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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Juvenile idiopathic arthritis (JIA) is the most common rheumatologic disorder of childhood. It is a group of diseases characterized by chronic synovitis and associated with many extra-articular manifestations including cardiac and pulmonary involvement. Cardiac involvement as pericarditis, myocarditis and valvular disease is common in JIA. ⋯ There was significant inverse correlation between lung function parameters and the rheumatoid factor titer, erythrosedimentation rate, disease duration and the duration of methotrexate use (P < 0.01). Despite of an asymptomatic cardiopulmonary status, significant systolic and diastolic functional abnormalities exist in children with JIA. Also, both restrictive and obstructive lung impairments were found.
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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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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.