Myo-Spain: Registro de pacientes con miopatía inflamatoria idiopática de España. Metodología

ObjectivesTo describe the methods of the Spanish Registry of patients with idiopathic inflammatory myopathy (IIM) (Myo-Spain), as well as its strengths and limitations. The main objective of the project is to analyse the evolution and clinical management of a cohort of patients with IIM.MethodsObservational, longitudinal, ambispective and multicentre study of a cohort of patients with IIM seen in rheumatology units in Spain. All patients with a diagnosis of IMM will be included in the regular follow-up of the participating centres, regardless of age on initiation of the process. Incident cases will be all patients who at the beginning of the study have been diagnosed for less than 12 months and prevalent cases for more than 12 months. The registry will include data from the visit at baseline, one year and two years. Socio-demographic, clinical, analytical variables, complications, comorbidities, association with other rheumatic diseases, hospital admissions, mortality and treatments will be collected. In addition, indices, scales and questionnaires of activity, muscle involvement, damage, disability, and quality of life will be determined. The recruitment period will be 23 months. The purpose is to obtain a cohort of 400 patients with IMM.ConclusionsMyo-Spain registry provides the opportunity to develop a cohort of incident and prevalent patients with IMM in Spain. Myo-Spain will be able to assess in detail the clinical characteristics of the disease at different times. The comprehensive information collected during the visits is expected to provide a broad source of data for future analysis.     

以肌损害为主要表现的原发性甲状腺功能减退症五例

目的提高对重症原发性甲状腺功能减退症伴随肌病患者临床特征、病变特点及其治疗的认识。方法回顾性分析1996年3月至2003年11月收治的5例重症原发性甲状腺功能减退伴肌损害患者的临床资料及治疗转归,并结合相关文献对该病的临床特点进行分析。结果5例患者突出的临床表现为对称性近端肌无力、气短、胸痛、呼吸及吞咽困难。血肌酸激酶均显著增高(2 885~10 450 U/L)。结论应将伴有肌损害的原发性甲状腺功能减退症患者与多发性肌炎鉴别,及时给予甲状腺激素替代治疗对甲状腺功能减退相关性肌病有较好疗效。     

Association of polymyositis with rheumatoid arthritis

The association of polymyositis (PM) and rheumatoid arthritis (RA) is described in a 40-year-old female Mexican patient who was studied for a long period of time. The characteristic changes of PM that preceded the onset of RA for 7 years included proximal symmetrical muscle weakness, increased creatine kinase activity, and distinctive electromyography and muscle biopsy results. The occurrence of RA during the final 4 years of the 11-year period was characterized by long-lasting deforming and symmetric polyarthritis, high positive rheumatoid factor, subcutaneous nodules, and erosive joint changes. Through observation, myopathic changes other than those from PM were excluded. Joint changes other than from RA were also ruled out. A review of the literature revealed few specific reports of the coexistence of both diseases.     

High single-dose alternate-day corticosteroid regimens in treatment of polymyositis

Summary High single-dose alternate-day prednisolone therapy (ADT) was compared with daily-dose prednisolone therapy (DDT) for treatment of polymyositis. Thirty patients with polymositis were treated with ADT for an average of 33.9 months. The combined number of improvements was 21 out of 30, a response rate of 70%. Side-effects were very rare and mild. On the other hand, 9 of 17 patients treated with DDT for an average 18.5 months improved, a response rate of 53%. The incidence of side-effects was strikingly higher than with ADT. ADT is therefore strongly advocated for treatment of polymyositis to avoid infectious complications and lessen cushingoid side-effects.     

Generalisierte Calcinosis cutis Die Suche nach der ursächlichen Autoimmunerkrankung

Wir berichten über eine 53-j?hrige Patientin mit einer schwergradigen generalisierten Calcinosis cutis bei seit 1982 bekannter systemischer Autoimmunerkrankung. Aufgrund der klinischen Trias Myositis, Lungenfibrose und Arthritis muss letztlich bei positiv nachweisbaren Antik?rpern gegen Jo-1 vom Vorliegen eines Jo-1-Syndroms ausgegangen werden.     

Glycogenosis Type V (McArdle’s Disease) Mimicking Atypical Myositis

A 13-year-old girl was referred to our clinic because of a positive rheumatoid factor test, muscle pain and weakness. Laboratory evaluation revealed an increased ESR, hypergammaglobulinaemia, antinuclear antibodies, circulating immune complexes, complement consumption and elevated serum creatine kinase (CK) activity. A needle biopsy of the dolent muscle showed normal routine histology. Immunohistochemistry disclosed single lymphocytes and a weak myocytic HLA class I expression. The diagnosis of myositis was considered and corticosteroids were initiated, leading to an increase of complement levels and a decrease of CK-activity and ESR. She subjectively felt stronger but still reported exercise intolerance and metabolic myopathy was considered. Myophosphorylase activity was completely lacking, establishing the diagnosis of McArdle's disease. CK level was found to be elevated in an obese 4-year-old brother too, who refused extensive walking but reported no muscle pain. Myophosphorylase deficiency was demonstrated by histochemistry and by biochemical analysis of his muscle. The female case illustrates that in children with the clinical picture of inflammatory myopathy and serological but not clinical response to therapy underlying metabolic muscle disorders should be excluded. Since the pathogenesis of polymyositis remains unclear, we speculate that inflammatory changes observed in the muscles may have been initiated by muscular damage resulting from the underlying metabolic disease. The serological changes remained unexplained and may contribute to a so far undeterminable connective tissue disease.     

205例多发性肌炎和皮肌炎临床分析

对２０５例ＰＭ／ＤＭ的临床分析表明：ＰＭ／ＤＭ除以肌无力为首发症状外,尚可以发热、呼吸困难、吞咽困难、雷诺征、心肌炎、关节炎及肿瘤就诊。ＰＭ／ＤＭ内脏受损部位有：心脏３６．５％（５４／１４８）;肺部２６．３％（５４／２０５）（其中肺间质病变９．０％,肺纤维化５．０％）;消化系２７．８％（５７／２０５）;血液系１５．５％（３２／２０５）;神经系９．７％（２０／２０５）,几乎无肾损害。在诊断ＰＭ／ＤＭ     

实验性肌炎动物模型制作的研究

目的 研究制作实验性肌炎动物模型的方法。方法 利用家兔骨骼肌匀浆加佛氏佐剂多次免疫豚鼠制成实验性肌炎模型，观察其在肌酶、肌电图（ＥＭＧ）、病理的改变。并与人类多发性肌炎（ＰＭ）作比较。结果 发现其与人类多发性肌炎（ＰＭ）在肌酶、肌电图（ＥＭＧ）、病理上的改变有相似之处。结论 提示其可作为研究人类ＰＭ的一个重要手段，为人类肌炎的发病机理及治疗提供理论依据。     

结缔组织病肌筋膜炎的MR和CT表现与穿刺活检的对照研究

目的 了解结缔组织病肌筋膜病变的影像表现特点，以及MRI、CT的应用价值。方法 经临床诊断的结缔组织病22例，其中皮肌炎（DM）8例、多肌炎（PM）12例和嗜酸性筋膜炎2例。所有病例均完成大腿CT平扫、MR的T1WI、T2WI、频率预饱和反转恢复（SPIR）序列及CT导向经皮穿刺活组织检查。结果 穿刺活检、MRI和CT发现肌组织病变分别有17例、14例和5例，发现筋膜病变分别为5例、9例和9例。肌炎、肌萎缩、筋膜炎可单独或合并存在，肌炎（9例）呈现T1WI低信号和T2WI或SPIR高信号改变，肌萎缩（9例）呈T1WI、T2WI高信号以及肌间隙增宽。SPIR检出肌炎明显优于CT和T1WI，P＜0．05。DM（6／8）的发生率明显高于PM（3／12），P＜0．05。病情加重期肌炎（7／11）的发生率明显高于缓解期（2／11），P＜0．05。结论 MRI和CT能够客观地反映结缔组织病肌筋膜病变的类型、累及部位和病情变化特点。