Middle lobe syndrome as the pulmonary manifestation of primary Sjögren's syndrome

Middle lobe syndrome - recurrent atelectasis and/or bronchiectasis involving the right middle lobe and/or lingula - has, up to now, not been reported as the pulmonary manifestation of primary Sj?gren's syndrome. We describe a patient in whom lymphocytic bronchiolitis in the atelectatic lobes was proved histologically from two separate transbronchial biopsies. The atelectasis responded well to glucocorticoid treatment, suggesting that the peribronchiolar lymphocytic infiltrates may have played an important role in the development of middle lobe syndrome in this patient.     

Increased serum CA 19-9 antibodies in Sjögren's syndrome

A 67-year-old woman with a history of thyroiditis presented with recent intermittent epigastric pain and nausea. Hyperamylasaemia, oedema of the pancreas, and high serum levels of lipase and CA 19-9 were found. Xerostomia and dry eyes developed later, accompanied by an abnormal Schirmer''s test. The diagnosis of Sjögren''s syndrome was confirmed by increased anti-Ro and anti-La antibodies and the histological findings of parotid gland biopsy. Two additional cases of Sjögren''s syndrome with elevated serum CA 19-9 are also described. These observations of elevated serum lipase and serum CA 19-9 in Sjögren''s syndrome without evidence of malignancy may reflect pancreatic involvement in this disorder.     

Eight-year follow-up of airway hyperresponsiveness in patients with primary Sjögren’s syndrome

Objective: To evaluate in a longitudinal study the influence of airway hyperresponsiveness (AHR) on lung function in patients with primary Sjögren’s syndrome (pSS).

Methods: Lung function was studied over an eight-year period in 15 patients who fulfilled the Copenhagen criteria for primary Sjögren’s syndrome and who were covered in our earlier published study on AHR in patients with Sjögren’s syndrome. Standard spirometry and measurements of lung volumes, diffusing capacity (DLCO), and AHR to methacholine were performed.

Results: A significant decline over time was found in total lung capacity (TLC), vital capacity (VC), forced vital capacity (FVC), functional residual capacity (FRC), and expiratory midflows (FEF₅₀). A sign of small airway obstruction (decrease in FEF₅₀) at entry correlated with VC at follow-up (r?=?.8,?P?<?.003), and the individual change in FEF₅₀ during the observation period correlated with the individual change in VC (r?=?.6, P?<?.05). Six patients had increased AHR, and three of them had decreased DLCO. Six of the patients progressively reduced DLCO over time, and five of them had spirometric signs of increased small airway obstruction.

Conclusions: During this eight-year follow-up we observed that one-third of the patients with pSS developed a significant reduction in lung function. Our findings suggest that small airways obstruction and AHR are associated with reduction of VC and development of impaired DLCO as a sign of interstitial lung disease in this group of patients.     

Clinical manifestations and immunological features of primary Sj?gren''s syndrome with liver involvement: analysis of thirty cases.

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Correlation between retinal fluorescein angiography and blood viscosity and other factors in patients with primary open angle glaucoma.

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Sex, smell and vasculitis: Henoch-Schönlein purpura in Kallmann's syndrome

Kallmann's syndrome (KS) is the most common cause of isolated hypogonadotropic hypogonadism. An increased prevalence of rheumatic and autoimmune diseases has been noted in patients with hypogonadism including Kallmann's syndrome. Both in vitro and in vivo studies indicate that testosterone deficiency may promote inflammatory response such as vasculitis by altering inmmunoglobulin and cytokines profiles. We report the novel occurrence of Henoch-Sch?nlein purpura in a patient with markedly low testosterone level due to Kallmann's syndrome. We discuss the potential mechanisms by which hypoandrogenism may lead to vasculitis.     

Traditional Chinese medicine compound ShengJinRunZaoYangXue granules for treatment of primary Sjögren’s syndrome: a randomized, double-blind, placebo-controlled clinical trial

Background Traditional Chinese medical treatment of primary Sj（o）gren＇s syndrome has advantages over Western medicine in terms of fewer side effects and improved patient conditions.This study was a multicenter,randomized,doubleblind,placebo-controlled clinical trial of the efficacy and safety of ShengJinRunZaoYangXue granules for the treatment of primary Sj（o）gren＇s syndrome,including the symptoms of dry mouth and dry eye.Methods We undertook a 6-week,double-blind,randomized trial involving 240 patients with primary Sj（o）gren＇s syndrome at five centers in East China.A computer-generated randomization schedule assigned patients at a 2∶1 ratio to receive either ShengJinRunZaoYangXue granules or placebo once daily.Patients and investigators were blinded to treatment allocation.The primary endpoints were the salivary flow rate,Schirmer test results,and sugar test results.Intention-to-treat and per-protocol analyses were performed.Results All 240 patients were randomly allocated to either the treatment group （n=160,ShengJinRunZaoYangXue granules） or placebo group （n=80） and were included in the intention-to-treat analysis.After program violation and loss to follow-up,a total of 199 patients were included in the per-protocol analysis.At six week,intention-to-treat and per-protocol analyses of the left-eye Schirmer I test results showed an improved difference of 1.36 mm/5 min （95% CI：0.03 to 2.69 mm/5 min） and 1.35 mm/5 min （95% CI：0.04 to 2.73 mm/5 min）,respectively,and those of the right-eye Schirmer I test results showed an improved difference of 1.12 mm/5 min （95% CI：0.02 to 2.22 mm/5 min） and 1.12 mm/5 min （95% CI：-0.02 to 2.27 mm/5 min）,respectively.There was no significant difference between the two groups before treatment.After treatment,the between-group and within-group before-and-after paired comparison results were statistically significant （P 〈0.05）.Intention-to-treat and per-protocol analyses showed an improved salivary flow rate by 0.04 ml/15 min ?     

Effect of exercise on left and right ventricular ejection fraction and wall motion in patients with coronary artery disease: an ultrafast computed tomography study

Theresponsesofleftventricularejectionfraction(LVEF)andrightventricularejectionfraction(RVEF)toexercise-inducedleftventricularregionalwallmotionabnormalities(LVWMA)andrightventricularregionalwallmotionabnormalities(R~A)areusefulindieatorsforthediagnosisofcoronaryarterydisease(CAD)inpatientswithchestpain.Thishadbeendemonstratedinpriorstudiesusingexerciseradionuclide,echocwhioguphicandultrafastcomputedtomography(UFCT)icingtech."I-7niqUes'.Variablesensitivityandspecificityinassessingbive…     

Relationship between Trp64Arg mutation in the β3-adrenergic receptor gene and metabolic syndrome: a seven-year follow-up study

Background It has been shown that the β3-adrenergic receptor （β3-AR） gene Trp64Arg mutation was closely related to obesity and insulin resistance, and may be related to the prevalence of metabolic syndrome （MS）. The aim of this study was to investigate the relationship between the 33-AR gene mutation and the prevalence of MS. Methods A seven-year follow-up study was initiated in 2000, with 496 samples of simplex obese subjects （body mass index ≥25 kg/m2） and 248 normal-weight subjects. According to the β3-AR genotypes, the subjects were classified as Trp64 homozygote group and Arg64 carrier group and after 7 years the prevalence of MS was determined. Results According to the baseline profile, there were no significant differences in the adiposity, blood pressure, lipid profile, fasting plasma glucose and fasting insulin between Trp64 homozygote group and Arg64 carrier group either in obesity or normal-weight subjects. The results of follow-up study indicated that in obese men the prevalence rate of MS was much higher in Arg64 carrier group than that in Trp64 homozygote group （54.76% vs. 40.85%, P 〈0.05）, but there was no statistical difference in women of the above groups. The prevalence rate of MS in obese men of both Trp64 homozygote group and Arg64 carrier obese group were obviously higher than that in women of the above groups （40.85% vs. 18.27% and 54.76% vs 21.28%, all P 〈0.005）. Differences were not statistically significant in the prevalence of MS for normal weight Trp64 homozygote group and normal weight Arg64 carrier group, either between men, between women, or between men and women. Comparison of populations indicated that no matter with the β3-AR gene mutation or not, the prevalence of MS in obese subjects was significantly higher than normal weight subjects （X2=28.240 and x2=15.586, all P 〈0.005）. Logistic analysis showed that the mutation of β3-AR gene was associated with the prevalence of MS in men.     

Factors and cytokine signatures associated with the response to therapy in patients with primary biliary cholangitis-autoimmune hepatitis overlap syndrome: a real-world study

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