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11.

Objective

Interstitial lung disease (ILD) is the most severe complication of idiopathic inflammatory myositis (IIM), resulting in significant increase in morbidity and mortality and for which the best treatment remains controversial. We conducted a meta-analysis to evaluate the efficacy of therapies used for the management of IIM-related ILD.

Methods

Studies were selected from MEDLINE up to July 2017. Two investigators independently extracted data on study design, patient characteristics, clinical features, treatment, follow-up and outcomes. Global survival rates and objectively confirmed lung function improvements were extracted as the main outcome for rapidly progressive IIM-related ILD (RP-ILD) and chronic forms of ILD (C-ILD), respectively, and pooled using the weighted mean proportion with fixed or random-effects models in case of significant heterogeneity (I2?>?50%).

Results

Twenty-seven studies encompassing 553 patients (male: 30.5%, age: 53.5?±?5.5?years) were included in the meta-analysis. Globally, retrieved studies were of limited methodological quality (no controlled studies and only 2 prospective studies). Dermatomyositis (40%) and anti-tRNA synthetase syndrome (45%) were the most represented IIM subtypes. In C-ILD, functional improvement rates were 89.2% (95%CI 82.5–93.6; 7 studies, n?=?124) for corticosteroids alone, 80.7% (95%CI 49.6–94; 6 studies, n?=?38) for cyclosporine A, 64.1% (95%CI 46.3–78.7; 4 studies, n?=?32) for azathioprine, 86.2% (95%CI 61.5–96; 2 studies, n?=?23) for tacrolimus, 56.4% (95%CI 44–68.0; 8 studies, n?=?71) for cyclophosphamide, and 76.6% (95%CI 50.4–96.0; 2 studies, n?=?20) for rituximab. In RP-ILD, survival rates at 3?months were 51.7% (95%CI 24.2–78.1; 2 studies, n?=?11) for corticosteroids alone, 69.2% (95%CI 55.0–80.5; 8 studies, n?=?146) for cyclosporine A and 72.4% (95%CI 6.4–99.0, 2 studies, n?=?16) for cyclophosphamide.

Conclusion

Despite aggressive immunosuppressive therapies, the short-term mortality of RP-ILD remains high. While immunosuppressive therapies are associated with significant functional improvements in most patients with C-ILD, substantial uncertainty remains about the best treatment strategy in the absence of good quality evidence.  相似文献   
12.
Summary Conditioning therapy with aggressive chemotherapy and irradiation induces a state of transient combined immunodeficiency in bonemarrow transplant recipients. This promotes the occurrence of severe cytomegalovirus (CMV) infections, the most frequent lethal complication after bone-marrow transplantation (BMT) at present.Forty-four BMT recipients received CMV-IgG-hyperimmunoglobulin for CMV prophylaxis intravenously. The efficacy of this prophylaxis and possible risk factors for the occurrence of CMV-induced interstitial pneumonia (IP) were analyzed. Risk factors for the promotion of a CMV-IP were: additional immunosuppressive therapy after BMT, CMV-positive serostatus of the recipient, CMV-seropositive granulocyte transfusion, CMV infection immediately prior to BMT, and HLA-haploidentical BMT. In this study the incidence of graftversus-host disease was low and was not associated with the incidence of CMV infections. The use of T-cell-depleted grafts did not result in increased CMV infections or IP and may possibly have improved the immunological reconstitution.Abbreviations BMT Bone-marrow transplantation - CMV Cytomegalovirus - CMV-IG CMV-IgG-hyperimmunoglobulin - GvHD Graft-versus-host disease - IP Interstitial pneumonia - IS Immunosuppressive therapy  相似文献   
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15.
100例系统性红斑狼疮患者的肺间质病变初探   总被引:13,自引:0,他引:13  
目的 调查系统性红斑狼疮 (SLE)患者肺间质病变 (ILD)的发生率及临床表现 ,并根据临床、影像学及肺功能检查制定新的分型方法。方法 随机对 10 0例SLE患者进行胸片 ,高分辨CT扫描 (HRCT) ,肺功能和动脉血氧分压的同步测定。结果 HRCT诊断SLE患者的ILD阳性率为 77% (77/10 0 ) ,其敏感性高于胸片(38% )。 77例HRCT有ILD者肺功能和动脉血氧分压异常 ,分别为 35 .1%和 6 %。提出SLE合并ILD的临床分型新方法。分 3型 :临床前期型 ,急性肺泡炎型和慢性肺间质病变型。 77例HRCT阳性者分型分别为 92 .2 %(71/77) ,2 .6 % (2 /77) ,5 .2 % (4/77)。结论 SLE患者进行肺HRCT的检查 ,结合肺功能检查能发现早期ILD。  相似文献   
16.
Balloon angioplasty is a standard treatment for artherosclerotic coronary artery disease. However, its clinical value is reduced by a high restenosis rate. A new concept in preventing restenosis is the use of a liquid-filled balloon containing a beta-emitting radioisotope. In this study, we performed biodistribution studies of Re-188 perrhenate and Re-188 diethylenetriaminopentaacetate (DTPA) to assess the resulting organ dose values in the event of balloon rupture if these agents are used for the clinical inhibition of restenosis after percutaneous transluminal coronary angioplasty (PTCA). After injecting Re-188 preparations intravenously, rats were killed at 10 min, 30 min, 60 min, 2 h, and 6 h ( n =5 per group). Tissue concentrations were calculated and expressed as percent injected dose per gram or per milliliter (%ID/g or %ID/mL). In addition, urine excretion and thyroid gland uptake were evaluated in rats ( n=5 per group) with a gamma camera after administration of 37 MBq (1 mCi) of each agent. Our data showed that both agents were excreted primarily via urine. However, the excretion of Re-188 DTPA was much faster than that of Re-188 perrhenate via the urinary system. The biodistribution data revealed that radioactivity levels in the stomach and the thyroid gland were high in the perrhenate group but low in the Re-188 DTPA group. The concentration levels in other tissues including lung, liver, testis, muscle, and blood were low throughout this study for both agents. The thyroid radiation value in the Re-188 perrhenate group was 0.163 mGy/MBq, which was much higher than that of the Re-188 DTPA group (0.0167 mGy/MBq). The stomach radiation value was as high as 0.127 mGy/MBq for Re-188 perrhenate, compared with 0.013 mGy/MBq for Re-188 DTPA. In conclusion, in the event of balloon rupture, the release of Re-188 DTPA results in lower radiation doses than Re-188 perrhenate, especially to the thyroid gland and the stomach. Our data suggest that Re-188 DTPA is a useful radiopharmaceutical for endovascular irradiation.  相似文献   
17.
Mumps is still a common childhood disease in rural areas where mumps vaccination is not widespread. A 14-year-old girl with fatal interstitial nephritis as a complication of mumps is reported. The patient had not been vaccinated with mumps vaccine and had contracted mumps during a village epidemic. The illness began with parotitis, and renal insufficiency developed within a week. The patient’s renal function rapidly deteriorated and the outcome was fatal. The postmortem renal necropsy sample demonstrated interstitial mononuclear cell infiltration, edema, and focal tubular epithelial cell damage, confirming the clinical diagnosis. In developing countries routine mumps vaccination may help to prevent possible fatal complications of mumps. Furthermore, patients with mumps, especially complicated cases, should be followed closely. Received: 19 August 1998 / Revised: 7 December 1998 / Accepted: 8 December 1998  相似文献   
18.
This review highlights characteristics of extracellular fluid (ECF) that are often overlooked. ECF has, in addition to plasma and interstitial fluid (ISF) surrounding cells, a third large compartment, the ISF of skin and connective tissue. This acts as a reservoir that gives up ECF to plasma volume (PV) in order to sustain circulation in the event of either shock or dehydration. While Starling forces drive filtration, ECF is returned to PV more by lymph and less by Starling forces than previously appreciated. Lymph return to PV is dependent on physical activity and muscle contraction to overcome gravity. Regional change in metabolic rate alters the need for oxygen and nutrients that is met by a regional increase in capillary blood flow. Blood flow is controlled by vasoactive compounds released in response to a drop in PO2; these relax capillary smooth muscle to increase blood flow and delivery of oxygen and nutrients. Plasma proteins, including albumin, are filtered into the interstitium through larger pores than those filtering ECF. The rate of protein filtration is set by size and charge of these larger endothelial pores and by size and charge of proteins. Charge of these pores, hence albumin permeability, is regulated by many of the same vasoactive compounds that control capillary flow. As a consequence, in response to gravitational stress and other forms of shock that reduce effective circulation, albumin as well as ECF is rapidly shifted from plasma and sequestered in ISF. When this has occurred, as in burn shock, restoration is better effected by generous expansion of ECF with Ringer’s solution alone, rather than with Ringer’s solution supplemented with human serum albumin or other colloid. Restoring both PV and ISF volume restores lymph circulation and returns sequestered albumin to PV. Received: 12 November 1998 / Revised: 30 March 1999 / Accepted: 2 April 1999  相似文献   
19.
通过对近10年来相关文献的整理和分析,显示中医药对间质性肺病(ILD)防治的研究进展。目前中医学对ILD病症、病因病机以及临床治疗的认识和研究都有了显著的提高,无率是单味中药还是组方用药,其疗效优于单用西药。在延长生存期、改善生存质量、缓解临床症状等方面效果尤为明显。  相似文献   
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