Background and purpose
The differences in the characteristics of thymus histology, coexisting autoimmune diseases and related autoantibodies between anti‐muscle‐specific receptor tyrosine kinase ...(MuSK)‐antibody (Ab)‐positive myasthenia gravis (MG) patients, and anti‐acetylcholine receptor (AChR)‐Ab‐positive MG patients are not clearly defined.
Methods
The types of thymus histology, coexisting autoimmune diseases and associated Abs in 83 MuSK‐Ab‐positive patients nationwide were investigated and were compared with those in AChR‐Ab‐positive patients followed at our institute (n = 83). As for the autoantibodies associated with thymoma, titin Abs were measured.
Results
Thymoma was not present in any of the MuSK‐Ab‐positive patients but presented in 21 patients (25.3%) amongst the AChR‐Ab‐positive patients. Titin Abs were absent in MuSK‐Ab‐positive patients but positive in 25 (30.1%) of the AChR‐Ab‐positive patients. Concomitant autoimmune diseases were present in eight MuSK‐Ab‐positive patients (9.6%) amongst whom Hashimoto's thyroiditis and rheumatoid arthritis predominated, whereas 22 AChR‐Ab‐positive patients (26.5%) had one or more concomitant autoimmune diseases of which Graves' disease predominated.
Conclusions
Differences in frequency of thymoma and thymic hyperplasia, coexisting autoimmune diseases and autoantibody positivity between MuSK‐Ab‐positive and AChR‐Ab‐positive MG were indicated, suggesting that, in contrast with AChR‐Ab‐positive MG, thymus does not seem to be involved in the pathogenic mechanisms of MuSK‐Ab‐positive MG.
The aim of the study was to assess the safety and efficacy of everolimus therapy for advanced renal cell carcinoma in Japanese patients receiving real-world care.
Patients who had been treated with ...everolimus for advanced renal cell carcinoma at 39 Japanese medical centers between January 2010 and November 2011 were retrospectively investigated to assess adverse events and the time to treatment failure.
A total of 180 patients were identified. Their median age was 65 years (range 23-93). The median time to treatment failure was 2.9 months (95% confidence interval 2.4-3.4). The median time to treatment failure was significantly longer in patients with dose modification (4.2 months; 95% confidence interval 3.4-5.0) than in patients without dose modification (1.7 months; 95% confidence interval 1.0-2.3; P < 0.01) after experiencing adverse events. Stomatitis (44%) was the most frequent adverse event, followed by thrombocytopenia (31%), anemia (22%), interstitial pneumonia (22%) and hyperglycemia (17%). Interstitial pneumonia was the most frequent cause of discontinuation in patients who discontinued everolimus due to intolerability regardless of the dose modification status. None of the patients with dose modification of everolimus discontinued everolimus due to thrombocytopenia or leukopenia.
The adverse event profile of everolimus may differ between Japanese and Caucasian patients. Dose modification of everolimus might be associated with longer treatment duration in patients with advanced renal cell carcinoma. Further studies are required to clarify this association. Interstitial pneumonia may be difficult to overcome by dose modification.
The authors characterized the clinical and immunologic features of 110 patients with Lambert-Eaton myasthenic syndrome (LEMS). Anti-P/Q-type voltage-gated calcium channels (VGCC) antibodies were ...detected in 85% of the patients (seropositive) but not in the rest (seronegative). Except for the indication that small cell lung carcinoma is less common in seronegative patients, no significant differences were found in the clinical characteristics of patients who had or did not have anti-P/Q-type VGCC antibodies. The results of passive transfer experiments suggest that seronegative LEMS is also an autoantibody-mediated disorder.
Heart tissues of patients with PD or incidental Lewy body (LB) disease (ILBD) were examined by light and electron microscopy. LBs and alpha-synuclein-positive neurites were identified in the hearts ...from 9 of 11 patients with PD and from 7 of 7 patients with ILBD. LBs were present in both tyrosine hydroxylase-positive and -negative nerve processes, which are nerves of extrinsic sympathetic and intrinsic origin, respectively. These findings provide histologic evidence that the postganglionic sympathetic and intrinsic neurons in the heart are involved in the PD disease process.
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