Objective To summarize the clinical characteristics of patients with connective tissue diseases (CTD) and autonomic neuropathy. Methods The medical records of inpatients with CTD and autonomic neuropathy from 2005 to 2015 were retrospectively analyzed including clinical manifestations, laboratory examinations, treatment and outcome. Categorical data were expressed in percentages. Kolmogorov-Smirnov test was used to examine normal distribution. Continuous data of normal distribution were expressed as x ±s deviation, while data without a normal distribution were described as median and interquartile range (P25, P75). Results Among the nine patients included in this study, all were female, and the median age was 42 years (32~50 years old). Four patients (4/9) were systemic lupus erythematosus (SLE), three patients (3/9) were primary Sj?gren's syndrome (pSS), two patients (2/9) were rheumatic arthritis (RA), and four patients were secondary Sj?gren's syndrome (SS) (two with SLE and two with RA). Five patients (5/9) had autonomic nervous dysfunction before they were diagnosed of CTD, while four patients (4/9) developed autonomic nervous dysfunction after diagnosis of CTD. The most common symptom of autonomic nervous dysfunction was postural hypotension (9 patients, 9/9), followed by hypohidrosis (4 patients, 4/9), urinary retention (2 patients, 2/9), gastrointestinal dysmotility (2 patients, 2/9) and tonic pupil (1 patient, 11%). After treatment of CTD, autonomic symptoms of three patients improved, while the others didn't. Four of the remaining 6 patients improved after receiving other assistant treatments including vasoconstrictor, pyri-dostigmine bromide, and plasma exchange. Conclusion Patients with CTD could present with autonomic neuropathy, which is mainly characterized with postural hypotension. If patients had related symptoms, clinicians should pay more attention to whether CTD exists. If treatment for CTD couldn't improve patients' condition, other assistant treatment might be considered.

Objective To analyze the genetic quality and to determine the strain of laboratory mice. Methods Ten strains of mice were analyzed by using AFLP method. Results Polymorphism was detected in 10 strains of mice by 17 single enzyme primers and 20 pairs of double enzyme primers amplification. A total of 251 bands were shown by single enzyme AFLP in agarose gel with the size of the bands ranging from 100 bp to 2 000 bp and 89 polymorphic loci were detected. A total of 1507 clear bands between 50 bp and 600 bp were shown by double enzyme AFLP and 378 polymorphic loci were detected. Through statistical analysis, we calculated the similar index and genetic distance index. Our results showed BALB/c and BALB/c nu had the closest relationship and KM had a closer relationship with TA2, BALB/c and BALB/c nu, while DBA/2 showed a distant relationship with T739, 615 and C57BL/6J, coinciding with the origins of breeds. Conclusion Each strain could be distinguished from others by using the AFLP polymorphic primers, which provides reference data for genetic quality analysis and strain determination of mice.

Objective To study the clinical profile of patients with central nervous system (CNS) involvement in relapsing polychondritis (RP).Methods Clinical data of five patients of RP with CNS lesions were collected,and compared with those from the literatures.T test and Fisher exact test were used for statistical analysis.Results Among 205 patients with RP in Peking Union Medical College Hospital,five cases (2.4％) had CNS damage.All 5 cases presented an active onset and occurred in the active phase of RP.All of them manifested as meningoencephalitis,complicating cranial neuropathies in 2 cases (2/5).Cerebrospinalfluid examination revealed non-specific meningeal inflammation,and magnetic resonance image (MRI) showed long T2 signals in brain lesions.Four patients (4/5) showed good response to high-dose glucocorticosteroid plus immunosuppressive agent combined therapy.The average age of our patients was younger than those in the literatures [(44± 14),(58± 11) years,respectively; t=2.547,P＜0.05],while other clinical features was not significantly different between the two groups.Conclusion CNS involvement is a rare condition in RP patients,and usually occurrs in the early course of active RP.Meningoencephalitis/ meningitis is the major clinical manifestations.MRI and cerebrospinal fluid examination may help to confirm the diagnosis.Treatment with corticosteroid and immunosuppressant can result in favorable response.

Objective To compare the clinical response with etoricoxib 60 mg once daily with diclofenac sodium tablet 75 mg two times daily in the treatment of osteoarthritis of the knee or hip joint.Methods A 4-week multicenter,randomized,double-blinded and active comparator-controlled clinical trial was performed during January 2005 and June 2005 in 6 medical centers in China.Eligible patients (≥40 years old Chinese patients with osteoarthritis of the knee and hip) were randomized (1:1 ratio) to receive etoricoxib 60 mg once daily (n=90),or diclofenac sodium 75 mg twice daily (n=90).Primary efficacy end point is the change of WOMAC (Western Ontario and McMaster Universities osteoarthritis index) pain subscale from baseline to 4 weeks; non-inferiority bounds were pre-defined [if the upper bound of 95％ confidence interval (CI) for the difference is less than 10 mm on a 100-mm VAS WOMAC pain subscale] for the comparison of the change between the two groups.The secondary efficacy endpoints include WOMAC physical function subscale,WOMAC stiffness subscale,patient's global assessment of response to therapy (PGART),investigator's global assessment of disease status (IGADS),discontinuation due to lack of efficacy and rescue paracetamol tablet count.Safety was assessed by physical examination,adverse experience reported,and laboratory safety data.Results C6mpared to baseline,the changes of WOMAC pain subscale after 4 weeks treatment were statistically significant (P＜0.01) in both groups (etoricoxib group:51±16 vs 21± 19; diclofenac sodium group:53±16 vs 22±19).There was no difference in the change of WOMAC pain subscale between the two groups.The change in WOMAC stiffness subscale,WOMAC physical function subscale,PGART and IGADS in both groups were statistically significant (P＜0.01),but there was no difference between treatment groups according to the pre-defined non-inferiority criteria.No drug related serious adverse events were observed during the study.The difference in drug-related adverse event incidence between the two groups was not statistically significant.Etoricoxib and diclofenac sodium were generally safe and well tolerated.Conclusion Etoricoxib 60 mg administered once daily is efficacious and shows clinical efficacy notinferior to that of diclofenac sodium 75 mg administered twice daily for the treatment of osteoarthritis.Etoricoxib 60 mg administered once daily for 4 weeks is generally safe and well tolerated.

Objective To summarize the clinical characteristics of SLE patients complicated with Listeria monocytogenes meningitis (LMM).Methods The clinical manifestations,systemic involvements,laboratory tests,cerebrospinal fluid (CSF) examinations,treatment and prognosis of SLE inpatients combined with LMM in Peking Union Medical College Hospital during Janurary 2000 to December 2015 were retrospectively analyzed.Results Eight SLE patients with LMM accounted for 0.14％ of the 5 675 hospitalized SLE patients.Seven were female and 1 was male,with the mean age of (35±16) year.Headache (7/8) was the most common neurological symptom followed by positive meningeal irritation sign (5/8),unconsciousness (3/8) and nausea or vomiting (2/8).Increased intracranial pressure was found in 7 cases and 4 of them were even higher than 330 mmH2O.CSF protein elevated in 6 cases and CSF glucose decreased in 6 cases.LMM often occurred in inactive or mild active SLE with the average SLE disease activity index (SLEDAI) score of 4.7±1.5.Systemic manifestations included hematologic changes (6/8),rashes (4/8),renal involvement (4/8) and arthritis (3/8),etc.Treated by autibiotics such as penicillin and medications for SLE,7 patients achieved clinical improvements,1 patient died.Conclusion LMM is one of the opportunistic infections in SLE patients and tends to occur in inactive lupus cases.There are unspecific clinical and laboratory features and CSF changes which maybe overlap and mix up with systemic manifestations of SLE.That's why it is necessary to take continuous cultures of CSF.Early identification and appropriate treatment lead to prognostic improvement of these patients.

Objective To study the clinical, laboratory, radiological and pathological findings of patients with hypertrophic cranial pachymeningitis (HCP) in Wegner's granulomatosis (WG) to improve the recognition of the disease, even when it occurs in limited form. Methods Three patients were described and English literatures of biopsy-proven pachymeningitis in WG were reviewed. Results The features of WG-associated pachymeningitis included: ① Frequently occurred early in the course of active limited WG; ② Commonly presented with sever headache and cranial neuropathies in the absence of other meningeal irritative signs; ③ Variable cerebrospinal fluid findings with mild predominantly lymphocytic pleocytosis and elevated protein concentration were major laboratorg findings; ④Elevated ESR and positive serum anti-neutrophilic cytoplasmic antibody (ANCA) could be found in most patients; ⑤ Gadolinium-enhanced brain MRI is very senitive in the detection of pachymeningitis; ⑥A dural biopsy showed granulomatous necrotizing inflammation, giant cell, and evidence of vasculitis;⑦ A favorable response to standard treatment with corticosteroid, cyclophosphamide or other cytotoxic drugs could be observed. Conclusion HCP may be the initial or cardinal manifestation of the limited form of WG. Early diagnosis by ANCA, MRI and dural biopsy may facilitate diagnosis Corticosteroid and immunosupressant are the choices of treatment.

Objective To study the clinical profile of patients with multicentric reticulohistiocytosis (MRH).Methods The clinical manifestations,image and histologic characteristics of ten patients with MRH were collected,and the relevant literatures were reviewed.Results Seven cases were women.The median (quartile) age was 46.5 (44.5-55.3) years.Clinical symptoms included papulonodular lesions on the skin (ten cases),erosive polyarthritis (ten cases),myositis (four cases),lung nodules (three cases),larynx nodules (one case) and serositis (one case).Biopsy demonstrated infiltration of multinucleated giant cells with CD68 positive and glassy cytoplasm.All of the ten patients were treated with corticosteroids and disease-modifying antirheumatic drugs,skin lesions of seven patients were ameliorated and arthritis was improved in three patients.Conclusion MRH is a systemic disease that has characteristic clinical manifestations and unique histological findings.Deep understanding of this disease can avcid.isdiagnose.Further studies are needed to explore effective treatment.

AIM: To assess the role of surface free radicals and electromotive voltage of fibrous mineral dusts in rabbit pulmonary alveolar macrophage injuries induced by fibrous mineral dusts. METHODS: Changes in cell death ratio, malandialdehyde (MDA) and cellur electrophoresis ratio, lactate dehydrogenate (LDH)and superoxide dismitase(SOD) activities were determined, the technique of cell culture and Scanning electron Microscopy were used to examine the change of membranous permeability, charge and cellular shape. RESULTS: Fibrous wollastonite and tabulate clinoptilolite, which had no OH-, had no cytotoxicity, while fibrous sepiolite, fibrous palygorskite, fibrous brucite and chrysolite asbestos damaged pulmonary alveolar macrophages in various degrees because of the different OH- levels. All the six fibrous mineral dusts changed the cellular electrophoresis ratio. CONCLUSION: The surface electromotive voltage of fibrous mineral dusts is not an important factor, and the cytotoxicity of them may be related to OH- levels on the mineral dust surface.

Objective To investigate the clinical features of patients with microscopic polyangiitis (MPA) and alveolar hemorrhage (AH),so as to raise the physicians' understanding of this condition.Methods The clinical data of 15 MPA patients combined with AH,who were hospitalized in Peking Union Medical College Hospital from January 2008 to January 2014,were retrospectively analyzed.Results These 15 subjects accounted for 18.1％ of all MPA patients,including 4 males and 11 females with mean age of (59.4 ± 17.9) years old and mean disease duration of 7.5 (1-48) months.Hemoptysis or bloody sputum (14/15) and dyspnea (11/15) were predominant pulmonary manifestations.Hypoxemia appeared in 6 patients (40.0％).An average 8-46 (22.7 ± 4.4) g/L drop of hemoglobin level was seen in all 15 patients.Imaging revealed new bilateral infiltrations or opacities in 13 cases,and bloody bronchoalveolar lavage fluid were found in 6 cases.Compared with MPA patients without AH,the incidence of myalgia [2/15 vs 50.0％ (34/68) ; P =0.009,OR =0.154] and hemoglobin level [(83.2 ± 21.0) g/L vs (102.5 ± 18.7) g/L; P =0.001] were statistically lower in MPA patients with AH.There were no statistical differences in age,sex,interstitial lung disease,renal involvement,peripheral neuropathy and the five factor score between two groups.The proportion of patients who received corticosteroid pulse treatment in MPA with AH group was significantly higher than that of MPA patients without AH [9/15 vs 19.1％ (13/68) ; P =0.003,OR =6.346].After treatment,13 cases achieved remission,while 2 died.Conclusion As an important pulmonary manifestation of MPA,AH could be insidious but fatal.The treatment with steroid,cyclophosphamide and plasma exchange could improve the prognosis.

Objective To investigate the changes of major causes of death in patient with dermatomyositis/polymyositis (DM/PM) and the factors affecting the mortality of DM/PM in the past 28 years.Methods Death cases with DM/PM from 1985 to 2013 were retrospectively analyzed.x2 test and Fisher exact test was used for count data analysis;independent samples t-test measurement was used for data analysis;P＜0.05 was consi-dered statistically significant.Results Out of 1 443 patients with DM/PM, 74 died, in whom 48 were female and 26 were male.The mortality rate was 4.7％ among female and 6.3％ among male cases and the total in-hospital mortality of DM/PM was 5.1％.The two most common causes of death in patients with DM/PM were infection and respiratory involvement, accounting for 62.2％ (46/74) and 14.9％ (11/74) respectively.Death occurred most commonly in the first year after disease onset, accounting for 79.7％(59/74).The leading causes of death in the first year were infection and respiratory involvement.The mortality of DM/PM patients was peaked 12.3％(18/146) from 1996 to 2000;infection was the most frequent cause of death.Conclusion Infection and respiratory involvement are the main causes of death in DM/PM.Death occurrs most commonly in the first year after disease onset.