Rheumatology international
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Review Case Reports
An unusual case of undifferentiated connective tissue disease presenting as cardiac tamponade.
Cardiac tamponade as an initial manifestation of undifferentiated connective tissue diseases (UCTD) is extremely rare, with only one case reported in literature thus far. We describe here, a case of a middle-aged man who presented with symptoms of fatigue, exertional dyspnea and orthopnea. His physical exam was significant for anasarca, elevated JVP and pulsus paradoxus. ⋯ He was therefore diagnosed with UCTD, and successfully treated with colchicine after emergency pericardiocentesis. This case presents UCTD as a rare cause of cardiac tamponade and large pericardial effusions and suggests that colchicine can be used to treat UCTD-associated effusions. These patients once diagnosed, are at risk of developing known connective tissue diseases within 5 years of disease onset and should be followed up in clinic periodically.
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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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Comparative Study
Deconditioning in chronic low back pain: might there be a relationship between fitness and magnetic resonance imaging findings?
The presence of persistent low back pain leads to avoidance of daily activities, contributes to further exercise intolerance and subsequent further loss of functional capacity. We sought to search for the relationship between lumbar magnetic resonance imaging findings and deconditioning in a homogeneous subset of patients with low back pain. We studied 20 sedentary, nonsmoking patients with chronic low back pain using symptom-limited cardiopulmonary exercise testing using treadmill breath-by-breath analysis using modified Bruce protocol. ⋯ The Pfirrmann grading of lumbar disc degeneration was positively correlated with Weishaupt grading of facet degeneration (r = 0.502, P = 0.024); however, VO(2) peak was only negatively correlated with facet degeneration (r = -0.5, P = 0.025). Facet joint-mediated pain is a significant problem in all patients suffering from chronic low back pain. Since in this study, aerobic fitness level was negatively correlated with facet degeneration, we suggest that degeneration of facet joints might better reflect the chronicity of low back pain when compared to intervertebral disc degeneration.
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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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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.