33 research outputs found

    Giantin is the major Golgi autoantigen in human anti-Golgi complex sera

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    Anti-Golgi complex antibodies (AGAs) are primarily associated with systemic lupus erythematosus and Sjögren's syndrome. Here we report on the immunoreactivity of AGAs against five Golgi autoantigens (giantin, golgin-245, golgin-160, golgin-95/GM130, and golgin-97) and provide data from epitope mapping on the most common Golgi autoantigen, namely giantin. A total of 80 human sera containing AGAs, as defined by indirect immunofluorescence on HEp-2 cells, were analyzed by ELISA using recombinant autoantigens and immunoprecipitation. The proportion of AGA sera that reacted with the five Golgi autoantigens was correlated with the molecular mass of the Golgi antigens. Autoantibodies to giantin, the largest Golgi autoantigen, were the predominant AGAs, being found in 50% of the AGA sera. Epitope mapping of giantin was performed using six recombinant fragments spanning the entire protein. Antigiantin-positive sera with low titer autoantibodies recognized epitopes in the carboxyl-terminal fragments that are proximal to the Golgi membrane, whereas higher titer sera exhibited strong reactivity to amino-terminal and central domains that are likely to extend from the Golgi membrane into the cytoplasm. Our working hypothesis is that aberrantly expressed Golgi complex autoantigens may be released into the immune system when cells undergo lysis. By virtue of a carboxyl-terminal transmembrane domain, giantin is likely to be more stably associated with the cytoplasmic face of the Golgi complex than are other golgins, which are peripheral proteins. The stable association of giantin with the putative released Golgi complex may contribute to its preferential autoantigenicity

    Associations between the distance covered in the incremental shuttle walk test and lung function and health status in patients with chronic obstructive pulmonary disease

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    Background: Field walk tests such as the incremental shuttle walk test (ISWT) are simple tests for assessing the degree of disability in individuals with chronic obstructive pulmonary disease (COPD). In the present study, the correlations between exercise performance in the ISWT, lung function, and health status were examined in patients with COPD of varying seventies. Methods: A retrospective examination of 277 COPD patients was performed using the ISWT and lung function tests along with assessment of health status using St. George's Respiratory Questionnaire (SGRQ). In addition, we assessed the correlations between the walking distance, lung function parameters, and SGRQ scores. Results: ISWT distances were poorly correlated with lung function parameters and SGRQ scores in mild COPD patients. In contrast, ISWT distances were significantly correlated with pulmonary function parameters, such as vital capacity (%predicted) and forced expiratory volume in one second, and SGRQ scores in moderate and severe COPD patients. Conclusions: The ISWT is more independent of health status and pulmonary function in patients with mild COPD compared to moderate or severe cases. Therefore, the exercise capacity of patients with mild COPD should be estimated by the ISWT. (C) 2016 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.ArticleRESPIRATORY INVESTIGATION.55(1):33-38(2017)journal articl

    A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity

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    Thrombotic thrombocytopenia purpura (TTP) caused by a deficiency in ADAMTS-13 activity is considered to involve a subset of thrombotic microangiopathy (TMA). Although concept of TTP is included under the umbrella of TMA, discrimination of TTP from TMA is occasionally difficult in an autoimmune disorder. Herein, we report a case with TTP associated with systemic lupus erythematosus (SLE). In this case, it was difficult to discriminate TTP from TMA and the measurement of ADAMTS-13 activity was useful for obtaining an accurate diagnosis. SLE patients having thrombocytopenia in complication with anemia should be considered a monitoring of ADAMTS-13 activity even though the patients lacked symptoms of TTP related to the microvascular coagulation

    Short Physical Performance Battery for cardiovascular disease inpatients : implications for critical factors and sarcopenia

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    We examined the relationship between Short Physical Performance Battery (SPPB) and clinical and laboratory factors and the effect of sarcopenia and sarcopenic obesity (SO) on clinical and laboratory factors for cardiovascular disease (CVD) inpatients. CVD male (n=318) and female (n=172) inpatients were recruited. A stepwise multiple-regression analysis was performed to predict total SPPB scores and assess clinical and laboratory factors (physical characteristics, functional and morphological assessments, etc.). Each test outcome were compared among sarcopenia, SO and non-sarcopenic groups. To predict total SPPB scores, the predicted handgrip, Controlling Nutritional Status score, % body fat, anterior mid-thigh muscle thickness, standing height and systolic blood pressure were calculated for males and anterior mid-thigh MTH, BMI, knee extension and fat mass were calculated for females. There were no differences in blood pressure, total SPPB scores and functional assessments between sarcopenia and SO groups for CVD male and female inpatients. In conclusion, the physical performance of CVD inpatients can be predicted by nutritional, functional, clinical and anthropometric variables, regardless the gender and the presence of sarcopenia. Furthermore, the presence of sarcopenia has a negative effect on the clinical and laboratory factors, but there is a difference in impact between sarcopenia and SO regardless the gender

    Blood Flow Restriction Increases the Neural Activation of the Knee Extensors During Very Low-Intensity Leg Extension Exercise in Cardiovascular Patients:A Pilot Study

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    Blood flow restriction (BFR) has the potential to augment muscle activation, which underlies strengthening and hypertrophic effects of exercise on skeletal muscle. We quantified the effects of BFR on muscle activation in the rectus femoris (RF), the vastus lateralis (VL), and the vastus medialis (VM) in concentric and eccentric contraction phases of low-intensity (10% and 20% of one repetition maximum) leg extension in seven cardiovascular patients who performed leg extension in four conditions: at 10% and 20% intensities with and without BFR. Each condition consisted of three sets of 30 trials with 30 s of rest between sets and 5 min of rest between conditions. Electromyographic activity (EMG) from RF, VL, and VM for 30 repetitions was divided into blocks of 10 trials and averaged for each block in each muscle. At 10% intensity, BFR increased EMG of all muscles across the three blocks in both concentric and eccentric contraction phases. At 20% intensity, EMG activity in response to BFR tended to not to increase further than what it was at 10% intensity. We concluded that very low 10% intensity exercise with BFR may maximize the benefits of BFR on muscle activation and minimize exercise burden on cardiovascular patients

    Synchronous malignant B-cell lymphoma and gastric tubular adenocarcinoma associated with paraneoplastic cutaneous vasculitis: hypereosinophilic syndrome with mixed cryoglobulinemia is an important sign of paraneoplastic syndrome

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    Gastric adenocarcinoma developing concomitantly with a lymphoma is rare. Furthermore, B-cell lymphoma, originating from lymph nodes, with eosinophilia is extremely rare. We report here a case with a synchronous diffuse large B-cell lymphoma (DLBCL) and an early adenocarcinoma of the stomach. In addition, this case seemed to be associated with paraneoplastic cutaneous vasculitis caused by hypereosinophilic syndrome (HES) with mixed cryoglobulinemia (MC). Many neoplastic diseases that affect internal organs display cutaneous manifestations, which may be the presenting signs and symptoms of the underlying malignancy. In particular, the association between cutaneous vasculitis and malignancy has been widely reviewed, and recently neoplasms have been suggested to produce antigens and the resultant immune complex formations, activating the serum complement, thus cause paraneoplastic vasculitis. In this case, severe eosinophilia and cryoglobulinemia with low complements were observed in a laboratory test. A biopsy specimen from a skin lesion revealed leukocytoclastic vasculitis with severe perivascular infiltration of eosinophils. The cutaneous vasuculitis was considered to be a manifestation of HES with MC, although there were no etiological factors of HES and MC. Therefore, the vasculitis seems to be a symptom of paraneoplastic syndrome in this case. Our finding suggests that the potential presence of malignancies should be kept in mind as a possible underlying disorder especially in the presence of HES with MC; this possibility is interesting also as regards at least part of the pathogenesis for paraneplastic syndrome

    Autoantibody to NA14 is an independent marker primarily for Sjögren's syndrome

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    12 páginas, 2 figuras, 2 tablas.-- El Pdf es el manuscrito de autor.-- et al.Nuclear Autoantigen of 14 kDa (NA14) was originally identified using the serum of a Sjögren's syndrome (SS) patient as probe in screening a human testis cDNA expression library. To date there is no report in the systematic analysis of the prevalence of autoantibodies to NA14. In this study, anti-NA14 was determined in several rheumatic diseases from independent cohorts in the US and Japan. The prevalence of anti-NA14 were 18/132 (13.6%) in primary SS, 0/50 (0%) secondary SS, 2/100 (2%) SLE, 1/43 (2.3%) scleroderma, 0/54 (0%) rheumatoid arthritis, 1/29 (3.4%) polymyositis/dermatomyositis, and 0/58 (0%) normal healthy controls. The frequencies of anti-NA14 positive sera in primary SS are statistically greater than normal healthy controls (p=0.006), secondary SS (p=0.044), and other rheumatic diseases. Furthermore, among 11 anti-NA14 positive primary SS sera, 4/11 (36.3%) sera were negative for both anti-SS-A/Ro and SS-B/La antibodies. Thus anti-NA14 autoantibodies may be useful for the discrimination of primary versus secondary SS and serve as a diagnostic marker for primary SS especially in seronegative (anti-SS-A/Ro and anti-SS-B/La antibodies negative) patients with SS.Peer reviewe
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