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Wang R.,Peking University | Wang R.,Beijing Key Laboratory of Molecular Diagnosis of Dermatoses | Li J.,Beijing Jishuitan Hospital | Wang M.,Peking University | And 8 more authors.
British Journal of Dermatology | Year: 2015

Background Paraneoplastic pemphigus (PNP) involves multiple organs, but little is known about its neurological involvement. Objectives To investigate the symptoms, prognosis and profiles of associated autoantibodies in myasthenia gravis (MG), and their correlations in patients with PNP. Methods Fifty-eight patients with PNP were assessed for myasthenic symptoms and laboratory evidence. Serum autoantibodies against acetylcholine receptor (AChR), acetylcholinesterase (AChE), titin, ryanodine receptor (RyR) and muscle-specific kinase (MuSK) were measured by enzyme-linked immunosorbent assay. Patients with pemphigus vulgaris (PV), pemphigus foliaceus (PF), connective tissue disease (CTD) and non-PNP MG (NP-MG), and healthy donors, served as controls. These autoantibodies in PNP were also compared in the presence or absence of dyspnoea or muscle weakness. Cox regression and log-rank tests were used for survival analysis. Results Overall 39% of patients with PNP experienced muscle weakness, and 35% were diagnosed with MG. Moreover, 35% had positive anti-AChR and 28% had anti-AChE antibodies, similarly to NP-MG (33% and 17%, respectively, P > 0·05). However, both were negative in all patients with PV, PF and CTD and healthy donors (P < 0·005). No other antibodies showed significant differences among groups. Anti-AChR and anti-AChE antibody levels were significantly increased in patients with PNP with dyspnoea, while anti-AChR, anti-titin and anti-RyR were significantly increased in patients with PNP with muscle weakness (P < 0·05). Nevertheless, levels and positive rates of these autoantibodies showed no significant differences between PNP with Castleman disease and thymoma. Although anti-AChE levels impacted survival duration (P- = -0·027, odds ratio 3·14), MG complications did not affect the overall survival percentage in PNP. Conclusions MG is a complication of PNP. Anti-AChR and anti-AChE antibodies are prominent in patients with PNP, especially those with dyspnoea. What's already known about this topic? Paraneoplastic pemphigus is a life-threatening multiorgan autoimmune syndrome. Myasthenia gravis is an autoimmune neurological disorder occasionally associated with various autoimmune diseases. What does this study add? This is the first detailed demonstration of a clear association between myasthenia gravis and paraneoplastic pemphigus. Antiacetylcholine receptor and antiacetylcholinesterase antibodies are prominent in patients with paraneoplastic pemphigus, especially those with dyspnoea. © 2014 British Association of Dermatologists. Source


Wang H.,Peking University | Wang H.,Beijing Key Laboratory of Molecular Diagnosis of Dermatoses | Wan Z.,Peking University | Wan Z.,Beijing Key Laboratory of Molecular Diagnosis of Dermatoses | And 4 more authors.
BioMed Research International | Year: 2015

As various new sibling species within the Scedosporium spp. have been described recently, this study was conducted to investigate distribution and antifungal susceptibility profiles of the different species of Scedosporium spp. in China. Twenty-one clinical strains of Scedosporium from China and two strains from Japan were reidentified by MLSA. The analysis included BT2, CAL, RPB, SOD, and ACT and the combination of the five loci. Pseudallescheria boydii complex (17 strains) and S. apiospermum (6 strains) were identified. P. boydii complex included four closely related subgroups: P. boydii (9 strains), P. ellipsoidea (6 strains), P. fusoidea (1 strain), and P. angusta (1 strain). There were no significant differences in MICs for neither VOR, POS, nor AMB over all the five species in study. For itraconazole, intraspecific diversity was evident. © 2015 Hong Wang et al. Source


Wang M.,Peking University | Wang M.,Beijing Key Laboratory of Molecular Diagnosis of Dermatoses | Gao Y.,Peking University | Gao Y.,Binzhou Medical University | And 8 more authors.
Journal of Dermatology | Year: 2016

Glucocorticoids are the first-line treatment for pemphigus vulgaris. Among 140 patients receiving systemic glucocorticoids, 124 patients achieved complete remission off or on a prednisone dose of ≤10 mg/day or less for 6 months or more. The mean average steroid controlling doses were 0.65, 0.62, 0.80, 1.08 and 1.38 mg/kg per day for the mucosal-dominant patients and the mild, moderate, severe and extensive cutaneous-involved patients, respectively (P < 0.001). The mean durations of the initial tapering after controlling doses started were 77.98, 48.78, 31.74 and 28.83 days when the disease was controlled with doses of 40 mg/day or less, 45-60 mg/day, 65-80 mg/day and more than 80 mg/day for the cutaneous-involved types, respectively (P < 0.005). Of the patients, 79.51% achieved complete remission within 3 years, 98.36% within 5 years and all within 6 years, which corresponded to a 50% yearly reduction of glucocorticoid dose. These successfully treated patients indicate that a severity-tailored initial dose of glucocorticoids, an initial tapering duration based on the initial dose and a subsequent 50% yearly tapering regimen may cure pemphigus vulgaris within 3-6 years. © 2015 Japanese Dermatological Association. Source


Zhang S.,Peking University | Zhang S.,Beijing Key Laboratory of Molecular Diagnosis of Dermatoses | Wang S.,Peking University | Wang S.,Beijing Key Laboratory of Molecular Diagnosis of Dermatoses | And 7 more authors.
Mycopathologia | Year: 2016

The diagnosis of invasive pulmonary aspergillosis (IPA) is still in challenge in clinical practice, particularly for those patients without an obvious neutropaenia. In this study, a well-validated qPCR method and Platelia galactomannan (GM) assay were compared for their diagnostic performance using paired samples of bronchoalveolar lavage (BAL) fluid and serum from predominantly non-neutropaenic patients. In the serum samples, qPCR showed a comparable performance with GM assay in terms of sensitivity and specificity. In the BAL samples, qPCR and GM assay both demonstrated a good sensitivity (90 vs. 90 %); however, the specificity of qPCR was higher than that of GM assay (92.5 vs. 68.8 %, P < 0.001) in these samples. A better sensitivity was obtained with BAL compared with serum samples for both GM assay (90 vs. 50 %) and qPCR (90 vs. 60 %). In conclusion, in non-neutropaenic patients, BAL appears to provide improved sensitivity for both GM and qPCR assays. BAL qPCR offers a better diagnostic value for IPA compared with BAL GM assay, significantly increasing the specificity without affecting the sensitivity. © 2016 Springer Science+Business Media Dordrecht Source

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