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61.
Abstract: Four patients with chronic, progressive demyelinating peripheral polyneuropathy were found to have paraproteinemia. Two of the patients had multiple myeloma [both immunoglobulin (Ig)G lambda]. The other two had benign gammopathies: one of IgG kappa type and the other one with marked polyclonal elevation of IgM. Immunofluorescence studies revealed deposits of the abnormal serum immunoglobulin along the myelin sheaths in two of the patients in whom sural nerve biopsies were performed. All four patients were treated with plasmapheresis in combination with immunosuppressive drugs. Favorable responses to the therapy was observed in all four patients, but the degree of response varied from patient to patient. Two patients who presented clinical and electromyographic findings consistent with polymyositis were found to have serum IgG kappa M components. Immunofluorescence studies performed on muscle biopsy material from both patients revealed deposits of the abnormal serum paraprotein along the sarcolemmal basement membrane. A treatment course of plasmapheresis and immunosuppressive drugs resulted in a sustained increase of muscle strength in both patients. 相似文献
62.
S. Currie 《Acta neuropathologica》1970,15(1):11-19
Summary 80% of cultures of foetal human and rat muscle underwent destruction when incubated with lymphocytes from patients with polymyositis compared with 30% of those incubated with serum from patients with polymyositis and 25% of those incubated with lymphocytes from patients with other neurological disorders. Cultures of dural fibroblasts were also destroyed in the presence of lymphocytes from patients suffering from polymyositis associated with collagen disorders. These effects may be the sequel to a specific sensitization of lymphocytesin vivo and the findings are therefore consistent with a mechanism of cellmediated hypersensitivity underlying polymyositis.The work was aided by grants from the Medical Research Council, the Muscular Dystrophy Association of America, Inc., and the Muscular Dystrophy Group of Great Britain. The author would like to thank Miss Aileen Brown for her advice and technical assistance, his co-workers. Dr. Michael Saunders and Mr. Malcolm Knowles of the M.R.C. Demyelinating Diseases Research Unit, the physicians of the region for allowing him to study their patients and Prof. John N. Walton for his advice and encouragement. 相似文献
63.
Search for autoantibodies to endothelial and smooth muscle cells in patients with multiple sclerosis
Summary The blood-brain barrier (BBB) is disrupted in many of the lesions of multiple sclerosis (MS). Immunologically mediated injury to one of the major components of this barrier, the cerebral capillary, may play a role in the development of the lesion. We therefore examined the sera of 51 cases of MS for the presence of autoantibodies to endothelial and smooth muscle cells, using the indirect immunofluorescent technique. The results were compared to those in other groups of patients with neuroimmunological disorders. We found no anti-endothelial cell antibodies, but autoantibodies to vascular smooth muscle were detectable in 31% of the MS sera tested. They were also present, however, in 30% of sera from cases of myasthenia gravis and in the serum of one of 12 cases of polymyositis. It is considered to be unlikely that antibodies to vascular tissues play any pathogenetic role in multiple sclerosis.Supported by the Epidemic Myalgic Encephalomyelitis Association and the Multiple Sclerosis Society of Great Britain 相似文献
64.
65.
Sae Ochi Ken Taniguchi Masakazu Nagashima 《Modern rheumatology / the Japan Rheumatism Association》2009,19(4):443-446
Although rheumatoid arthritis (RA) and myositis are major autoimmune diseases, co-occurrence of the two is rare. We treated
a patient who developed polymyositis (PM) following the treatment of RA with leflunomide. Prednisolone (PSL) in combination
with methotrexate (MTX) was effective in managing the PM, but the RA relapsed during the treatment. Based on the clinical
course, we suspect that the PM was induced by the leflunomide treatment and suggest that clinicians should consider the possibility
of this rare adverse event in cases of cholestyramine-resistant elevation of transaminases. 相似文献
66.
We describe a patient who presented with polymyositis with anti-Jo-1 antibodies at 18 years after the onset of rheumatoid
arthritis and was successfully treated with the immunosuppressive drug mizoribine at the time of exacerbation. She had developed
diabetes mellitus, cerebral infarction, and myocardial infarction after high-dose steroid therapy was initiated. Therefore,
an immunosuppressant was preferred as the second-line agent. Treatment with 150 mg/day of mizoribine and 8 mg/day of prednisolone
resulted in eventual normalization of muscle enzyme levels. Mizoribine is a purine antimetabolite that inhibits T cell activation/proliferation
and B cell proliferation. The potential efficacy of mizoribine for polymyositis was suggested by this case. 相似文献
67.
Pregnancy is an important condition that can affect and be affected by rheumatic disease. Overall, pregnancy is viewed as
a Th2-predominant state, but several Th1-related cytokines are vital to early pregnancy. In rheumatoid arthritis for example,
the majority of women improve by the beginning of the second trimester, but the majority (90%) will flare in the first 3 to
4 months postpartum. In contrast, systemic lupus erythematosus has an unpredictable course in pregnancy, leaving most rheumatologists
to recommend a disease-quiescent state prior to conception. Other diseases such as scleroderma are less clear because the
disease less commonly presents in the childbearing period. Many immunosuppressive medications for the rheumatic diseases are
contraindicated in pregnancy because of their mechanisms of action leaving only a select few “safe” medications. Significant
heterogeneity between the Food and Drug Administration (FDA) category for a medication and what a rheumatologist does in clinic
leads to confusion on how a patient should be treated for active rheumatic disease both peripartum and postpartum, particularly
if the patient is breastfeeding. We review the general state of pregnancy and how it is affected by prototypical rheumatic
diseases including rheumatoid arthritis and systemic lupus erythematosus. In addition, we present the most commonly used disease-modifying
antirheumatic drugs and immunosuppressants and explain the difference between the FDA category and clinical practice among
rheumatologists. Finally, we provide some general recommendations on how to manage a rheumatic disease during pregnancy including:
(a) preconception planning to ensure no teratogenic medications on board, (b) early disclosure of pregnancy to all caregivers
including the rheumatologist, family physician, obstetrician, and maternal–fetal medicine specialist, and (c) planning of
safe medication use for acute flare-ups and disease suppression peripartum and postpartum.
Presented as Medical Grand Rounds at the University of Alberta. 相似文献
68.
目的探讨皮肌炎/多发性肌炎(DM/PM)合并肺间质病变(ILD)的临床特征和预后。方法回顾住院诊治及门诊长期随访的141例DM/PM患者的临床资料及实验室指标,对PM/DM合并ILD的生存时间、预测因素和预后不良因素进行分析。结果141例DM/PM患者中合并ILD 42例(29.8%),其1、5、10年生存率分别为76%、66%和57%,中位生存时间为127.3个月。多因素Cox回归分析得出:合并ILD、年龄>50岁、声音嘶哑、低白蛋白血症、低氧血症是DM/PM预后不良的独立危险因素(均P<0.05)。logistic回归显示,低白蛋白血症、技工手和抗Jo-1抗体与ILD呈正相关(均P<0.05),成为ILD相关预测因子。急性/亚急性ILD进展快,病死率高(75%)。丙种球蛋白治疗与病死率呈负相关(P<0.05)。结论 ILD是DM/PM的常见并发症,也是影响预后的危险因素。技工手、抗Jo-1抗体和低白蛋白血症是DM/PM合并ILD的预测因子,低氧血症是影响ILD预后的不良因素,早期使用丙种球蛋白冲击治疗有助改善预后。 相似文献
69.
目的研究活动期多发性肌炎患者外周血白细胞细胞因子信号转导蛋白抑制因子(SOCS)1、SOCS2、SOCS3和细胞因子诱导的含SH2区域蛋白1(CIS)与正常人表达的差异,探讨SOCS在多发性肌炎发病中可能的作用。方法 2011年6月-12月,采用实时荧光定量聚合酶链反应法检测了14例活动期多发性肌炎患者和14例正常人外周血白细胞中SOCS1、SOCS2、SOCS3和CIS1基因的相对表达量。结果与对照组相比,多发性肌炎症患者外周血白细胞基因SOCS 1~3表达明显降低(P值均<0.05),CIS1基因的表达较对照组明显升高(P<0.05),差异有统计学意义。结论 SOCS基因家族可能参与了多发性肌炎的发病,该蛋白分子家族的成员可能会成为多发性肌炎治疗的一种新的候选基因。 相似文献
70.
《Seminars in arthritis and rheumatism》2019,48(6):865-869
ObjectiveThe idiopathic inflammatory myopathies dermatomyositis (DM) and polymyositis (PM) are autoimmune diseases that can affect females of childbearing potential. We assessed pregnancy outcomes in DM and PM patients compared with the general obstetric population.MethodsThe Nationwide Inpatient Sample (NIS) (1993–2007) was used to identify delivery-associated hospitalizations in women with DM or PM (DM/PM, n = 853). Controls were from the general obstetric population delivery-associated hospitalizations matched to each case by year of delivery. Pregnancy outcomes included hospital length of stay (LOS), hypertensive disorders (HTN), premature rupture of membranes (PROM), intrauterine growth restriction (IUGR), and cesarean delivery. Multivariate regression analyses were performed using maternal age, race/ethnicity, and diabetes mellitus as covariates.ResultsOn multivariate analysis, patients with DM/PM had longer LOS compared to controls (p < 0.001). DM/PM was associated with an increased risk of hypertensive disorders compared to controls (OR = 2.90, 95% CI: 2.00–4.22). There were no differences in rates of PROM, IUGR, or cesarean section in patients with DM/PM compared with controls. Independent of a DM/PM diagnosis, African-American race, older age, and diagnosis of diabetes increased the hospital LOS (p < 0.001). African-American race and diabetes increased the risk of hypertensive disorders (OR = 1.38, 95% CI: 1.19–1.60; OR = 2.94, 95% CI: 2.04–4.23, respectively) compared to controls.ConclusionThese data suggest that patients with inflammatory myopathies are at increased risk of hypertensive disorders of pregnancy and longer length of hospitalization. Vigilant monitoring of blood pressure is advisable in pregnant patients with DM or PM. 相似文献