BackgroundBehcet's disease (BD) is a chronic and multisystemic inflammatory disorder affecting the skin, mucosa, joints, eyes, arteries, veins and the nervous and gastrointestinal systems. The symptoms may be separated by long or short intervals, occur simultaneously or in sequence, and exhibit a pattern of exacerbation and remission. The disease itself or the impact of the symptoms affect patients physically, mentally and socially with a negative effect in the quality of life (QoL).ObjectivesTo determine the health status and quality of life in patients with BD followed in a rheumatology unit and to identify associated demographic and disease-related parameters influencing them.MethodsCross-sectional study of 44 BD patients and 39 healthy controls matched for age and sex. All subjects completed the Health Assessment Questionnaire (HAQ) to assess impairment in daily activities due to illness, Short Form-36 (SF-36) and EuroQol Visual Analogic Scale (EQ-VAS) to assess health related quality of life (HRQL). The disease characteristics, including disease duration and clinical involvements were collected. The Birmingham Vasculitis Activity Score (BVAS) was applied for the evaluation of current disease activity among BD patients. P value <0.05 was defined as statistically significant.ResultsAmong 44 patients, 35 (79.5%) were female, with a mean age of 40.07 years and mean disease duration of 5.93 years. BD patients had significantly higher HAQ score (p=0.009) and lower levels of SF-36 (p<0.001) than the healthy controls. The predominant contributors to this low SF-36 were general health, vitality and role-emotional domains. In comparison with healthy controls, patients with inactive disease (BVAS=0) also had a higher HAQ, but without significant differences (p=0.53). The total SF-36 score also showed lower levels in patients with inactive disease (p=0.04), but when compared the different components, only half of them maintained significant differences, namely role-physical (p=0.03), general health (p<0.001), vitality (p<0,001) and social functioning (p=0.05). The controls showed a higher EQ-VAS, with a mean of 88.44 comparing to 68.36 in all BD patients (p<0.001) and 74.12 in the subgroup with inactive disease (p=0.003). SF-36 score was negatively correlated with HAQ (r=-,553, p<0.001) and positively correlated with EQ-VAS (r=.388, p<0.001). Longer disease duration correlated with lower levels of only some SF-36 domains, namely physical functioning (r=-.436, p=0.003), role-physical (r=-.533, p<0.001) and role-emotional (r=-.465, p=0.001). There was no correlation between disease activity and disease duration (p=0.678) or different scores evaluating QoL (p=0.876). The gender was not associated with statistical differences when compared clinical involvements, disease duration, current disease activity, HAQ, EQ-VAS or SF-36 scores. The heterogeneous nature of the disease expression did not allow the study of the association with the levels of health status and QoL.ConclusionsOur findings showed lower levels of QoL and glob...
BackgroundThe muscle biopsy may be a fundamental technique in the suspicion of myopathy, with high specificity to distinguish between normal or abnormal muscle tissue. In association with clinical and laboratory findings, the muscle biopsy has an important role to a more accurate diagnosis.ObjectivesTo evaluate the usefulness and safety of muscle biopsies performed in a Rheumatology Unit in patients with suspected myopathy.MethodsRetrospective analysis of the clinical charts of patients submitted to muscle biopsy between January 2010 and December 2016 at our Rheumatology Unit. Demographic, clinical, laboratory, electromyographic and histological data were collected. The histological study was performed in a Neuropathology Specialized Unit.ResultsA total of 46 patients, 19 men and 27 women, with a mean age of 53.3±17.1 years, were evaluated. Clinical manifestations included muscle weakness, myalgia and decreased muscle strength. Most patients also had increased muscle enzymes, particularly creatine kinase, but in a patient with generalized muscle atrophy, muscle enzymes were overall diminished. Of the 46 biopsies, 12 (26.1%) did not show alterations, 8 (17.4%) showed nonspecific alterations and only 1 biopsy was not conclusive because the sample was inadequate. In 4 patients, the histological features did not present specific characteristics of a myopathy, but revealed a preferential atrophy of type 2 fibbers, usually associated with prolonged corticosteroid therapy. Among the others, 9 (19.6%) were compatible with inflammatory myopathies, namely polymyositis (6), dermatomyositis (1), inclusion body myopathy (1), and localized nodular myositis (1). In the latter case, the patient had a different clinical presentation, with intermittent episodes of pain, oedema and flushing of different muscle groups. In addition, 5 metabolic myopathies (2 McArdle's diseases and 3 non-specific metabolic disorders), 2 muscular dystrophies (1 Becker's muscular dystrophy and 1 dystrophinopathy), 1 suspected case of myotonic dystrophy and 1 myopathy associated with statins use were diagnosed. In a patient with muscle weakness and prior diagnosis of systemic vasculitis, the histology showed a chronic inflammatory process with no specific alterations. In the patient with overall decrease in muscle enzymes, the biopsy revealed neurogenic atrophy, without inflammatory infiltrates. Overall, the results of electromyography (EMG) did not correlate with the histological findings, because EMG identified alterations both in cases with histologically compatible inflammatory myopathy and in cases without histological pathology. On the other hand, EMG did not reveal any changes in some of the metabolic myopathies. Muscle biopsies were performed mainly in the deltoid muscle. There were no relevant immediate or late complications with this technique.ConclusionsAlthough muscle biopsy is an invasive technique, it is a safe technique and allows the differential diagnosis between the various myopathies, which is fundamental to an appropriate treatment.Ref...
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