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Objective:To investigate the clinical characteristics and treatment of Beh?et′s disease complicated with cardiac valve involvement.Methods:We searched the wanfang medical database and Medline database to reviewed the domestic and foreign literature reports on cardiac Beh?et′s disease and analyzed their clinical features and therapeutic strategies. Chi-squared test was used for data analysis.Results:It was shown that Beh?et′s disease with cardiac valve involvement mainly affect men. The male to female ratio was 3.86∶1 in China and 2.50∶1 in foreign patients( χ2=1.32, P=0.251). The preoperative diagnosis rate was not high(60.3% in China, 57.1% abroad) ( χ2=0.13, P=0.716). Aortic valve and perivalvular lesions were the most common involved sites, of which aortic regurgitation was the most frequenty occurred, followed by mitral valve lesions. Glucocorticoids was still the main means treatment for medical(93/235 in China, 28/420 abroad), cyclophosphamide was more widely used in China(28/235), azathioprine was more widely used in foreign countries (12/42). Aortic replacement (AVR) was the mainly surgical approach, followed by artificial aortic valve replacement and left ventricular outflow tract plasty (Bentall).The incidence of postoperative perivalvular leakage or valve prolapse was higher with AVR than with Bentall(AVR 76.3%/Bentall 21.8% at home, χ2=32.60, P<0.001, AVR 71.4%/Bentall 0 abroad, χ2=13.84, P<0.001). Conclusions:Cardiac valve involvement is a severe complication of Beh?et′s disease. Heart involvement are more common, and the preoperative diagnosis rate is lower in China. The incidence of perivalve leakage (PVL) or valve prolapse (PD) after operation is higher with AVR than with Bentall surgery.The Bentall operation could improve prognosis and the postoperative complications abroad are lower than domestic.
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Objective:To improve the diagnosis of anti-synthetase antibody syndrome(ASS) by analyzing the clinical features of 6 patients.Methods:Six cases of ASS with complete data were included in this study as they were diagnosed or other CTD during the period of hospitalization in Wuxi People's Hospital from January 2016 to February 2020. Their clinical and laboratory characteristics, and follow-up information were analyzed. Features and changes in the course of disease were analyzed.Results:Four out of 6 patients were females, with age of disease onset as 34-72 years, and an interval of 4-59 months from the first diagnosis to the diagnosis of ASS. The first diagnosis was Sjogren's syndrome (SS) in 2 cases, rheumatoid arthritis (RA) in 1 case, mixed connective tissue disease (MCTD) in 1 case, systemic sclerosis (SSc) in 1 case, and undifferentiated connective tissue disease (UCTD) in 1 case. At the first diagnosis, 5 cases had dry cough and/or dyspnea, followed by fever (4 cases), arthritis and Raynaud's phenomenon (3 cases). Anti-nuclear antibody(ANA), which was more common in cytoplasmic type, and anti-SSA/52 000 antibody were mostly positive(5 cases). The presence of non-specific interstitial pneumonia (NSIP) pattern (6 cases) in high resolution CT(HRCT) was found at the initial diagnosis. During follow-up, patients developed repeated liver function or muscle enzyme abnor-malities (3 cases), mechanic's hand (MH) (3 cases), and lung interstitial disease progression (4 cases). The myositis antibodies were found to be positive.Conclusion:ASS can occur in the course of or at the same time as other CTDs. ASS should be considered in patients with interstitiallung disease (ILD) (especially NSIP pattern) in HRCT, and/or positive of cytoplasmic type ANA and/or anti-SSA/52 000 antibodies At the same time, if repeated liver function or muscle enzyme abnormalities, new onset of MH, and lung interstitial disease progresses during treatment, consideration may be given to the possibility of complicated ASS. Myositis-specific antibodies are helpful in the diagnosis of ASS.
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Objective To investigate the level of IL-17 and its clinical significance in patients with primary Sj(o)gren's syndrome (pSS) complicated with interstitial lung disease (ILD).Methods IL-17 levels were detected by enzyme linked immunosorbent assay (ELISA) in 53 untreated patients with pSS (25patients with only lacriminal and/or salivary gland involvement,28 with only interstitial lung disease involvement) and 15 healthy controls.The related clinical and laboratory data were recorded.ANOVA,LSDtest and Pearson test were used for statistical analysis.Results There were significant differences between the 3 groups(F=22.504,P=0.000).The mean concentration of sera IL-I7 in the patients with ILD was significantly higher than in patients with only lacriminal or salivary gland involvement (P＜0.05).There was no difference in the levels of sera IL-17 between the patients with only lacriminal and/or salivary gland involvement and healthy controls (P＞0.05).Conclusion Our results show that patients with pSS concomitant with ILD have high serum IL-17 levels,which highlight the role of IL-17 in Sj(o)gren's syndrome interstitial lung injury.