Fibromyalgia in primary antiphospholipid (Hughes) syndrome.

Fibromyalgia (FM) is a syndrome that can be associated with several rheumatic diseases. However, no study has evaluated its frequency in patients with primary antiphospholipid syndrome (PAPS). The objective of this study was to analyze the frequency of FM in PAPS patients compared with healthy controls, to determine the possible associations between FM and PAPS features, and also to evaluate quality of life and depression in these patients. This case-control study included 30 PAPS patients (by the Sapporo criteria) and 40 healthy subjects. Demographic and clinical data, drug use, and antiphospholipid antibodies were analyzed. FM was diagnosed based on international criteria (ACR). Questionnaires on quality of life, including the Short Form 36 Health Survey (SF-36), Beck Depression Inventory (BDI), Fibromyalgia Impact Questionnaire (FIQ), and Visual Analog Scale (VAS), were also applied. PAPS patients and controls were similar in mean age as well as in distributions of gender and Caucasian race. Mean disease duration was 5.4 ± 4.2 years. A diagnosis of fibromyalgia was made in five (16.7%) PAPS patients and no controls (p = 0.012). PAPS patients had more diffuse pain (53% vs. 0%, respectively, p < 0.0001), ≥11 tender points (23% vs. 5%, respectively, p = 0.032), and a greater total number (175 vs. 57, respectively, p < 0.0001) as well as median number of tender points per patient than controls (5 [0-18] vs. 0 [0-11], respectively, p < 0.0001). PAPS patients had lower values in all dimensions of the SF-36, as well as higher FIQ scores, higher BDI scores, more depression diagnoses according to BDI results, and increased VAS in comparison with controls. Analysis of PAPS patients with FM compared with those subjects without FM revealed no significant differences regarding demographic features or thrombotic or clinical events; however, PAPS patients who also had FM had lower values in SF-36 dimensions as well as higher FIQ (82.6 ± 9.6 vs. 33.6 ± 29.8, respectively, p < 0.0001) and VAS scores (6.6 ± 2.97 vs. 3.25 ± 3.11, respectively, p = 0.03). BDI scores, in contrast, were similar in both groups. In conclusion, one-fifth of PAPS patients had fibromyalgia and a low quality of life when compared with healthy subjects.

Primary antiphospholipid syndrome with thrombotic thrombocytopenic purpura: a very unusual association.

This report considers the rare situation in which primary antiphospholipid syndrome (PAPS) is linked with thrombotic thrombocytopenic purpura (TTP). It describes the case of a young lady with PAPS, characterized by recurring cerebro-vascular abnormalities and marked livedo reticularis, combined with circulating anticardiolipin and lupus anticoagulant antibodies. On follow-up, while on oral anticoagulation, she developed severe thrombocytopenia associated with hematuria, microangiophatic anaemia and neurological manifestations consistent with a diagnosis of TTP. The patient was treated with pulses of methylprednisolone and plasmapheresis with plasma exchange. The result was a favourable outcome. To our knowledge, this is the seventh report on this rare association in the English-language literature of this field.

Effectiveness of muscle stretching exercises with and without laser therapy at tender points for patients with fibromyalgia.

OBJECTIVE: To assess the efficiency of a treatment composed of muscle stretching exercises, associated or not to laser therapy at tender points, for patients with fibromyalgia (FM), in view of bettering their quality of life. METHODS: Twenty FM patients were randomly assigned to two groups: one submitted to laser therapy and stretching (LSG, n=10), and the other only to stretching exercises (SG, n=10). The visual analog scale of pain (VAS) and dolorimetry at tender points were used to assess pain; life quality was evaluated by means of the Fibromyalgia Impact Questionnaire (FIQ) and the 36-item Short-Form Health Survey (SF-36). RESULTS: After the treatment program, both in LSG and SG were detected pain reduction, higher pain threshold at tender points (all p<0.01), lower mean FIQ scores, and higher SF-36 mean scores (all p<0.05). No significant differences were found between both groups. CONCLUSION: The stretching exercises program proposed is efficient to reduce pain and painful sensibility at tender points, thus enhancing patients' quality of life. Laser therapy has not shown advantages when added to muscle stretching exercises.

Cross-cultural adaptation of the Behçet's Disease Current Activity Form (BDCAF) to Brazilian Portuguese language.

The Behçet's Disease Current Activity Form (BDCAF) is a clinical instrument used to assess the activity of Behçet's disease (BD), which was originally developed in English. The aim of the present study was to perform a cross-cultural adaptation of the BDCAF to Brazilian Portuguese language and to evaluate its reliability in a population of Brazilian patients with BD. Brazilian Portuguese version of the BDCAF, named BR-BDCAF, was obtained according to established guidelines. Forty Brazilian patients with BD diagnosed according to the International Study Group for Behçet's Disease criteria were assessed by two rheumatologists in independent sessions and submitted to the BR-BDCAF. Inter- and intraobserver agreement were then evaluated by kappa scores (values higher than 0.6 indicated good agreement). Good inter- and intraobserver agreements were achieved for the most common manifestations of BD: kappa scores higher than 0.6 were obtained for oral and genital ulcerations, skin lesions, and articular and general complaints. Moderate interobserver agreement was obtained for ocular activity (kappa 0.483) and fair interobserver agreement was obtained for gastrointestinal (kappa 0.322), major vessel (kappa 0.281), and central nervous system activity (kappa 0.304). BR-BDCAF was found to be a reliable instrument for the classic mucocutaneous and articular manifestations of BD and for general complaints, but complementary assessment is needed to evaluate specific visceral involvement for disease activity.

Radionuclide imaging for the assessment of esophageal motility disorders in mixed connective tissue disease patients: relation to pulmonary impairment.

Esophageal functional abnormalities may lead to regurgitation, chronic esophagitis and life-threatening conditions such as aspiration pneumonia. In mixed connective tissue disease patients, previous reports showed that esophageal dysfunction varies according to the method employed for investigation. Our study was conceived to: (i) assess esophageal motility and mucosal aspects in patients with mixed connective tissue disease by endoscopy, cine-esophogram and scintigraphy focusing on the prevalence of each exam; and (ii) verify the association between pulmonary and esophageal dysfunctions. Twenty-four mixed connective tissue disease patients were enrolled for this study. Cine-esophogram and upper digestive endoscopy with mucosal biopsy were performed according to previous standardization. Radionuclide esophageal scintigraphy was performed with a semisolid meal with (99m)Tc. Eleven healthy individuals voluntarily submitted to scintigraphy as controls. Cine-esophogram showed esophageal delayed emptying in 90% of patients. At scintigraphy there was a significant delay in total esophageal transit time in the group of patients when compared to healthy controls (35.3 +/- 8.2 s. vs. 13.6 +/- 9.5 s.; P < 0.0001). The whole esophageal body showed dysmotility in 96% of patients. The cine-esophogram detected functional esophageal impairment similar to scintigraphic findings. Histopathologic examination found esophagitis in 95% of studied patients. Reduced lung volumes were associated with esophagitis and delayed esophageal clearance at scintigraphy, observed at the distal portion of the esophagus. Esophageal scintigraphy is easy to perform, with good acceptance by patients with low radiation exposition. It is a useful non-invasive test for follow-up and interventional studies concerning esophagus dysfunction.

Depressão e qualidade de vida em pacientes com fibromialgia / Depression and quality of life among patients with fibromyalgia

CONTEXTO: Fibromialgia é uma síndrome reumática caracterizada por dor musculoesquelética difusa e crônica, e sítios dolorosos específicos à palpação (tender points). Freqüentemente é associada à fadiga generalizada, distúrbios do sono, rigidez matinal, dispnéia, ansiedade, alterações no humor que podem evoluir para um quadro de depressão. Sendo assim, afeta negativamente a qualidade de vida das pessoas. No entanto, ainda não se sabe o poder de discriminação dos instrumentos de avaliação da depressão e qualidade de vida. O objetivo desse estudo foi avaliar a depressão e a qualidade de vida de pacientes com fibromialgia e avaliar o poder de discriminação de instrumentos relacionados a esses aspectos. MÉTODO: Para avaliar a qualidade de vida foram utilizados dois instrumentos: um específico, o Fibromyalgia Impact Questionnaire (FIQ) e outro genérico, o Medical Outcomes Study 36-item Short-Form Healthy Survey (SF-36); para avaliar a depressão, a Escala de Depressão de Beck (BDS). Participaram do estudo 40 mulheres sendo 20 com fibromialgia - Grupo teste e 20 saudáveis - Grupo controle. Os três protocolos foram aplicados aos indivíduos dos dois grupos em uma única sessão de avaliação. Toda a análise estatística foi realizada utilizando-se o teste "t" Student, com significância alfa = 0,05. RESULTADOS: Houve diferença estatisticamente significante entre os grupos controle e teste em todos os questionários (p<0,05). Os três questionários têm bom poder discriminatório para diferenciar os dois grupos, porém o FIQ é o que tem maior poder de discriminação e o BDS o menor. CONCLUSÃO: O grupo de pacientes com fibromialgia tem pior qualidade de vida quando comparado com o grupo controle. Comparando os três questionários todos são eficientes não somente para avaliar a qualidade de vida mas também para avaliar a depressão. Entretanto, como o FIQ é um instrumento específico é o que melhor discrimina o grupo teste do grupo controle, fato este mostrado...

BACKGROUND: Fibromyalgia is a rheumatic syndrome characterized by widespread chronic musculoskeletal pain and specific palpable tender points. It is often associated with generalized fatigue, sleep disturbances, morning stiffness, dyspnea, anxiety and mood disturbances that may evolve into depression. Thus, such individuals' quality of life is negatively affected. However, the discriminating power of quality-of-life and depression assessment instruments is still unknown. OBJECTIVE: To assess depression and quality of life among patients with fibromyalgia, and the discriminating power of such instruments. METHOD: Quality of life was assessed by one specific instrument, the Fibromyalgia Impact Questionnaire (FIQ), and another generic one, the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36). The Beck Depression Scale (BDS) was used to assess depression. Forty women participated: 20 with fibromyalgia (test group) and 20 healthy women (control group). The three protocols were applied to all individuals in both groups in a single evaluation session. All statistical analyses were performed using Student's t-test, with alpha = 0.05. RESULTS: Statistically significant differences between test and control groups were found via all questionnaires (p<0.05). The three questionnaires had good discriminatory power for differentiating between the two groups, but FIQ had the greatest and BDS least. CONCLUSION: Patients with fibromyalgia presented poor quality of life, compared with the control group. Comparing the three questionnaires, they were all efficient for both quality-of-life and depression assessments. However, since FIQ is a specific instrument, this gave the best discrimination between test and control groups, and this was shown through higher, statistically significant values.