Renal disorders in patients with collagen vascular diseases

Membranous nephropathy, mesangial proliferative glomerulonephritis and renal amyloidosis are common renal pathology in RA patients. However, IgA nephropathy and diffuse thinning of glomerular basement membrane are described as common and characteristic renal lesions in Japanese RA patients. Glomerular filtration rate may decrease significantly in active lupus nephritis, but renal plasma flow does not change or even increase. These findings seem to be characteristic of SLE patients with active renal disorders. Therefore, filtration fraction may be a useful clinical parameter to evaluate SLE patients. Scleroderma renal crisis(SRC) has been believed to be the most serious renal disorder in systemic sclerosis (SSc). Recently, the presence of an antibody to RNA polymerase has been associated with a high prevalence of SRC.     

Pulmonary lesion associated with collagen vascular diseases: interstitial pneumonia

Various pulmonary lesions can be found in patients with collagen vascular diseases. Not only the diseases but also treatment with various drugs relate to an occurrence of the pulmonary lesions such as infection, drug reaction, bronchiolitis and interstitial pneumonias. We focused on interstitial pneumonias based on our own experiences with patients whose lesions were diagnosed by surgical lung biopsy. Incidence of the interstitial pneumonias and diagnostic problems before biopsy using HRCT and BAL were evaluated. Comparison of interstitial pneumonias between idiopathic and associated with collagen vascular diseases is a problem to be solved.     

血浆置换治疗在儿科危重患者抢救中的经验

目的探讨血浆置换治疗在儿科危重症患者的应用价值和治疗方案。方法应用GAMBR0-PRISMA床旁血滤机和TPE2000膜式血浆分离器对15例危重症患儿（1岁10月～15岁，平均6．8岁）进行39次血浆置换治疗；以新鲜冰冻血浆作置换液，置换量为40-70ml／（kg·次），血泵速度为50～120ml／min，治疗时间2～3h／次：结果39次血浆置换治疗均顺利成功实施，无明显并发症出现；14例在治疗后临床症状及生化指标好转，5例痊愈。结论血浆置换可以应用于多种危重症儿科疾病，治疗方案需根据病情制定。     

Miller Fisher syndrome with central involvement: successful treatment with plasmapheresis.

Miller Fisher syndrome is characterized by external ophthalmoplegia, ataxia, and areflexia. Most researchers favor a peripheral origin while others suggest a brainstem inflammatory lesion or a combination of central and peripheral demyelination. We report 2 cases of Miller Fisher syndrome with the typical triad of ataxia, areflexia, and ophthalmoplegia. Strong clinical evidence of central involvement included initial drowsiness, bilateral Babinski sign, and quadriparesis. Evoked potential studies showed prolongation of central conduction time. Plasmapheresis was performed to relieve respiratory failure in Patient 1 and to shorten the duration of nasogastric tube feeding due to severe bulbar palsy in Patient 2. Significant improvement of electrophysiologic parameters was recorded after plasmapheresis. Abnormal evoked potentials, together with clinical evidence of central nervous system abnormalities, support the hypothesis that there is a combination of peripheral and central involvement in Miller Fisher syndrome in our patients. Plasmapheresis is highly effective in relieving the profound neurological deficits of this atypical syndrome.     

Prosthetic valve endocarditis caused by Bartonella quintana in a patient during immunosuppressive therapies for collagen vascular diseases

Bartonella quintana, known to cause various clinical symptoms, is increasingly recognized as one important cause of culture-negative endocarditis. We report a case of infectious endocarditis with B. quintana on the prosthetic valve, accompanied by proteinase 3-antineutrophil cytoplasmic antibody-positive collagen vascular disease-like symptoms 1 year earlier.     

Outcome after distal vascular reconstruction in women.

Although women are less likely to develop symptomatic atherosclerosis, little data exists on the long-term outcome of women undergoing infrainguinal arterial reconstructions. This study analyzes the operative mortality rate, complications, and the short-term and long-term results after these procedures in both men and women who had symptomatic vascular disease. From 1984 to 1997, 3956 infrainguinal arterial reconstructions were performed at Albany Medical Center. A total of 2474 (62.54%) reconstructions were performed in men and 1482 (37.46%) in women. The mean ages were 67 years for men and 71 years for women. Forty-three percent of the men were smokers compared with 26% of the women. Diabetes was present in 51% of the men and 55% of the women. Claudication was the indication for bypass in 298 (12.05%) men and 110 (7.42%) women. Limb salvage occurred in 2176 (87.95%) men and 1372 (92.58%) women. Perioperative 30-day patency rates were 96.66% in men and 96.50% in women. Primary patency rates at 1, 3, 5, and 10 years were compared; no significant difference existed between men and women. Secondary patency also was similar in both men and women. These results indicate that women requiring arterial reconstruction for infrainguinal occlusive disease had short-term and long-term graft patency results that were comparable with the results of men.     

Double filtration plasmapheresis in the treatment of inflammatory polyneuropathy.

Twelve cases of recognized inflammatory polyneuropathy were treated by plasmapheresis (PP) at Shin-Kong Wu Ho-Su Memorial Hospital from November 1993 to November 1995. These include 6 cases of acute inflammatory demyelinating polyneuropathy (AIDP), 4 cases of chronic inflammatory demyelinating polyneuropathy (CIDP), one case of Fisher syndrome, and one case of Sj?gren's syndrome with polyneuropathy. The patients chosen for PP met the inclusion criteria of severely disabled, i.e., Grade 4 (bed or chair bound) or Grade 5 (required assisted ventilation), or an unremittingly progressive course. Plasmapheresis was carried out by the double filtration method. Each patient received a standard course of at least 4 sessions of pheresis. All the patients receiving PP showed a beneficial response. The treatment for 10 patients was judged to be effective. The other 2 patients (one AIDP and one CIDP), though improved, were unable to reach Grade 2. Among the complications of PP, only 4 episodes of symptomatic hypotension were noted in a total of 73 sessions of PP. In conclusion, PP is safe and effective in treating inflammatory polyneuropathy.     

Immunomodulation during treatment of polymyositis with plasmapheresis and immunosuppressive drugs

Immunologic studies were carried out in a patient with polymyositis (PM), who showed increasing muscle strength and decreasing serum creatine phosphokinase levels during 20 weeks of treatment with plasmapheresis in conjunction with prednisone and cyclophosphamide. After an initial rise, serum IgG declined with treatment. Natural killer (NK) lymphocytes were reduced by 74%, B cells by 95%, and T cells by 38%. Spontaneous proliferation of peripheral blood mononuclear cells increased dramatically. Within the CD4+ T cell subset there was increasing maturation as shown by a rise in percent mature (CD29+) cells and reciprocal decline of immature (CD45RA+) cells. At the same time CD4+ T cells became increasingly activated as shown by HLA-DR expression. The percentage of CD8+ T cells increased strongly with treatment, and they showed increased activation and expression of the cytotoxic CD29+ and CD11b- phenotypes. CD8+ T cells exhibiting CD45RA or CD11b+ suppressor phenotypes were overall unchanged; however, on follow-up a proportion of CD8+ cells expressed the activated suppressor effector (CD11b—CD28—) phenotype. In addition to control of PM by the possible deletion of activated autoreactive B and T lymphocyte clones with cyclophosphamide, the activation and maturation of CD4+ T cells during treatment may have downregulated the autoreactive disease process, either through direct antiidiotypic suppression or by induction of the observed increase in cytotoxic and suppressor CD8+ T cells. © 1994 Wiley-Liss, Inc.     

Kell hemolytic disease of the fetus. Combination treatment with plasmapheresis and intrauterine blood transfusion

We report the case of a 36-year old pregnant woman with a Kell alloimmunization (anti-K1), probably secondary to a previous blood transfusion, and a severe hemolytic disease of the fetus. Once the first fetal blood transfusion by cordocentesis was performed, we started treatment with repeated plasmapheresis to maintain anti-K1 titer below 1:32. With this scheme we did not need to perform a second intrauterine fetal blood transfusion and only mild anemia was found in the newborn. Taking into account that the rate of serious complications with plasmapheresis is lower than that related with intrauterine blood transfusion, this could be an alternative approach to repeated transfusions.     

The treatment potentials of plasmapheresis in glomerulonephritis

Plasmapheresis (PA) was used in the treatment of 26 patients with primary glomerulonephritis (GN) and in 12 patients suffering from secondary GN. In spite of large volumes of plasma exfusions and their high rate, the authors did not notice that PA produced a beneficial effect on the main symptoms of GN. The clinical effect due to PA was attained in one patient with pseudo-tuberculous interstitial nephritis. Some parameters of homeostasis considerably improved and/or returned to normal for a brief period. The initial refractory nature of GN as regards the immunotropic agents and/or its hormonal dependence did not reduce after PA. It has been shown that replenishment of the removed protein within the range of 10 to 50% does not interfere with the reserve potentialities of restorative protein synthesis in GN patients.     

胸痛相关肺血管病的诊治进展

胸痛是临床常见症状之一,本文针对以胸痛特点为临床表现的肺血管疾病进展予以综述,尤其是致死性肺栓塞的危险因素、临床表现、诊疗及预防等方面的进展,提高对肺栓塞的认识水平,为临床肺血管病的诊治提供参考,并提出了围绕肺血管病的诊治管理模式。     

双重血浆滤过治疗脂蛋白肾病的护理

目的探讨双重血浆滤过(DFPP)治疗脂蛋白肾病(LPG)的临床护理要点。方法选择LPG患者12例行DFPP治疗,共治疗53例次,并对患者实施以保证治疗安全、提高治疗效果为目的整体护理。结果所有患者均顺利完成治疗,经治疗后免疫学指标均明显下降,无1例发生低血压、导管感染、溶血、过敏等并发症。结论 DFPP治疗LPG效果显著,优质的专科护理技术是保证治疗安全有效完成的关键。