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Evidence have indicated the impairment of central nervous system (CNS) and neuropsychiatric disorder in patients with systemic lupus erythematosus (SLE). However, little is known to improve the brain abnormality in SLE. To investigate the effect of cystamine on brain abnormality in SLE, NZB/W F1 mice were used as the animal model. Notably, significantly reduced neural Nitric Oxide Synthase (nNOS), inducible Nitric Oxide Synthase (iNOS), p53, p21WAF1/CIP1, and heat shock protein (HSP)-90 proteins were detected in the brain of NZB/W F1 mice that were treated with cystamine. In contrast, no variation was observed between the brain samples of BALB/c mice that were treated with PBS or cystamine. Moreover, significantly reduced Toll-like receptors- (TLR-) 4, 5 and 7 were detected in the brain samples of NZB/W F1 mice that were treated with cystamine whereas no variation of TLR-4, TLR-5, TLR-7, and TLR-9 was observed in BALB/c mice that were treated with PBS or cystamine. These findings demonstrated the beneficial effects of cystamine on brain abnormality in NZB/W F1 mice and probably suggested the potential of cystamine on treating patients with neuropsychiatric SLE.

Hemolytic uremic syndrome (HUS) is not a commonly reported complication in post-transplantation patients treated with cyclosporine-A (CSA), and is extremely rare in systemic sclerosis (SSc) patients treated with this drug. CSA may contribute to the development of chronic ischemic glomerulonephropathy and vasculopathy, features not easily distinguished from SSc-related nephropathy. Here, we describe a 41-year-old Chinese man with diffuse-type SSc treated with CSA who developed thrombocytopenia, acute renal failure and hemolytic anemia and was diagnosed with HUS. Renal function and thrombocytopenia improved gradually after intensive treatment of plasma exchange (PE) and high-dose steroid therapy. After PE, renal biopsy showed ischemic glomerulonephropathy and obliterative vasculopathy. This case illustrates that PE can improve the hematological disorders and characteristic renal changes of HUS in SSc patients treated with CSA. However, this therapy may not be effective in normalizing serum creatinine level in SSc patients once CSA has triggered the normal kidney to develop glomerulonephropathy and vasculopathy with ischemic and sclerotic changes.

To understand the cytokine levels in different disease activities of patients with ankylosing spondylitis (AS), we measured proinflammatory and antiinflammatory cytokine production from peripheral blood mononuclear cells (PBMC) in patients with AS and their first-degree relatives (FDR).PBMC were obtained from 26 patients with AS and 24 FDR and then stimulated with PHA for 72 h. In the supernatants, the following three cytokines, tumor necrosis factor-alpha (TNF-alpha), interleukin 1beta (IL-1beta), and IL-10, were measured by ELISA. Disease activity in AS patients was divided into high disease activity (Group 1) and low disease activity (Group 2), based on the Bath AS Disease Activity Index (BASDAIor =4 or4). Healthy FDR of AS patients (Group 3) and healthy subjects (Group 4) were used as a control group.TNF-alpha production from PBMC was significantly increased in Group 1 patients compared to Group 2 patients (1371 +/- 1008 pg/mL vs. 355 +/- 89 pg/mL, p0.05) or FDR (1371 +/- 1008 pg/mL vs. 552 +/- 89 pg/mL, p0.05) or healthy subjects (1371 +/- 1008 pg/mL vs. 436 +/- 114 pg/mL, p0.01). IL-1beta also showed a similarly significant difference between the two groups (Group 1 vs. Group 2, Group 1 vs. Group 4) (p0.05). In contrast, IL-10 was significantly decreased in Group 1 when compared to Group 2 (126 +/- 64 pg/mL vs. 272 +/- 150 pg/mL, p0.05).Patients with high BASDAI had increased production of TNF-alpha and IL-1beta compared to those with low BASDAI or healthy FDR, suggesting that proinflammatory cytokines may play an important role during active inflammation.

Acute anterior uveitis (AAU) is the most frequently extra-articular manifestation of ankylosing spondylitis (AS). To investigate whether AAU has an association with disease activity, functional ability and physical mobility in AS patients, 146 Chinese AS patients in Taiwan were enrolled in a cross-sectional study. These patients fulfilled the 1984 modified New York criteria and visited the Outpatient Department of the Veterans General Hospital-Taipei from April 2004 to July 2005. Patients completed questionnaires assessing disease activity [Bath Ankylosing Spondylitis Disease Activity Index (BASDAI)], functional ability [Bath Ankylosing Spondylitis Functional Index (BASFI)] and patient's global assessment [Bath Ankylosing Spondylitis Patient Global Score (BAS-G)]. Meanwhile, physical examinations were performed, including Schober test, finger-to-floor, lateral spinal flexion, occiput-to-wall and chest expansion. The history of AAU was accepted only if diagnosed by an ophthalmologist. The prevalence of AAU in this Chinese AS cohort was 15.8% (23/146). Patients with AAU had a significantly higher BASDAI than those without [absolute differences=0.96, 95% confidence intervals (CI): 0.35-1.88]. Additionally, patients with AAU had significantly increased BASFI than those without (absolute differences=1.46, 95% CI: 0.33-2.59). Moreover, there was advanced limitation of physical motility in patients with AAU, including finger-to-floor, occiput-to-wall distances and Schober test, (95% CI: 3.89-16.95 and p=0.046, respectively). Disease duration mildly correlated with BASFI (r=0.24, p=0.003) but not with BASDAI (p=0.838). There was no difference of disease duration between patients with and without AAU (p=0.343). These results suggested that the presence of AAU in AS patients may be associated with higher disease activity, poor functional ability and advanced physical impairment.

Background Undifferentiated spondyloarthropathy (USpA) is a unique group in spondyloarthropathy (SpA). This study will investigate the clinical and laboratory characteristics of USpA in the Chinese population. Methods Forty two patients with USpA were enrolled from our rheumatology outpatient facility in this retrospective study. SpA was diagnosed according to the European Spondyloarthropathy Study Group (ESSG) criteria. Patients were considered having USpA when they had SpA but did not meet the criteria for the diagnosis of ankylosing spondylitis (AS), Reiter's syndrome or reactive arthritis (ReA), psoriatic arthritis (PsA), and inflammatory bowel diseases (IBD)-related arthritis, etc. Laboratory tests included erythrocyte sedimentation rate (ESR), serum C-reactive protein (CRP), immunoglobulin A (IgA), and human leukocyte antigen B27 (HLA-B27). Results Among the 42 USpA patients, the ratio of men to women was 1.47:1, and the age at onset was 32.33 ± 10.83 years old. Approximately 61.9% of patients had peripheral arthritis, 30.95% had uveitis, and 64.29% had positive HLA-B27. Among these female USpA patients, compared to males, there is a trend of older age at disease onset, higher percentage of HLA-B27 positive, more peripheral arthritis and uveitis, longer disease duration, and higher level of ESR, and IgA and CRP in serum. The items reaching significant difference between males and females were longer disease duration ( p p p = 0.03). There was no significant difference in clinical and laboratory characteristics between HLA-B27-positive and -negative groups. Conclusions Studies on USpA patients have not been reported in the Chinese population. In this study, we demonstrate the unique demographic characteristics, clinical and laboratory data of USpA in the Chinese population. These findings should be confirmed by analyzing larger number of patients and longer time for further follow-up. Such studies are crucial to understand the pathogenesis of USpA and evaluate its prognosis.

Hookworms, together with other helminthes infestations, were serious health problems in Taiwan before. With improvements in public health and medicine, the prevalence of parasite infestations drops significantly and current attention has been shifted to foreign laborers coming from the Southeast Asia. Herein we reported a case of hookworm infestation who presented as subacute diarrhea. Initially, only peripheral eosinophilia was revealed. Anti-helminth treatment, but not the immunosuppressant for eosinophilic gastroenteritis, was given when the hookworm ova were finally harvested after repeated stool examinations. Please remember the old diseases when you meet a patient with gastrointestinal complaints and peripheral eosinophilia, especially when there are intermittent skin atopic manifestations. Repeated parasite ova checks with concentration method are necessary in acute stage, especially when the worm burden is not heavy.

Adult onset Still's disease (AOSD) is frequently manifested by the appearance of fever, skin rash, arthritis, sore throat, leucocytosis, hypertransaminasaemia, or hepatosplenomegaly [1]. Severe lif...

Spondyloarthropathy (SpA) is a family of chronic arthritis, characterized by inflammatory back pain, peripheral arthritis, and enthesitis [1]. SpA comprises at least five major subtypes, including ...

A 79-year-old male with acute myelogenous leukemia developed acute right knee arthritis during admission, after the use of broad-spectrum antibiotics before chemotherapy. The initial synovial fluid sample appeared to be mildly inflammatory with a low white cell count. The fungal septic arthritis was not diagnosed until Candida tropicalis, a rare species of Candida, was isolated in the synovial fluid. Although fluconazole is effective in treating the microorganism, the untreated leukemia rendered the infection incurable and led to the growth of fluconazole-resistant Candida krusei. We reported the unusual case of fungal arthritis and reviewed the literature.

Objective.Sjögren’s syndrome (SS) is an inflammatory autoimmune disease. We investigated important factors associated with the expression of inflammation-related molecules in minor salivary gland (MSG) mononuclear cells in patients with SS.Methods.Thirty-four patients with SS with a MSG biopsy grading of either grade III (10 patients) or grade IV (24 patients) were enrolled. The age, sex, autoantibodies, cell infiltration, and intercellular adhesion molecule-1 (ICAM-1), matrix metalloproteinase-3 (MMP-3), tumor necrosis factor-related apoptosis-inducing ligand (TRAIL), or CXCR3 expression were also analyzed.Results.Ten of the 34 patients with SS were diagnosed with secondary SS; in these patients, the diagnosis of rheumatoid arthritis was confirmed in 8 and systemic lupus erythematosus in 2. TRAIL and ICAM-1 were overexpressed in patients with antinuclear antibodies (ANA) > 1:160, compared to those with titer < 1:160 (45.1 ± 4.4 vs 41.2 ± 3.9, p = 0.021, and 15.2 ± 5.7 vs 10.8 ± 3.3, p = 0.018, respectively). Higher erythrocyte sedimentation rate (ESR; ≥ 20) was associated with higher TRAIL expression and CD20 cell infiltration in contrast to lower ESR (< 20; p < 0.05). ICAM-1, TRAIL, and MMP-3 were expressed more predominantly in anti-SSA-positive than in anti-SSA-negative patients with SS. There was a significant difference in CD20 cell infiltration and MMP-3 expression between primary SS and secondary SS. Biopsy of a grade IV showed a significantly increased expression of TRAIL (44.9 ± 4.5 vs 40.8 ± 3.6, p = 0.013) and MMP-3 (62.7 ± 6.3 vs 54.4 ± 7.3, p = 0.003) in mononuclear cells as compared to those of grade III.Conclusion.In our study, pathologic grading with a higher grade (grade IV) and the presence of SSA or a higher titer of ANA were significantly associated with the overexpression of TRAIL, MMP-3, or ICAM-1 in the salivary gland mononuclear cells in patients with SS.

Autoimmune inner ear disease (AIED) is a very rare disorder with distinct clinical features and can occur in patients with malignancy or autoimmune diseases. We report a 72-year-old male patient with polyarteritis nodosa treated continuously for 5 years with aggressive immunosuppressive drugs, including cyclophosphamide, who experienced three episodes of acute hearing loss during treatment. Organic lesions of the external and middle ear were excluded by repeated examinations, and if one subscribes to McCabe's (Ann Otol Rhinol Laryngol 88:585-589, 1979) definition of AIED, this condition must be considered as the likely cause of the hearing loss. During the period of treatment, three episodes of AIED occurred, and eventually, lung cancer developed. From the time relationship and clinical manifestations of neuropathy and livedo reticularis, the first episode of hearing loss was more likely to be related to vasculitis itself, while the third episode may well have been associated with the development of lung cancer given the dramatic improvement in the clinical condition following treatment of the tumor by excision and cancer chemotherapy. Coexistence of AIED, vasculitis, and malignancy in the same patient has only been reported infrequently, and our case suggests that this coexistence may not be coincidental. For those patients with autoimmune disease who are on long-term immunosuppressive drug therapy, active surveillance for a nascent malignant tumor should be exercised if AIED recurs or persists.

Ankylosing spondylitis (AS) is a chronic inflammatory rheumatic disease that affects the spine and sacroiliac joints, as well as the peripheral joints [1]. Hip arthritis occurs in 30% of all AS pat...