Background::Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease characterized by complex and various clinical manifestations. The study aimed to analyze clinical features and cerebral magnetic resonance imaging (MRI) changes of hyperintense white matter (WM) lesions in SLE patients.Methods::This was a retrospective study based on a consecutive cohort of 1191 SLE patients; 273 patients for whom cerebral MRI data were available were enrolled to assess hyperintense WM lesions associated with SLE. Patients were assigned to two groups, ie, with or without hyperintense WM lesions. The MRI assessment showed that the hyperintense WM lesions could be classified into three categories: type A, periventricular hyperintense WM lesions; type B, subcortical hyperintense WM lesions; and type C, multiple discrete hyperintense WM lesions. The clinical and MRI characteristics were analyzed. Factors related to hyperintense WM lesions were identified by multivariate logistic regression analysis.Results::Among the 273 SLE patients with available cerebral MRI scans, 35.9% (98/273) had hyperintense WM lesions associated with SLE. The proportions of types A, B, and C were 54.1% (53/98), 11.2% (11/98), and 92.9% (91/98), respectively. Fifty-one percents of the patients showed an overlap of two or three types. Type C was the most common subgroup to be combined with other types. Compared with those without hyperintense WM lesions, the patients with hyperintense WM lesions were associated with neuropsychiatric SLE (NPSLE), lupus nephritis (LN), hypertension, and hyperuricemia ( P = 0.002, P = 0.018, P = 0.045, and P = 0.036, respectively). Significantly higher rates of polyserous effusions and cardiac involvement were found in the patients with hyperintense WM lesions ( P = 0.029 and P = 0.027, respectively), and these patients were more likely to present with disease damage ( P < 0.001). In addition, the patients with hyperintense WM lesions exhibited a higher frequency of proteinuria ( P = 0.009) and higher levels of CD8 + T cells ( P = 0.005). In the multivariate logistic analysis, hyperuricemia and higher CD8 + T cells percentages were significantly correlated with hyperintense WM lesions in SLE patients ( P= 0.019; OR 2.129, 95% confidence interval [CI] 1.313-4.006 and P < 0.001; OR 1.056, 95% CI 1.023-1.098, respectively). Conclusions::Hyperintense WM lesions are common in SLE patients and significantly associated with systemic involvement, including NPSLE, LN, polyserous effusions, cardiac involvement, and disease damage. Hyperuricemia and a higher number of CD8 + T cells were independent factors associated with hyperintense WM lesions in SLE.

Objective:The investigation and research on the application status of Hepatic Venous Pressure Gradient (HVPG) is very important to understand the real situation and future development of this technology in China.Methods:This study comprehensively investigated the basic situation of HVPG technology in China, including hospital distribution, hospital level, annual number of cases, catheters used, average cost, indications and existing problems.Results:According to the survey, there were 70 hospitals in China carrying out HVPG technology in 2021, distributed in 28 provinces (autonomous regions and municipalities directly under the central Government). A total of 4 398 cases of HVPG were performed in all the surveyed hospitals in 2021, of which 2 291 cases (52.1%) were tested by HVPG alone. The average cost of HVPG detection was (5 617.2±2 079.4) yuan. 96.3% of the teams completed HVPG detection with balloon method, and most of the teams used thrombectomy balloon catheter (80.3%).Conclusion:Through this investigation, the status of domestic clinical application of HVPG has been clarified, and it has been confirmed that many domestic medical institutions have mastered this technology, but it still needs to continue to promote and popularize HVPG technology in the future.

Objective:To investigate the role of conjunctival impression cytology in the diagnosis of Sj?gren's syndrome (SS) and the immunological factors influencing conjunctival lesions.Methods:A total of 57 patients complaining about dry eye were collected, including 38 patients with primary Sj?gren's syndrome (pSS) and 19 patients with non-SS. Conjunctival impression cytology tests were performed for all patients, and they were scored by the Nelson method. Thirty-one patients with SS underwent serological tests such as autoantibodies, immunoglobulins, and complement. The correlation between the relevant data was compared using the t test and the rank sum test. Results:The Nelson grade ≥2 is the positive cut-off value for the diagnosis of SS. The sensitivity was 68.4%, and the specificity was 89.5%, and the area under the receiver operating characteristic curve (ROC) was 0.767. In patients with SS, there was statistical correlation between the results of conjunctival impression cytology and antinuclear antibody (ANA) ( χ2=4.664, P=0.031), anti-SSA antibody ( χ2=8.58, P<0.01), anti-SSB antibody ( χ2=6.13, P=0.013), anti-SSA-52 antibody ( χ2=6.48, P=0.011), immunoglobulin (Ig)G ( t=-4.344, P<0.01) and rheumatoid factor (RF) ( U=25.0, P<0.01). Conclusion:Con-junctival impression cytology has certain value in the diagnosis of SS and can be used to evaluate conjunctival lesions in SS. Serum ANA, anti-SSA antibody, anti-SSB antibody, anti-SSA-52 antibody, IgG, and RF levels are significantly associated with the degree of conjunctival lesions, and can be considered as an indirect evidence of conjunctival involvement in SS.

Objective To investigate the causes of misdiagnosis related to rheumatism diseasecases.Methods A search was performed in database WanFang to identify the misdiagnosed clinical cases reports which were published in Chinese Journal and a retrospective analysis was conducted.All data were analyzed by chi-square test and Fisher's exact test.Results We screened 705 citations and identified 215 articles on the rheumatic diseases,finally,187 reports and 195 cases in total with definite diagnosis were included,accounting for 25.5％ of the total number of misdiagnosed cases.Primary vasculitis (72 cases),rheumatoid arthritis (25 cases),spondyloarthropathy (17 cases),polymyositis (14 cases),systemic lupus erythematosus (13 cases) and Sj(o)gren's syndrome (SS) (12 cases) were amongst the top misdiagnosed rheumatic diseases.There was no difference between 1998-2006 and 2007-2015 in the overall misdiagnosis cases.Single disease comparison,polymyositis,spondyloarthropathy and IgG4 related disease were increased (P=0.002;P=0.034;P=0.060;respectively),while polymyositis was delayed (P=0.002).Rheumatism misdiagnosed cases reported mainly came from tertiary hospitals and the department of rheumatology.Conclusion Misdiagnosed rheumatism are common in clinic.Strengthen the physicians' continuous education,the validity of clinical thinking mode and rational use of diagnostic criteria are important to make correct diagnosis.

Objective To investigate the common initial clinical presentations of primary Sj(o)gren's syndrome (pSS) with pulmonary complications,and to explore the differences between patients with extraglandular manifestations at disease onset (EGM) and those with glandular manifestations at disease onset (GM).Methods A total of 1 341 hospitalized SS patients from 2003 to 2012 were retrospectively reviewed.Of them,102 hospitalized patients with pSS'associated lung disease were analyzed and included.Case control study was performed to explore the differences between the EGM group and the GM group.Results Fifty-one percent of patients were presented with EGM at onset,with significantly shorter disease duration [36 (12,156) m vs 102 (48,159) m,x2=-2.41,P=0.016].Although the mean diagnose time was similiar,only 4％ of the EGM group could be confirmed the pSS diagnose at onset,which was significantly less frequently than that of the GM group (34％,22=15.29,P＜0.01).Case control study revealed that hyperglobulinemia,elevated RF titers and anti-SSA and/or anti-SSB test positive were less predominant in the EGM group [IgG 16(12,21) g/L vs 21 (15,28) g/L,x2=-2.15,P=0.032;22 (20,171) U/ml vs 104 (20,238) U/ml,x2=-l.98,P=0.048;33％ vs 72％,x2=15.78,P＜0.01].The predicted value of TLC and FVC were lower [(87±23)％ vs (97±20)％,x2=-1.96,P=0.050;(8±28)％ vs (100±27)％,x2=-1.70,P=0.089] and HRCT score was higher in EMG group [12(88,15) vs 8(5,13),x2=-1.82,P=0.070].Conclusion EMG at onset is the common initial manifestation of pSS'associated lung involvement.Pulmonary complication is more progressively and severe than those with MG at onset.Anti'SSA positive,elevated RF titer and hyperglobulinemia are not predominant for patients with EMG at onset.

Objective To evaluate the clinical value of Chinese Multi-Dimensional Health Assessment Questionnaire (MDHAQ-C) in patients with rheumatoid arthritis (RA). Methods From December 2015 to October 2016,four hundred and twelve RA patients were recruited in this study and completed the MDHAQ-C independently and routine assessment of patient index data 3 (RAPID3) was calculated. Then correlations were assessed by Spearman correlation coefficient of RAPID3 with indices of clinical relevance and disease activity to evaluate its clinical values. Results MDHAQ-C is moderately correlated with ESR (r=0.399), CRP (r=0.381), SJC (r=0.499), TJC (r=0.558) (P<0.01), but highly correlation with VAS of pain (r=0.836), VAS of global status (r=0.915) and overall assessment of physician (r=0.784)(P<0.01), and highly correlated with a Spearman's coefficient of 0.784 for DAS 28-ESR, 0.734 for CDAI and 0.682 for SDAI (P<0.001). Conclusion MDHAQ is a reliable, valid instrument for disease activity assessment and a feasible index for clinical settings in Chinese RA patients.

Objective:To compare the therapeutic efficacy and the recurrence rate between the simple repositioning method and the reposition manoeuvre plus medication in the treatment of horizontal semicircular canal otolith benign paroxysmal positional vertigo (HSC-BPPV). Method:Sixty-two patients diagnosed with otolith HSC-BPPV by roll maneuver test were randomly divided into canalith repositioning group (32 patients) and reposition plus drug treatment group (30 patients). Patients in the canalith repositioning group were treated only with Barbecue reposition maneuver; patients in the reposition plus drug treatment group were treated firstly with Barbecue reposition maneuver and then were given Alprostadil, Cinepazide and Betahistine drug treatment. Both groups were evaluated after 7 days and 28 days treatment, and the recurrence rate was analyzed after 3 months. Result:After 7 days of the treatment , the recovery rate of the two groups was 62.5% and 73.3%, respectively. There's no significant difference between the two groups. However, the total effective power of the reposition plus drug treatment group was 96.7%, which was significantly higher than that of the canalith repositioning group (75.0%) (χ²=5.858, P<0.05). There were 8 patients in the canalith repositioning group showed changes of BBPV types after treatment, while only 1 patient in the reposition plus drug treatment group showed lesion changes. The difference was statistically significant (χ²=4.061, P<0.05). After 28 days of the treatment, the recovery rate and the total effective power of the two groups was 100%, respectively. There is no statistical difference in the total effective rate between the two groups. After 3 months follow-up, 2 patients in the canalith repositioning group (6.25%) and in the reposition plus drug treatment group (6.67%) showed BBPV recurrence, and no significant difference in the recurrence rate was found between the two groups (P>0.05). Conclusion:The repositioning maneuver is the preferred method for treating HSC BPPV. Canalith reposition maneuver plus medication has no obvious effect on the recovery rate and the recurrence rate, it only increases the effective rate and reduces the changes of the BBPV types.

Objective:To report a rare type of aortic arch aneurysm. Methods: Three cases of aortic arch aneurysm derived from the fourth aortic arch were retrospectively analyzed. The pathogenesis and treatment of this type of aortic arch aneurysm were investigated. Results:Most of the aneurysm body was located in the Z2 zone, which was the stem from the fourth aortic arch in the embryonic development period. All of the 3 cases could not be explained by common etiology. We speculated that the cause might be developmental anomaly of the fourth aortic arch. All the 3 aortic arch aneurysms were totally ex-cluded with a covered stent. The technical success rate was 100%. Endoleak of typeⅠwas seen in one case, which was resolved in a later open surgery. During the follow-up, no type of complications was found. Conclusion:To the best of our knowledge, this is the first report of this type of aortic arch aneurysm. The cause may be developmental anomaly of the fourth aortic arch. Endovascular treatment of this type of aortic arch aneurysm is feasible.

SUMMARY An involved internal iliac artery is usually embolized when performing endovascular aneu -rysm repair for aortoiliac or isolated iliac artery aneurysm .This can lead to complications such as buttock claudication ,colon ischaemia and erectile dysfunction .Iliac branch device ( IBD ) is an endograft de-signed specifically for iliac bifurcation to preserve internal iliac flow .It was performed with high technical success rates and encouraging mid-term patency .Here we report a case of right iliac aneurysm developed 3 years after endovascular aneurysm repair for an aortoiliac aneurysm , with the patient ’ s left internal ar-tery been sacrificed then .Using a handmade IBD , we excluded the aneurysm without occlusion of the ip-silateral internal iliac artery or any type of endoleak .Both the design and deployment of this IBD are dis-tinctive that we would like to share our experience with all the colleagues .

Objective To evaluate iliac artery rupture during endovascular treatment of aorto-iliac disease and treatment with covered stent.Methods From November 2008 to August 2014,iliac artery rupture happened in 8 patients of 225 patients of aorto-iliac artery disease admitted to our hospital.Clinical data of the 8 patients were analyzed retrospectively.Four were of intraoperative rupture and four were of delayed rupture.The aorto-iliac diease of all the 8 patients were TSAC D.Direct placement of a covered stent was performed in all the cases,except for one patient who refused further treatment.Results All of the procedures finally achieved technical success and all patients become hemodynamically stable.One patient was complicated with heart failure who died of multiple organ dysfunction syndrome.Another one died of aspiration.5 patients were followed up from 9-52 moths,average 9-52 moths without recurrence.Conclusions Iliac artery rupture is a serious and life-threatening complication in the endovascular treatment of aorto-iliac disease.Covered stent provided a safe and effective treatment for iliac perforations.