Effects of a respiratory functional training program on pain and sleep quality in patients with fibromyalgia: A pilot study.

OBJECTIVE: To evaluate the effect of 8-week respiratory functional training program on pain tolerance, sleep, and urinary antioxidant and cortisol levels in 18 patients with fibromyalgia. METHODS: Participants underwent a 12-week intervention: 4 weeks as control and 8 weeks of breathing exercises. Pain tolerance assay was done by using an algometer, whereas sleep quality was evaluated by actigraphy and by the Pittsburgh Sleep Quality Index. Cortisol and antioxidant levels were determined using commercial assay kits. RESULTS: Increases in the pain tolerance threshold were detected in the occiput point after one month of intervention as well as in the low cervical and second rib points after one and two months. Actigraphy revealed a decrease in sleep latency, whereas sleep questionnaire showed improvements in sleep quality, sleep duration and sleep efficiency. No changes in cortisol and antioxidant levels were detected. CONCLUSION: The 8-week breathing exercise intervention reduced pain and improved sleep quality.

Effect of Comedication With Conventional Synthetic Disease-Modifying Antirheumatic Drugs on Retention of Tumor Necrosis Factor Inhibitors in Patients With Spondyloarthritis: A Prospective Cohort Study.

OBJECTIVE: To evaluate whether use of comedication with conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) influences the retention of tumor necrosis factor inhibitors (TNFi) in patients with spondyloarthritis (SpA). METHODS: Patients with SpA from the Rheumatic Diseases Portuguese Register who started treatment with their first TNFi between 2001 and 2014 were included in this study. Cox regression analysis was used to estimate the effect of comedication with csDMARDs on TNFi retention in 2 types of models: a model in which baseline (time-fixed) variables were included, and a second model incorporating time-varying variables, including sociodemographic features, measures of disease activity, measures of physical function, and cotreatment with other drugs (nonsteroidal antiinflammatory drugs and oral steroids). To control for possible confounding by indication, the effect of csDMARD comedication on TNFi retention was also tested after adjustment for the treatment propensity score. RESULTS: In total, 954 patients were included in the study, of whom 289 (30.3%) discontinued treatment with their first TNFi after a median follow-up time of 2.5 years (range 0.08-13 years). Inefficacy was the most common reason for TNFi discontinuation (55.7% of patients). In the multivariable analyses, comedication with csDMARDs had no measurable effect on TNFi retention, neither in the baseline model (hazard ratio [HR] 0.83, 95% confidence interval [95% CI] 0.59-1.16) nor during follow-up in the model adjusted for time-varying covariates (HR 1.07, 95% CI 0.68-1.68). The effect of csDMARD comedication remained nonsignificant after propensity score adjustment. CONCLUSION: Comedication with csDMARDs does not prolong TNFi retention in patients with SpA in clinical practice, suggesting that there is no benefit conferred by the concomitant use of these drugs.

[Relationships between widespread pain and thresholds pain tolerance on tender points in Portuguese women with fibromyalgia: impact on daily life]. / Relação entre a dor generalizada e o limiar de tolerância à dor nos pontos sensíveis em mulheres portuguesas com fibromialgia: impacto sobre a vida diária.

OBJECTIVES: to establish a relationship between widespread pain subjectively perceived and threshold pain tolerance on tender points, and to determine whether there are differences in threshold pain tolerance on tender points between the upper and lower body, as well as between the dominant and non-dominant side, and whether these differences have an impact on the daily life of Portuguese women with fibromyalgia (FM). MATERIAL & METHODS: thirty-one women with FM aged between 34 and 67 years volunteered for the study. Threshold pain tolerance was assessed at critical points using a digital algometer pressure; the widespread pain index (WPI) was constructed by the addition of 19 painful body regions; and the impact on the daily life assessed by the Portuguese version of the Fibromyalgia Impact Questionnaire (FIQ), with individual interviews. RESULTS: significant differences between the percentage of threshold pain tolerance of the whole body and the scales of widespread pain subjectively perceived were observed, showing that the widespread pain subjectively perceived by patients was between +25.9% and +27.5%. Also, significant differences between threshold pain tolerance of tender points located on the upper and lower body (1.9 ± 0.5 kg/cm2 vs. 2.6 ± 0.7 kg/cm2; respectively) were observed. However, no significant differences were found between threshold pain tolerance of tender points located on dominant and non-dominant sides (2.1 ± 0.5 kg/cm2 e 2.1 ± 0.6 kg/cm2; respectively). Additionally, the analysis showed significant correlations between pain and patient`s daily life in: FIQ total score, physical function, feel good, job ability and fatigue. CONCLUSIONS: the women with FM show higher widespread pain subjectively perceived than threshold pain tolerance on tender points. Furthermore, the pain suffered by the patients with FM, especially that located on the upper body, either on the dominant or on the non-dominant side, has a negative influence on physical function, job ability, fatigue and feel good, affecting daily life.

Childhood hypophosphatasia with myopathy: clinical report with recent update.

Hypophosphatasia is a rare genetic disease with low tissue nonspeficic alkaline phosphatase activity (TNSALP), due to ALPL gene mutation. There are 6 clinical forms. Childhood form is caractherized by short stature, premature loss of decidous teeth and diffuse bone pain associated with a pathological bone fracture in the past. Laboratory findings present low serum level of alkaline phosphatase and high levels of serum and urinary extracelular metabolytes. It is described a case report of a 34 years old woman with previous diagnosis of childhood hypophosphatasia, caryotype 46,XX, and molecular screening for the gene ALPL with a c.1426>A p.E476K mutation, who complained of proximal muscular weakness intensified with the cold weather, exercise, and a waddling gait. The electromyography was compatible with myopathy but the muscle biopsy was normal. The serum creatine kinase levels were normal, as well as the others muscle enzymes. Clinical and laboratory/ /imaging dissociation is frequent in other metabolic bone diseases as osteomalacia. The rarity of this case of childhood hypophosphatasia with "de novo" non-progressive myopathy of the lower limbs, justified a case report with literature revision.

[Fibrous dysplasia and McCune-Albright syndrome: case-report and differential diagnose with Paget bone disease]. / Fibrodisplasia óssea e síndrome de McCune-Albright: descrição de um caso clínico e diagnóstico diferencial com doença óssea de Paget.

Fibrous dysplasia of bone or Jaffe Lischtenstein's disease is a genetic, non-inheritable disease of bone development, characterized by bone pain, deformities and fracture, mainly observed in yo ung adults. The frequency is equal between sexes. Mutations in the gene coding the Gsα, GNAS complex, results in osteoblastic differentiation defects, and bone resorption. The disease can have a monostotic or polyostotic form, or be associated with café-au-lait skin spots and precocious puberty (McCune-Al bright syndrome). The normal bone and bone marrow is replaced with abnormal benign intramedullary fibro-osseous tissue, and can involve any bone in the body. The vertebral involvement is rare. Radiological and pathological findings can be diagnostic. Biphosphonates and calcium, vitamin D and phosphorus supplements have been used in fibrous dysplasia treatment. Osteosarcoma is a rare, but serious malignant complication. We report the case of a 68 year old woman with a history of hypofisectomy, with a progressive low back pain, without systemic or neurological symptoms. The bone scan, the ra di o graphs and the computed tomography findings revealed polyostotic fibrous dysplasia, with vertebral and mandibular involvement. In this paper we compared fibrous dysplasia of bone with Paget bone disease.

EULAR evidence-based recommendations for the management of fibromyalgia syndrome.

OBJECTIVE: To develop evidence-based recommendations for the management of fibromyalgia syndrome. METHODS: A multidisciplinary task force was formed representing 11 European countries. The design of the study, including search strategy, participants, interventions, outcome measures, data collection and analytical method, was defined at the outset. A systematic review was undertaken with the keywords "fibromyalgia", "treatment or management" and "trial". Studies were excluded if they did not utilise the American College of Rheumatology classification criteria, were not clinical trials, or included patients with chronic fatigue syndrome or myalgic encephalomyelitis. Primary outcome measures were change in pain assessed by visual analogue scale and fibromyalgia impact questionnaire. The quality of the studies was categorised based on randomisation, blinding and allocation concealment. Only the highest quality studies were used to base recommendations on. When there was insufficient evidence from the literature, a Delphi process was used to provide basis for recommendation. RESULTS: 146 studies were eligible for the review. 39 pharmacological intervention studies and 59 non-pharmacological were included in the final recommendation summary tables once those of a lower quality or with insufficient data were separated. The categories of treatment identified were antidepressants, analgesics, and "other pharmacological" and exercise, cognitive behavioural therapy, education, dietary interventions and "other non-pharmacological". In many studies sample size was small and the quality of the study was insufficient for strong recommendations to be made. CONCLUSIONS: Nine recommendations for the management of fibromyalgia syndrome were developed using a systematic review and expert consensus.

[Systemic lupus erythematosus and anaemia]. / Lúpus eritematoso sistiémico e anemia.

The authors report the case of a 48-years-old Caucasian women, with a previous diagnosis of systemic lupus erythematosus characterized by asthenia, fever, skin rash, alopecia, Raynaud's phenomenon, arthritis, pericardial effusion, interstitial pulmonary involvement, diffuse proliferative glomerulonephritis with crescents and anemia. The presence of severe anemia refractory to high doses of glucocorticoids (1 mg/ /Kg/day), iron therapy and blood transfusions, associated with a low reticulocyte count determined the execution of a bone marrow aspiration, biopsy and immunophenotyping, which were compatible with the diagnosis of Myelodysplastic Syndrome. The treatment with erythropoietin (5.000U 3x/week) and cyclophosphamide pulses (1 gr/m(2) month) induced complete regression of morphologic bone marrow changes and anemia. The main causes of anemia in lupus patients are discussed.

[The diagnosis and treatment of fibromyalgia]. / Diagnóstico e tratamento da fibromialgia.

Fibromyalgia syndrome (FMS) affects predominantly females and is characterised by widespread musculoskeletal pain, fatigue, insomnia, nonrefreshing sleep, diffuse stiffness and other organic and psychic signs and symptoms. Diagnosis is essentially based on the 1990 American College of Rheumatology Classificative Criteria, but if, in some cases, they are not completely fulfilled, diagnosis is not excluded in a particular patient. The causes of the enhanced pain perception and of all the other clinical characteristics are unknown. Both the central hypothesis (sleep disturbance; psychological affection; hypothalamus-hypophysis-adrenal axis disorder; neuromediators disregulation; etc.) and the peripheral theory (anatomical and/or functional muscle disturbance) try to explain FMS etiopathogenesis. Tricycles antidepressants (i.e. amitriptilin) and some muscle relaxants (i.e. ciclobenzaprine) have demonstrated some beneficial effect contrary to the classic antirheumatic drugs (NSAID; corticosteroids; etc.). Physical exercise, multidisciplinary support (behavioural therapy, physical agents; etc.) and patient education are some of the other approaches which contribute to the correct management of FMS.

[Viral infection and fibromyalgia]. / Infecção viral e fibromialgia.

Fibromyalgia (FM) is a very frequent syndrome of unknown cause, characterized by generalized pain, fatigue and a number of tender points to palpation. Among the several etiopathogenic hypotheses discussed, the association of FM with some viral infections has been the object of multiple studies due to its relation and similarity with the chronic fatigue syndrome, acknowledges as being related, although not exclusively, with the chronic infection by the Epstein-Barr virus. Many individual descriptions of association between infection with the human parvovirus B19 and FM led us to carry out this study, comparing the serology for that virus in 52 patients with FM and 39 healthy controls. The titers of specific IgG anti-parvovirus B19 antibodies, indicating previous infection with that virus, were determined in all 91 individuals through ELISA method and at the same laboratory. Results revealed, though not significantly, a greater prevalence of positive titers, of which the mean was also higher, in patients than in controls. When comparing the women from both groups, this tendency was even less perceptible. These data imply that there is no etiologic association between infection with the human parvovirus B19 and FM.