進(jin)(jin)(jin)(jin)行(xing)性系(xi)統(tong)(tong)(tong)性硬皮(pi)(pi)病(bing)(bing)(bing)(bing)(bing)(PSS)是病(bing)(bing)(bing)(bing)(bing)原(yuan)尚(shang)未闡(chan)明的(de)(de)多(duo)系(xi)統(tong)(tong)(tong)疾病(bing)(bing)(bing)(bing)(bing),特征為皮(pi)(pi)膚(fu)和各內臟器(qi)官(如腸道、肺、心(xin)和腎等(deng))的(de)(de)炎癥、血管(guan)和纖維化(hua)的(de)(de)病(bing)(bing)(bing)(bing)(bing)損。患者可有(you)不(bu)(bu)(bu)同程度和范(fan)圍的(de)(de)其(qi)(qi)他(ta)系(xi)統(tong)(tong)(tong)受累,且與病(bing)(bing)(bing)(bing)(bing)變進(jin)(jin)(jin)(jin)行(xing)程度相一致。硬皮(pi)(pi)病(bing)(bing)(bing)(bing)(bing)的(de)(de)局灶型(xing)(xing)(xing)如硬斑病(bing)(bing)(bing)(bing)(bing)、線狀硬皮(pi)(pi)病(bing)(bing)(bing)(bing)(bing)、刀砍后(hou)硬疤病(bing)(bing)(bing)(bing)(bing)等(deng)。特征是在皮(pi)(pi)膚(fu)或(huo)鄰近(jin)皮(pi)(pi)膚(fu)的(de)(de)組(zu)織呈(cheng)(cheng)點狀纖維化(hua),此型(xing)(xing)(xing)不(bu)(bu)(bu)伴有(you)其(qi)(qi)他(ta)系(xi)統(tong)(tong)(tong)病(bing)(bing)(bing)(bing)(bing)損。在系(xi)統(tong)(tong)(tong)型(xing)(xing)(xing)其(qi)(qi)進(jin)(jin)(jin)(jin)展不(bu)(bu)(bu)一定(ding)并有(you)多(duo)度分期。每一期的(de)(de)臨床表現不(bu)(bu)(bu)一,可從(cong)輕(qing)度至(zhi)(zhi)嚴重,也可緩慢進(jin)(jin)(jin)(jin)展至(zhi)(zhi)嚴重或(huo)為迅(xun)速進(jin)(jin)(jin)(jin)展。雖然本病(bing)(bing)(bing)(bing)(bing)稱為進(jin)(jin)(jin)(jin)行(xing)性,但不(bu)(bu)(bu)意味(wei)均是進(jin)(jin)(jin)(jin)展性,有(you)的(de)(de)患者皮(pi)(pi)膚(fu)病(bing)(bing)(bing)(bing)(bing)變可呈(cheng)(cheng)穩定(ding)性或(huo)自發性緩解(jie)。雖如上述一般局灶型(xing)(xing)(xing)不(bu)(bu)(bu)演(yan)變為系(xi)統(tong)(tong)(tong)型(xing)(xing)(xing),但二者本質(zhi)并無區別,故局灶型(xing)(xing)(xing)可以隨(sui)病(bing)(bing)(bing)(bing)(bing)情進(jin)(jin)(jin)(jin)展成(cheng)為系(xi)統(tong)(tong)(tong)型(xing)(xing)(xing)。
遺傳因素
根(gen)據部分(fen)患(huan)(huan)者(zhe)有明顯家族史,在重癥患(huan)(huan)者(zhe)中HLA-B8發生率增加及(ji)患(huan)(huan)者(zhe)親屬中有染色(se)體異常,認為遺(yi)傳類型的特征可能在X染色(se)體的顯性等位基因上(shang)。
感染因素
不少患者發(fa)病前常有急(ji)性(xing)感染,包(bao)括(kuo)咽峽炎、扁桃體炎、肺炎、猩(xing)紅熱、麻(ma)疹(zhen)、鼻竇炎等。在(zai)患者的橫紋肌和腎臟中曾發(fa)現副粘病毒樣包(bao)涵(han)體。
結締組織代謝異常
患者顯(xian)(xian)示廣泛(fan)的(de)結締組織(zhi)病變,皮(pi)膚(fu)中膠原含量明顯(xian)(xian)增(zeng)多,在病毒活期皮(pi)膚(fu)損(sun)害內存在較多的(de)可溶性膠原和不(bu)穩定的(de)分子間(jian)側鏈。對患者的(de)成纖維細胞培養顯(xian)(xian)示膠原合(he)成的(de)活性明顯(xian)(xian)增(zeng)高。
血管異常
患(huan)者(zhe)多(duo)有(you)(you)雷諾現象,不僅限于(yu)肢端,也發生于(yu)內臟(zang)血(xue)管(guan);組織病(bing)(bing)(bing)(bing)理顯示(shi)皮損及內臟(zang)多(duo)可(ke)有(you)(you)小血(xue)管(guan)(動脈)攣縮(suo)及內膜增生,故有(you)(you)人認為本病(bing)(bing)(bing)(bing)是一種原發性血(xue)管(guan)病(bing)(bing)(bing)(bing),但由于(yu)血(xue)管(guan)病(bing)(bing)(bing)(bing)變并非(fei)在所有(you)(you)患(huan)者(zhe)中都能見(jian)到,故也有(you)(you)認為血(xue)管(guan)病(bing)(bing)(bing)(bing)變并非(fei)是本病(bing)(bing)(bing)(bing)唯一發病(bing)(bing)(bing)(bing)因素。
免疫異常
這(zhe)是近年(nian)來最為(wei)重(zhong)視的(de)一(yi)種看法。在(zai)患者(zhe)體(ti)(ti)內可測(ce)出多種自身(shen)抗(kang)(kang)(kang)(kang)(kang)體(ti)(ti)(如抗(kang)(kang)(kang)(kang)(kang)核(he)抗(kang)(kang)(kang)(kang)(kang)體(ti)(ti)、抗(kang)(kang)(kang)(kang)(kang)DNA抗(kang)(kang)(kang)(kang)(kang)體(ti)(ti)、抗(kang)(kang)(kang)(kang)(kang)ssRNA抗(kang)(kang)(kang)(kang)(kang)體(ti)(ti)、抗(kang)(kang)(kang)(kang)(kang)硬皮病(bing)(bing)皮膚提取液的(de)抗(kang)(kang)(kang)(kang)(kang)體(ti)(ti)等);患者(zhe)體(ti)(ti)內B細胞數增(zeng)多,體(ti)(ti)液免(mian)疫(yi)明顯增(zeng)強,在(zai)系統型患者(zhe)循環免(mian)疫(yi)復合物測(ce)定(ding)陽性(xing)率(lv)高達(da)50%以上,多數患者(zhe)有高丙球蛋白血癥;部(bu)分病(bing)(bing)例(li)常與(yu)紅(hong)斑狼瘡、皮肌(ji)炎(yan)、類風(feng)濕(shi)性(xing)關節炎(yan)、干(gan)燥綜合征(zheng)或橋本氏甲狀腺(xian)炎(yan)并發。目前(qian)多數認為(wei)本病(bing)(bing)可能是在(zai)一(yi)定(ding)遺(yi)傳背景(jing)基礎上再加持久的(de)慢性(xing)感(gan)染(ran)而導致的(de)一(yi)種自身(shen)免(mian)疫(yi)性(xing)疾(ji)病(bing)(bing)。
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