Rheumatology international
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Hyperimmunoglobulin D and periodic fever syndrome (HIDS) is a rare, hereditary autoinflammatory condition, characterized by recurrent inflammatory episodes. There is no proven treatment for HIDS, but various drugs including, non-steroidal anti-inflammatory drugs, colchicine, steroids, statins and thalidomide have all been tried. Recently, some patients have demonstrated a good clinical response to either etanercept or anakinra. We report a case of a 10-year-old girl who experienced prolonged and severe inflammatory attacks, when she was treated with etanercept, and later with anakinra.
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The aim of the study was to investigate the validity and reliability of the Turkish version of the fatigue severity scale (FSS) in fibromyalgia (FM) patients. Sixty-one FM patients and 54 healthy controls were evaluated using the Turkish version of the FSS. Reliability was investigated using test-retest reliability and internal consistency. ⋯ For the convergent validity, correlations between the FSS and BDI, BAI, FIQ, pain intensity were moderate to good in both groups (P: 0.000). The Turkish version of the FSS has been proved to be valid and reliable to detect severity of fatigue in FM patients. We recommend the use of it in clinical practice.
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Objective of this study is to asses the relationship between tender point count (TPC) and the severity of disease in patients with primary fibromyalgia (FM). One hundred and seven female patients with FM diagnosis according to ACR criteria were included to the study. Main outcome measures were fibromyalgia impact questionnaire (FIQ), Beck depression inventory (BDI), visual analog scale (VAS) and tender point assessment. ⋯ TPC was correlated positively with FIQ and VAS scores. There was no correlation between TPC and age and duration of symptoms. In conclusion, TPC is a simple and noninvasive examination finding that can supply information about the disease severity and the depression in FM.
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We performed a cross-sectional study of the demography, clinical and laboratory features of patients with polymyositis and dermatomyositis followed up in our centre from 2006 to 2009. There were 12 cases, with the majority of them (58.3%) being woman. They have a mean age of 57.8 years and mean disease duration of 11.83 (SD 9.92) months. ⋯ The mortality rate in our group was 4(33.3%) over the 4-year period. This study demonstrated the rarity of PM/DM in our centre with considerable lag time to diagnosis in our patients. Despite lack of muscle biopsy in our centre, our centre achieved appropriate diagnosis and management of PM/DM.
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Disseminated intravascular coagulation (DIC) may complicate a variety of disorders that contribute to mortality, particularly those related to bleeding. It is therefore very difficult to manage DIC in patients with known bleeding disorders. We treated a 62-year-old woman with idiopathic thrombocytopenic purpura (ITP) complicated with sepsis-induced DIC. ⋯ Based on these findings, the patient was diagnosed with sepsis-induced DIC due to urinary tract infection. Thrombocytopenia was refractory despite the use of antibiotics and platelet transfusion, but it was promptly improved in response to recombinant human soluble thrombomodulin (rTM). We suggest that rTM provides a new therapeutic strategy for DIC patients with high hemorrhagic risk.