhage. Methods A retrospective analysis was conducted in 8 ANCA associated microvasculitis patients with gastrointestinal tract hemorrhage admitted to our hospital from May 1987 to May 2007. The statistical treatment is Kaplan-Meier. Results This group contained 4 male and 4 female patients. The average age was (39±20) years. The time from the onset of their disease to gastrointestinal bleeding was (8 ±8) months. Wegener's granulomatosis was the most common disease.Hematochezia(7/8 ) was the most common clinical manifestation and the distal small bowel (5/8) was the most common involved site. After the diagnosis was confirmed, methylprednisolone bolus therapy was used in 2 patients and 1～2 mg·kg-1·d-1 prednisone in 6 patients. Surgical operations were performed for 3 patients.However, 5 patients died. The prognosis was poor with the average survival time of (373±108) days. The mean survival time was (46 ±10) days after bleeding. Conclusion ANCA associated microvasculitis with gastrointestinal tract hemorrhage is rare. Early diagnosis and treatment could improve the prognosis.

Objective To investigate the clinical and prognosis characteristics of SLE patients whose initial clinical presentations were Sj(o)gren's syndrome (SS).Methods Medical charts of 41 consecutive SS/SLE inpatients admitted to Peking Union Medical College Hospital (PUMCH) from February 1998 to February 2008 were systematically reviewed,including demographic data,clinical features,laboratory findings,treatment as well as prognosis.Two hundred and fourteen cases were randomly selected as controls from 2331 non SS-onset lupus (NSs/SLE) inpatients weated in PUMCH at the same time period.Results There were significant differences between SS/SLE and NSS/SLE patients in the following aspects (P＜0.05):①gender composition:(F/M) (41/0 vs 184/30),age at the diagnosis of SLE [(43±41) yrs vs (32±31) yrs)],disease duration L(114±84) mollths vs (45±18) months];②clinical features:xerostomia (85.3% vs 6.1%),xerophthalmia (75.6% vs 2.3%),faeial rash (9.8% vs 46.3%),renal tubular acidosis (21.9% vs 0),nephrotic syndrome (7.3% vs 31.3%),central nervous system invoivement (4.9% vs 19.6%),interstitial lung disease (12.2% vs 2.8%);③labratory findings:ESR[(65±75) mm/1 h vs (46±34) mm/1 h)],patients with elevated IgG level (56.4% vs 29.9%) and IgA level (38.5% vs 20.4%),RF,prevalence of anti-SSA and anti-SSB antibodies (70.8% vs 20.3%,82.9% vs 43.4% and 39.0% vs 7.9%);④SLEDAI score (8±8 vs 10±10),glucocorticosteroid treatment (methylprednisolone bolus/1～2 mg·kg-1·d-1 prednisone/1 mg·kg-1·d-1 prednisone)(8/26/7 vs 91/102/21),and rate of death and/or severe irreversible organ failure (2.4% vs 14.9%).SS/SLE patients were followed up for (33±34) months,40 cases remained stable and only one patient died of acute pulmonary embolism.Conclusion Compared to NSS/SLE,SS/SLE patients ale older and have more insidious disease course.They have higher prevalence rate of anti-SSA and anti-SSB antibodies,renal tubular acidosis and interstitial lung disease,but less severe neuropsychiatric and renal involvements and much better prognosis.

Objective To investigate the frequence and clinical characteristics of peripheral neuropathy (PN) of patients with systemic lupus erythematosus (SLE) in China.Methods The data of 67 consecutive PN related with SLE (SLE-PN) patients admitted to Peking Union Medical College Hospital (PUMCH) during January 1995 to August 2011 were retrospectively analyzed.At the same time,a total of 201 cases were randomly selected as controls from 4447 SLE patients without SLE-PN in PUMCH during the same period.Chi-square test,t test and Mann-Whitney test were used for statistical analysis.Results The prevalence of SLE-PN in SLE patients was 1.5％(67/4514).Seventy-two cases of PN were discovered in 67 patients.Polyneuropathy was the most frequent presentation and was diagnosed in 45 cases(62.5％),mononeuropathy in 9 cases (12.5％),myasthenia gravis in 8 cases (11.1％),cranial neuropathy in 7 cases (9.7％),autonomic disorder in 2 cases (2.8％) and acute inflammatory demyelinating polyradiculoneuropathy in 1 case (1.4％).No plexopathy was diagnosed.No symptoms were observed in 5 cases (6.9％) PN.There were differences between SLE patients with and without SLE-PN in age[(37±14) years vs (32±14) years,P＜0.05],fever(65.7％ vs 47.8％,P＜0.05),myositis (17.9％ vs 5.0％,P＜0.01),skin and mucous involvement of skin and mucous (80.6％ vs 59.7％,P＜0.01),central nervous system involvement (38.8％ vs 19.9％,P＜0.01),gastrointestinal involvement (6.0％ vs 18.4％,P＜0.05),anti-Sm antibody positivity (34.4％ vs 18.6％,P＜0.05),anti-RNP antibody positivity (46.9％ vs 29.9％,P＜0.05),elevation of IgG (52.4％ vs 30.1％,P＜0.01),and SLEDAI score [12.0(9.0) vs 9.5(10.8),P＜0.05].Conclusion SLE-PN is not rare in patients with SLE.SLE patients with peripheral neuropathy tend to have more active disease.Multidisciplinary collaboration and comprehensive treatment can help to relieve pain and improve the quality of life.