皮肌炎与多发性肌炎临床表现和实验室检查对比分析

目的比较皮肌炎(DM)和多发性肌炎(PM)临床表现和实验室检查的异同。方法回顾性分析2012年1月1日至2015年10月31日中国医科大学附属第一医院风湿免疫科收治的62例DM和29例PM患者的临床资料,分析其临床特征的异同。并比较伴有间质性肺病(ILD)的DM和PM患者实验室检查的异同。结果乏力、肌痛、吞咽困难、发热等症状的发生频率在DM和PM组间差异均无统计学意义。DM组外周血淋巴细胞计数为(1.13±0.48)×10~9/L,显著少于PM组的(1.78±0.90)×10~9/L(P=0.01)。DM患者CD3~+T细胞数[(612±332)个/μL对(1543±945)个/μL,P=0.01]、CD4~+T细胞数[(370±175)个/μL对(741±450)个/μL,P=0.02]、CD8+T细胞数[(219±178)个/μL对(738±644)/μL,P=0.02]均显著少于PM组。PM患者CD4+T/CD8+T比值为(2.6±1.5),显著高于DM患者的(1.5±1.0)(P=0.03)。与PM患者相比,DM患者血清白蛋白水平[(34.2±4.7)g/L对(38.1±5.5)g/L,P=0.01]明显降低。伴有ILD的DM(DM-ILD)患者外周血淋巴细胞计数[(1.47±1.04)×10~9/L对(1.70±0.85)×10~9/L,P=0.04]、血清肌酸激酶水平显著低于伴有ILD的PM患者(PM-ILD)[(1220.8±2118.5)U/L对(3150.8±2965.9)U/L,P=0.02]。DM-ILD患者CD4~+T/CD8~+T比值为2.3±1.4,显著高于PM-ILD患者的1.4±0.4(P=0.00)。结论 DM和PM在临床症状上具有一定相似点,但外周血T细胞总数及不同亚群计数却存在显著差异,提示免疫失衡在DM患者的发病机制中具有重要作用。     

皮肌炎合并间质性肺疾病患者的临床特征分析

目的探讨皮肌炎(dermatomyositis,DM)合并间质性肺病(interstitial lung disease,ILD)患者的临床特点,以提高对DM-ILD的认识。方法搜集南京鼓楼医院自2006年6月至2013年6月期间临床确诊、资料完整的皮肌炎患者91例,对其中39例DM-ILD患者的临床特点及影像学检查进行分析。结果 91例患者中有间质性肺病者39例(42.8%),DM-ILD组发病年龄及病程与DM-无ILD组比较无明显差别(P0.05);DM-ILD组更常出现雷诺现象、关节痛、低热、咳嗽、咳痰及呼吸困难;DM-ILD组血沉(ESR)明显增快,C反应蛋白(CRP)明显增高,两组比较差异均有统计学意义(P0.01);抗核抗体(ANA)、乳酸脱氢酶(LDH)在两组间无明显差异,DM-ILD组抗Jo-1抗体阳性率增高;肺功能检查示DM-ILD组以限制性通气功能障碍及弥散功能障碍为主,DM-无ILD组肺功能正常;DM-ILD组胸部CT改变以磨玻璃影、条索状影及网格样改变最常见,胸腔积液少见;DM-ILD可增加死亡风险达2.3倍,感染是最常见的死亡原因。结论 ILD是DM常见的系统损害,DM-ILD的发生与疾病的活动及某些自身抗体有关,严重影响疾病预后,因此确诊DM的患者均应进一步明确肺部病变情况。     

多发性肌炎／皮肌炎肺间质病变的治疗及预后

多发性肌炎（polymyositis，PM）／皮肌炎（dermatomyositis，DM）是一种以近端骨骼肌受累为主的系统性结缔组织疾病，可以累及全身多个脏器，包括肺脏。间质性肺疾病（interstitial lung disease，ILD）是其常见的并发症，而且影响PM／DM的预后。检测技术的提高包括HRCT及肺功能等对诊断早期ILD提供了更敏感的工具。通过系统性使用这些检查手段，发现新近诊断的PM／DM有2／3的患者合并有ILD。     

以急进性肺间质病变为突出表现的无肌炎的皮肌炎

目的了解以急进性肺间质病变为突出表现的临床无肌炎的皮肌炎的临床特点转归和治疗。方法回顾分析本院1998—2005年住院的成人特发性皮肌炎/多发性肌炎(DM/PM)202例,按临床无肌炎的皮肌炎(CADM)、典型DM、PM进行分组,有无合并临床肺间质病变(ILD)进行分层。对随访的145例DM/PM行生存分析和COX回归,分析预后和危险因素。横断面研究用Logistic回归分析ILD相关的预测因子。结果①存在临床ILD是DM/PM预后的最重要危险因素,OR为4．826(95%CI为1．60～14．56．P=0．005)；而亚临床ILD与无ILD的DM/PM生存曲线差异无统计学意义。②CADM、典型DM、PM之合并临床ILD存在不同的病情经过。CADM-ILD呈急进性ILD的临床模式,其6个月存活率仅为40．8%,中位数存活时间仅为10．2个月；而典型的DM-ILD呈现“次急进性”(sub-rapid progressive)的临床模式,5年存活率为54．0%,中位数存活时间为90个月；PM-ILD则为慢性经过,5年和10年存活率分别为72．4%和60．3%,中位数存活时间可达10年,部分DM-ILD和个别PM-ILD患者也可呈现急进性ILD的疾病模式。③Logistic回归显示：四肢近端肌力OR 3．374(95%CI 1．729～6．583,P＜0．01)和血沉OR 1．025 (95%CI 1．002～1．049,P=0．032)与临床ILD呈正相关,血白蛋白水平OR 0．877(95? 0．792～0．970,P= 0．011)则与临床ILD呈负相关。④随访CADM(28例)的病程为3～80个月,其中CADM-ILD超过半数在1年内死亡(12/21)；而病程＞24个月的CADM(8/28例)预后良好。对于无ILD的CADM,主要予中小剂量的激素(＜0．5～1mg·kg~(-1)·d~(-1))治疗,而CADM-ILD则多接受较大剂量的激素(≥1～2mg·kg~(-1)·d~(-1)),且多联用包括硫唑嘌呤、环磷酰胺、环孢素、霉酚酸酯在内的细胞毒药物,但对呈急进性ILD者缺乏疗效。⑤DM/PM随访病例的病死率为24．8%(36/145例),ILD及其并发症作为直接死因的占69．4%(25/36例),与ILD无直接相关的感染占25．0%(9/36例)。纵隔(皮下)气肿,气胸发生率占所有DM/PM-ILD的9．3%(8/86例),也是提示预后不良指征之一。结论ILD是DM/PM的常见并发症,也是最重要危险因素。本文提出了一个新的DM/PM疾病分类模式：即DM/PM是皮损、肌炎和ILD三个维度上相组合的立体疾病谱。CADM是该疾病谱的组成部分,CADM-ILD可呈急进性,是疾病谱中特殊的临床表型。     

多发性肌炎/皮肌炎合并间质性肺疾病的临床分析

目的探讨多发性肌炎/皮肌炎(PM/DM)合并间质性肺疾病(ILD)患者的临床表现、实验室检查、胸部影像学及肺功能的变化及意义。方法对住院的PM/DM并ILD患者7例(男性5例,女性2例,平均年龄55.7岁;5例为DM,2例为PM)均进行血清酶学和自身抗体测定、高分辨率CT(HRCT)及肺功能检查和皮肤肌肉活检。结果 6例患者出现酶学增高,以肌酸激酶(CK)和乳酸脱氢酶(LDH)增高明显;4例患者抗核抗体(ANA)(+),2例抗Jo-1抗体(+);皮肤肌肉活检病理诊断符合肌炎、皮肌炎改变。HRCT提示4例肺出现网点影及斑片阴影,3例出现双肺磨玻璃样病变和实变阴影。7例患者均出现限制性通气功能障碍,一氧化碳弥散量(DLCO)明显降低。6例患者使用糖皮质激素和免疫抑制剂治疗。5例患者治疗后病情稳定,1例患者死亡。结论 HRCT可以及早发现PM/DM肺部病变。CK明显增高和抗Jo-1抗体阳性是诊断DM/PM并ILD的重要指标。患者可出现严重的弥散功能低下,应用激素合并免疫抑制剂治疗可取得较好疗效。     

铁蛋白/淋巴细胞比值在评估DM/PM-ILD严重程度中的诊断准确性研究

目的 探讨铁蛋白/淋巴细胞比值对皮肌炎/多肌炎合并间质性肺病(DM/PM-ILD)预后不良的预测价值。方法 回顾性收集2018年6月-2020年12月南昌大学第一附属医院风湿免疫科就诊具有完整临床资料的皮肌炎/多肌炎合并间质性肺病患者86例。根据HRCT和美国胸科学会和欧洲呼吸学会关于特发性肺纤维化的国际共识声明将患者分为缓解组(n=29)和加重组(n=57)。对两组的人口学特征,临床症状和实验室检查进行比较。通过计算患者ROC曲线,确定FLPR对DM/PM-ILD严重程度的诊断能力。结果 加重组患者ESR、CRP、SF、LDH、LLR、SII和FLPR显著高于缓解组患者(P<0.001)。多因素Logisitic回归分析得出,SF、LDH、低淋巴细胞、LLR、SII和FLPR是加重组DM/PM-ILD患者的独立危险因素。ROC曲线分析得出,FLPR曲线下面积为0.947,敏感度为0.649,特异度为1,界值为599。结论 铁蛋白/淋巴细胞比值是评估DM/PM-ILD患者严重程度的有效生物标志物,为DM/PM合并ILD患者提供了最佳的临界值,从而使诊断效率最大化。     

血清KL-6检测在特发性炎性肌病伴肺间质病变诊断中的价值

目的探讨血清KL-6检测在特发性炎性肌病(IIM)合并肺间质病变(ILD)诊断中的作用。方法多发性肌炎(PM)和皮肌炎(DM)患者53例,其中合并有ILD的22例,正常对照者50名及肺部感染患者22例。酶联免疫吸附试验(ELISA)检测血清KL-6浓度。分析PM/DM患者临床表现及预后与血清KL-6水平的相关性。结果血清KL-6浓度在合并ILD的PM/DM组、未合并ILD的PM/DM组、肺部感染组和正常对照组的平均值分别是(1543±761)、(429±106)、(336±196)和(289±105)U/ml。合并ILD的PM/DM组血清KL-6水平较未合并ILD的PM/DM组、肺部感染组和正常对照组均显著升高(P＜0．01)。而未合并ILD的PM/DM组、肺部感染组和正常对照组间差异无统计学意义(P＞0．05)。血清KL-6的升高与肺间质病变呈显著正相关(P＜0．01),KL-6诊断PM/DM合并ILD的敏感性为90．9%,特异性为80．6%。随访分析表明,6例死亡的PM/DM患者血清KL-6水平明显高于其他伴发ILD的PM/DM患者(P＜0．01)。结论血清KL-6浓度是PM/DM合并ILD特异和敏感的血清学指标,它可用来鉴别肺部感染和肺间质病变。高水平的血清KL-6浓度可能提示预后差。     

多发性肌炎/皮肌炎合并肺间质病变的临床特征和预后

目的 研究多发性肌炎(PM)和皮肌炎(DM)合并肺间质病变(ILD)的临床特点和预后.方法 回顾性分析107例PM/DM患者的临床资料,包括首发症状、临床表现、实验室检查、影像学资料、治疗及预后.结果 107例PM/DM患者合并ILD有28例,ILD发生率为26.2%.①合并ILD的首发症状为关节炎或关节痛者高于无ILD(P<0.05);合并ILD临床表现为关节炎或关节痛、发热、干咳气促者明显高于无ILD(P<0.05).②DM合并ILD患者大多有特异性皮疹,呼吸困难较重(P<0.05),而PM合并ILD患者肌痛、肌无力较重(P<0.05).③合并ILD的红细胞沉降率(ESR)和C反应蛋白(CRP)明显高于无ILD(P<0.05);DM-ILD组肌酶谱以羟丁酸脱氢酶(HBDH)、天冬氨酸转氨酶(AST)升高为主(P≤0.05),PM合并ILD患者肌酶谱以肌酸激酶(CK)和肌酸激酶同工酶(CK-MB)为主(P<0.05).④合并ILD的28例PM/DM患者经治疗,20例病情改善,8例重症7例均为DM合并ILD,5例治疗无效因Ⅰ型呼吸衰竭死亡(病死率占PM/DM合并ILD患者的17.9%).结论 ①首发症状为关节炎或关节痛,临床表现有关节炎或者关节痛、发热以及ESR和CRP高者易合并ILD;有特异性皮疹、AST升高的DM易合并ILD;肌酶以CK和CK-MB升高为主的PM易合并ILD.②DM合并ILD病情进展凶险,病死率高,预后不良.     

结缔组织病肺间质病变的临床特点及治疗效果分析

目的探讨不同结缔组织病肺间质病变（CTD-ILD）的临床特点和治疗效果。方法分析82例CTD-ILD患者的临床特征及胸部高分辨CT（HRCT）特点并观察治疗后HRCT的变化。结果（1）SSc—ILD的发生率最高，为70．0％。其次为pSS—ILD、PM／DM-ILD、RA—ILD、SLE—ILD，系统性红斑狼疮继发的ILD（SLE—ILD）发病年龄（26．2±7．8）较其他CTD-ILD早（P〈0．05）。RA—ILD、SSc—ILD病程较长，pSS—ILD、SLE一[LD和PM—ILl）病程较短。RA．ILD出现活动后气促少见（P〈0．05）。pSS—ILD出现呼吸道症状（40％）和体征（50％）最多见。85．7％SSc-ILD出现雷诺现象，支持雷诺现象与ILD相关。（2）SLE—ILD以磨玻璃影多见，丽蜂窝状改变较少；pSS-ILD以蜂窝状改变和纵隔淋巴结肿大相对较多。（3）大部分患者治疗后肺部间质病变吸收好转（56．7％），其中88．2％SLE—ILD吸收好转，28．4％患者肺部病变处于静止，14．9％患者间质病变较前进展。结论CTD—ILD患者一旦确诊，应尽早用激素联合免疫抑制剂治疗。     

类风湿关节炎继发不同类型肺间质病变临床分析

目的比较类风湿关节炎(RA)继发不同类型肺间质病变(ILD)患者的临床、肺部影像以及肺功能特点。方法选择2007年1月至2013年4月北京大学第一医院风湿免疫科或呼吸科住院经高分辨CT(HRCT)确诊、资料完整的RA-ILD的患者51例,收集临床、血气分析、RA血清学及肺功能等指标,比较不同肺HRCT分类患者临床特点的差异。结果肺HRCT下寻常型间质性肺炎(UIP)改变13例,非特异性间质性肺炎(NSIP)改变10例,机化性肺炎(OP)改变8例,无法分类20例,各组在性别、年龄、吸烟史、血清学指标方面差异无统计学意义;12例患者在确诊RA前出现ILD,其余39例患者ILD出现在RA诊断后或与RA同时出现,RA诊断后继发NSIP的中位时间间隔为1.45年(IQR 4.37,范围0~9.75年),早于其他类型ILD患者(P0.05),OP患者的Pa O2[(7.65±1.38)k Pa]低于其他类型ILD患者[UIP(10.23±1.58)mm Hg,NSIP(9.56±3.05)mm Hg,无法分类(10.41±2.39)mm Hg,P0.05]。不同类型ILD患者的肺功能水平比较差异无统计学意义。Logistic回归分析发现,ILD病程(OR=1.451,95%CI1.032~2.040,P=0.032)、NSIP(OR=8.326,95%CI 1.243~55.778,P=0.029)以及OP(OR=17.431,95%CI 1.901~159.811,P=0.011)是RA-ILD出现Ⅰ型呼吸衰竭的独立危险因素。结论 UIP是RA-ILD患者经肺HRCT可以明确分型间质病中最常见的类型,部分患者在RA发病后短期内出现ILD、NSIP、OP以及ILD病程是出现Ⅰ型呼吸衰竭的危险因素,因此,RA患者应积极筛查ILD。     

The immunoneuroendocrine role of melatonin

Abstract: A tight, physiological link between the pineal gland and the immune system is emerging from a series of experimental studies. This link might reflect the evolutionary connection between self-recognition and reproduction. Pinealectomy or other experimental methods which inhibit melatonin synthesis and secretion induce a state of immunodepression which is counteracted by melatonin. In general, melatonin seems to have an immunoenhancing effect that is particularly apparent in immunodepressive states. The negative effect of acute stress or immunosuppressive pharmacological treatments on various immune parameters are counteracted by melatonin. It seems important to note that one of the main targets of melatonin is the thymus, i.e., the central organ of the immune system. The clinical use of melatonin as an immunotherapeutic agent seems promising in primary and secondary immunodeficiencies as well as in cancer immunotherapy. The immunoenhancing action of melatonin seems to be mediated by T-helper cell-derived opioid peptides as well as by lymphokines and, perhaps, by pituitary hormones. Melatonin-induced-immuno-opioids (MHO) and lymphokines imply the presence of specific binding sites or melatonin receptors on cells of the immune system. On the other hand, lymphokines such as -γ-interferon and interleukin-2 as well as thymic hormones can modulate the synthesis of melatonin in the pineal gland. The pineal gland might thus be viewed as the crux of a sophisticated immunoneuroendocrine network which functions as an unconscious, diffuse sensory organ.     

Response of rat pineal melatonin to calcium, magnesium, and lithium is circadian stage dependent

Abstract: Herein we documented the response of pineal melatonin production to electrolytes known to be effective on pineal function in view of a possible circadian stage dependence. We studied the release of melatonin by perifused rat pineal glands at 2 different circadian stages corresponding to the middle of the light and dark periods, i.e., respectively, 7 and 19 HALO (Hours After Light Onset, L:D = 12:12). The initial efflux rates were, as expected, much higher in the perifusates of glands removed from rats sacrificed during the dark phase than of those removed during the light phase. After 3 hr of perifusion, melatonin release reached similar levels which were found constant up to the 8th hr of perifusion, whatever the circadian stage. Perifusion of the glands with physiological concentrations for the rat of calcium (5.2 mmol/1) and magnesium (1.34 mmol/1) resulted in a stimulatory effect on the pineal glands removed from rats sacrificed in the middle of the dark period (19 HALO), whereas no effects were observed on the pineal glands removed from rats sacrificed during the light (7 HALO). Lithium (0.28 and 0.55 mmol/1) was ineffective on melatonin release in pineal glands removed 7 and 19 HALO. Our results show differences in the initial efflux rates of melatonin and in the response of perifused pineal glands to calcium and magnesium according to the circadian stage.     

Changes in connexin43, the gap junction protein of astrocytes, during development of the rat pineal gland

Abstract: The abundance of gap junctions between rat pineal astrocytes formed by connexin43 (Cx43) was studied during development. Levels and distribution of Cx43 were measured by immunoblotting and indirect immunofluorescence, respectively. The amount of Cx43 in cells located within the gland was low until about the 7th postnatal day and increased to adult values between the 14th and 21st days postpartum. Although astrocytes, recognized by their vimentin immunoreactivity, were scarce before birth, they were abundant by the 7th postnatal day suggesting that the low levels of Cx43 found at this age corresponded to a low expression of this protein. Localization of the immunoreactivity to Cx43 and vimentin showed a close correlation, indicating that mature or immature pineal astrocytes form gap junctions made of Cx43. Since Cx43 levels attained their adult values at about the time the innervation and the functional state of the gland reached maturity (2–3 weeks after birth), it is proposed that astrocyte gap junctions are involved in the function of the adult rat pineal gland.     

Conservative management of perforated duodenal diverticulum： A case report and review of the literature

Duodenal diverticula are a relatively common condition. They are asymptomatic, unless they become complicated, with perforation being the rarest but most severe complication. Surgical treatment is the most frequently performed approach. We report the case of a patient with a perforated duodenal diverticulum, which was diagnosed early and treated conservatively with antibiotics and percutaneous drainage of secondary retroperitoneal abscesses. We suggest this method could be an acceptable option for the management of similar cases, provided that the patient is in good general condition and without septic signs.     

Presence of immunoreactive growth hormone and prolactin in the ovine pineal gland

Abstract: The use of antisera raised against bovine growth hormone (GH) and ovine prolactin (PRL) enabled the detection of related immunoreactive (ir) sequences of proteins in ovine pineal tissue. The isolation of PRL-like ir-material was accomplished using a 0.25 M ammonium sulphate (pH 5.5) extraction followed by ethanol precipitation, whereas the resulting 2.0 M ammonium sulphate (pH 7.0) precipitate contained a GH-like immunoreactivity. Gel chromatography of the GH-like immunoreactivity (Sephadex G-100) indicated the presence of several GH-like fragments ranging in the M_r range of 7,000 to 55,000. Analyses of the PRL-like ir-material found in pineal tissue on HPLC using a TSK 545-DEAE column led to the resolution into a single peak of immunoreactivity. A single peak of activity was also observed following chromatofocusing and hydrophobic interaction chromatography of the ir-peak from the TSK 545-DEAE column. The PRL-like ir-material inhibited the binding of [¹²⁵I]ovine PRL-S14 to anti-ovine PRL antibodies without showing an affinity for binding to anti-rat PRL or anti-bovine GH antibodies. Scatchard analysis of the binding of pineal PRL-like ir-material and pituitary ovine PRL-S14 to liver membranes from day-20 pregnant rats revealed similar affinity constants (K_a of 4.7 ± 0.2 × 10⁹ M^-1). In addition, the replication of Nb 2 Node rat lymphoma cells was stimulated by pineal PRL-like ir-material, an effect known to be specific for lactogenic hormones. The pineal PRL-like immunoreactivity appeared on sodium dodecyl sulfate polyacrylamide gels as a single major band of M_r 24,000. The functional status of PRL-and GH-like ir-material in the ovine pineal remains to be determined, but evidence is presented that the overall protein synthesis rate of the rat pineal responded to circulating concentrations of PRL.     

Microbiology of human immunodeficiency virus anorectal disease

PURPOSE: Individuals who are seropositive for the human immunodeficiency virus are at high risk for opportunistic infection and anorectal disorders. Little prospective information is available regarding anorectal pathogens in these patients. METHODS: One hundred sixty-three HIV-seropositive patients presented to the colorectal clinic between 1989 and 1992. Forty-seven (29 percent) patients were thought to have an infectious process and were prospectively studied using a standardized multiculture protocol. RESULTS: Mean age was 33 (range, 19–59) years. All were male; high-risk behavior accounted for 87 percent of HIV transmissions. Presenting complaints included anorectal pain (79 percent), pus per anum (28 percent), and blood per anum (26 percent). Examination revealed perianal tenderness (60 percent), condyloma (38 percent), perianal ulcers (38 percent), and anal fissures (34 percent). Sixty-six sets of cultures were performed; 28 patients had one set, 15 had two sets, and 4 had three sets. Thirty-two of these 47 patients (68 percent) had positive cultures including herpes (50 percent), cytomegalovirus (25 percent),Neisseria gonorrhoeae (16 percent), chlamydia (16 percent), acidfast bacilli (2 percent), and others (9 percent). Six of 32 patients with positive cultures had more than one organism cultured. Sixteen (50 percent) patients with positive cultures were treated medically, 8 (25 percent) were treated surgically and 8 (25 percent) were treated with both modalities. Sixty-one procedures were performed on 17 patients for condylomata. Eighteen patients had 20 procedures for abscesses, 50 percent of whom had positive cultures for other than common bowel flora; all improved. Fourteen patients underwent 33 procedures for perianal fistulas.Mycobacterium fortuitum was cultured from one patient who required 13 procedures for abscesses and fistulas. Forty-five (96 percent) patients were followed for an average of 12.5 months ±2.9 SEM (range, 1–94 months). Symptoms were improved or resolved in 22 of 32 (69 percent) patients with positive cultures and in 11 of 13 (84 percent) with negative cultures. CONCLUSIONS: Specific pathogens may often be identified in human immunodeficiency virus-seropositive patients with anorectal disorders if aggressively sought. Although patients without specific pathogens identified may be expected to improve with planned empiric treatment, positive identification allows more directed therapy.