系统性硬皮病的治疗及其机制的探讨

系统性硬皮病(SSc)也称进行性系统性硬化症,以弥漫性的皮肤和内脏器官的炎症和纤维化为特征,依临床表现的不同而可分为肢端型、弥漫型及其亚型CRSET综合征。病人往往开始先有雷诺现象,以后再发生皮肤硬化和累及其他器官如骨和关节、胃肠道、肺、心脏和肾脏等。虽然过去已有各种     

系统性硬皮病46例临床分析

为探讨系统性硬皮病(SSc)的皮肤、内脏损害特点和治疗方法,对2001~2005年在上海华山医院住院的46例SSc的临床表现、实验室检查及辅助检查结果、治疗方法进行统计分析。结果:46例均有不同程度皮肤损害,其中肢端型41例,弥漫型5例;45例双手皮肤累及,32例面部出现皮损,内脏损害以肺部最常见,27例检查肺功能有17例发现肺功能障碍,其中仅3例有呼吸道症状。本病目前尚无特效治疗方法。SSc皮肤损害以肢端型为主,双手、面部累及率高;内脏损害以肺部最常出现,应在疾病早期拍胸片、检测肺功能和行肺部高分辨率CT检查,以及早发现肺部病变及时治疗。     

系统性硬皮病患者血浆肾上腺髓质素及内皮素水平的测定

目的:探讨肾上腺髓质素(ADM)和内皮素(ET)在系统性硬皮病(SSc)发病中的作用。方法:采用放免法测定28例SSc患者和20名健康人的血浆ADM和ET-1水平。结果:SSc患者血浆ADM水平为(222.26±48.64)pg/mL,高于正常对照组(89.07±13.00)pg/mL,两组间比较,差异有显著性(P<0.01);SSc伴肺动脉高压者其血浆中ADM水平(254.73±39.36)pg/mL明显高于不伴肺动脉高压者(206.88±45.64)pg/mL,二者之间差异有显著性(P<0.01)。SSc患者血浆ET-1水平为(196.45±65.20)pg/mL,高于正常对照组(54.17±11.70)pg/mL,两组间比较,差异有显著性(P<0.01);SSc伴肺动脉高压者其血浆中ET-1水平(273.19±48.97)pg/mL明显高于不伴肺动脉高压者(160.11±31.17)pg/mL,二者之间差异有显著性(P<0.01)。血浆中ADM和ET-1水平呈正相关(r=0.5148,P<0.01)。结论:ADM和ET与SSc关系密切,可能在SSc发病和防治中具有重要的生理及病理学意义。     

指间关节病性银屑病14例临床分析

关节病性银屑病(PsA)又称银屑病性关节炎,远端指间关节受累、不对称性关节炎、附着点炎及指(趾)炎是PsA的经典表现[1].Moll-Wright分类标准将本病分为5种类型:①远端指(趾)间关节炎型(DIP);②非对称性少关节炎型(AO);③对称性多关节炎型(SP);④残毁性关节炎型(AM);⑤脊椎关节病型(SPON)[2].为探讨特定类型PsA的临床特点,本文对首发类型为DIP型PsA患者的临床资料进行归纳总结.     

关节病性银屑病12例临床分析

关节病性银屑病(arthritic psoriasis，PA)是银屑病的特殊临床类型，临床上呈血清学阴性和关节非对称性受累的特点，通常累及脊柱、骶髂关节和远端指趾间关节。大多数患者表现为慢性进行性过程。目前，PA通常分为以下5种临床类型：④非对称性远端指趾骨间关节炎型；②残毁性关节炎型；⑧类风湿性关节炎样的对称性多关节炎型；④非对称性少关节炎型；⑤强     

风湿性疾病中抗白细胞介素-6自身抗体

收集了52例系统性硬化病(SSc,男7例、女45例、平均年龄52±12岁),其中弥漫型38例、局限型14例;76例 SLE(男4例、女72例);140例类风湿性关节炎(男22例、女118例);28例多发性肌炎或皮肌炎(PM/DM,男9例、女19例),平均年龄分别为38±13岁、49±14岁和47±16岁,将患者的血清与104名健康者血清作对照(男52例、女     

蝮蛇抗栓酶治疗系统性硬皮病30例

1987年～1995年我们用蝮蛇抗栓酶治疗系统性硬皮病30例,并以低分子右旋糖酐加复方丹参注射液治疗30例作对照,结果表明蝮蛇抗栓酶的效果更佳,副作用小,可作为系统性硬皮病的一种常规治疗方法。临床资料　60例系统性硬皮病均为住院患者,根据临床表现及实验室检查确诊。男16例,女44例。年龄18～72岁,平均42岁。病程2月～19年,平均3年。60例随机分为二组,蝮蛇抗栓酶治疗组中肢端型25例,弥漫型5例。低分子右旋糖酐加复方丹参注射液对照组中肢端型26例,弥漫型4例。治疗方法　患者入院后检查肝、肾功能、血小板、出、凝血时间均正常。30例用蝮蛇抗…     

界线类偏结核样型麻风一例

患者男,76岁.肢端溃疡、畸形、感觉障碍20年,伴全身水肿性红斑半年.20年前患者无明显诱因手指、足趾溃疡、皲裂,之后逐渐出现骨质吸收,肢端形成畸形.发病初期溃疡疼痛,之后痛觉逐渐减轻,肢体远端麻木明显,时有关节疼痛,曾先后在多家医院就诊,拟诊为"类风湿性关节炎"和"外周神经炎",给予泼尼松及中草药等治疗,疗效不显著.     

男性系统性硬皮病的临床分析

我们对我院 1958～ 1997年期间,资料较完整的 22例男性系统性硬皮病 (SSc)的发病情况、临床表现及实验室检查作分析研究,报道如下。 一、临床资料 (一 )一般资料： 131例 SSc均为我院住院患者,均符合 SSc诊断标准。 131例中男 22例 (17％ ),年龄 19～ 72岁,平均 44.1岁;病程 11 d至 19年,平均 3.9年,其中 15例 (68％ )病程 4～ 8个月。 22例中弥漫性 14例 (64％ )、肢端性 8例 (36％ ),女性 SSc 109例,年龄 14～ 72岁,平均 38.8岁;病程 1个月至 33年,平均 5.3年, 1～ 4年为最多,共 58例 (53％ )。弥漫性 14例 (13％ )…     

关节病型银屑病30例临床分析

目的:总结关节病型银屑病的临床特点.方法:对30例关节病型银屑病患者的临床资料进行回顾性分析.结果:本组病例的平均发病年龄为31.4岁,男:女为1:1.以皮损为首发者26例(86.6%);皮损和关节症状同时出现者2例(6.7%);以关节炎为首发者2例(6.7%).皮损类型为寻常型27例(90%).关节炎分类为对称性多关节炎型12例(40%);其次为远端指(趾)关节炎型7例(23.3%);非对称性关节炎型6例(20%);最后为残毁性关节炎型3例(10%);脊柱关节病变型2例(6.7%).经X线检查的26例患者中,23例阳性(88.5%).常见的改变有关节间隙变窄、骨质疏松、骨质吸收等.结论:各型银屑病皮损都可伴发关节炎病变.对称性多关节炎和远端指(趾)关节炎、非对称性少数关节炎是本病的主要特点.     

Biogenic amines derived from tryptophan in systemic and cutaneous scleroderma.

Blood levels of serotonin (5-HT) and urinary excretion of 5-hydroxyindole-acetic acid (5-HIAA), tryptamine (T), and indole-acetic acid (IAA) were determined in 39 cases of systemic scleroderma and in 7 cases of very severe cutaneous scleroderma. The T/IAA ratio was normal and serotonin elevated in mild and rather severe acrosclerosis alike, i.e. in systemic scleroderma with pronounced vascular involvement. The T/IAA ratio was increased and serotonin normal in severe acroscleroderma, representing an intermediate form or transition to diffuse scleroderma, in severe diffuse scleroderma, and in severe cutaneous scleroderma. The T/IAA ratio and serotonin level were both elevated in a few cases of systemic scleroderma and in severe generalized morphea with concomitant vascular changes. The findings suggest impaired monoamine oxidase (MAO) activity in scleroderma with consequent accumulation in the organism of biogenic amines derived from tryptophan. An increased T/IAA ratio seems to be of prognostic significance in scleroderma, suggesting an adverse course of the disease.     

Systemic scleroderma. Clinical and pathophysiologic aspects

Systemic scleroderma is a generalized disease of connective tissue involving mainly the skin, the gastrointestinal tract, the lungs, the heart, and the kidneys. It can be present in different forms, of which acroscleroderma, with limited cutaneous and extracutaneous involvement, and diffuse scleroderma within a more rapid progression are most characteristic. Circulating antibodies against antinucleolar antigens are present in most patients with systemic scleroderma. They are helpful for establishing a classification and for determining the prognosis of the disease; their involvement in the pathogenesis, however, is still unclear. Alterations of the blood vessels and induction of fibroblasts by potent mediators are thought to play an important role in the early phase of scleroderma. Therefore early diagnosis is required, which then can initiate vasoactive therapy. In patients with systemic scleroderma, who also suffer from additional myositis, interstitial lung diseases, or arthritis, anti-inflammatory treatment with prednisolone and azathioprine is suggested. Development and progression of fibrosis cannot yet be influenced sufficiently. Only D-penicillamine affecting cross-linking of collagen has been widely used in scleroderma and has some beneficial effect.     

Scl 70 antibody—a specific marker of systemic sclerosis

Scl 70 antibodies were tested for in 107 patients with systemic sclerosis: 68 with acrosclerosis and 39 with diffuse scleroderma. Anticentromere antibodies (ACA) and other antinuclear antibodies (ANA) were tested for by indirect immunofluorescence on HEp-2 cells. Positive results for Scl 70 antibodies were obtained in 77% of cases of diffuse scleroderma and 44% of acrosclerosis. ACA and Scl 70 antibodies were found to be mutually exclusive. If acrosclerosis cases positive for anticentromere antibodies are excluded, the percentage of acrosclerosis cases positive for Scl 70 was 63%. ACA were found to be a marker of a benign, abortive subset of acrosclerosis with almost no cutaneous involvement (CREST), whereas Scl 70 did not discriminate between acrosclerosis and diffuse scleroderma. On HEp-2 cells Scl 70 positive sera gave a characteristic, fine speckled, almost homogeneous nuclear staining pattern.     

Anticentromere antibody: an immunological marker of a subset of systemic sclerosis

Our clinical and immunological studies of 114 cases of systemic sclerosis, 54 of Raynaud's disease and 46 of other connective tissue diseases, centered on the diagnostic and prognostic significance of anticentromere antibodies (ACA). The ACA occurred in 21 of 84 patients with acrosclerosis, in four of 54 patients with Raynaud's disease but in none of 30 patients with diffuse scleroderma or transitional form, acrosclerosis-diffuse scleroderma, or 46 cases of other connective tissue diseases. The ACA-positive patients had no contracture or immobilization of the fingers, the indurations and/or indurative oedema were confined to fingers and usually no other types of ANA were detected. However, systemic involvement and the course of the disease were comparable in ACA-negative and ACA-positive acrosclerosis patients. The studies indicate that there is a subset of acrosclerosis with minimal indurations confined to the fingers, and ACA appears to be its serological marker. We propose to use the term CREST for this subset, which to date has not been exactly defined and is regarded by some authors as synonymous with acrosclerosis.     

Eosinophilic fasciitis: an early variant of scleroderma

Eosinophilic fasciitis, originally reported as a syndrome distinct from scleroderma, appears now to be an early inflammatory variant of scleroderma. No less than one half of the cases reported as eosinophilic fasciitis have convincing features of scleroderma, including Raynaud's phenomenon, esophageal dysmotility, restrictive lung disease, diffuse hyperpigmentation, synovitis, flexion contractures, dermal sclerosis, colonic diverticula, scleroderma kidney, positive latex fixation test, and the presence of serum antinuclear antibodies (ANA). Clinical presentations of scleroderma range from isolated acrosclerosis to rapidly progressive systemic sclerosis. As clinical experience and long-term follow-up data on eosinophilic fasciitis accumulate, it appears that the syndrome may well represent another variant in the scleroderma spectrum. Reported here is a case which presented clinically and histologically as eosinophilic fasciitis, but which progressed over 3 years to diffuse, histologically confirmed scleroderma.     

系统性硬皮病甲皱微循环及其与内脏受累关系的研究

本文研究了50例系统性硬皮病患者的甲皱微循环变化及其与内脏受累的关系.得出如下结论:1.系统性硬皮病甲皱微循环确有异常改变.2.肢端型与弥漫型硬皮病甲皱微循环改变和内脏器官受累的严重程度相一致.3.有雷诺氏现象的硬皮病在甲皱微循环改变和内脏器官受累上都比无雷诺氏现象者重.4.系统性硬皮病甲皱微循环的变化较精确地反映出内脏器官受累的严重程度.     

Clinical usefulness of determining the antibodies to soluble nuclear antigens in various collagen diseases

The purpose of the study was evaluation of the clinical usefulness of determination of antibodies to soluble nuclear antigens. The study was carried out in 71 cases of various collagen diseases. Antibodies dsDNA (IIF method with Crithidium luciliae as substrate) were found only in patients with SLE and renal involvement. RNP antibodies (double immunodiffusion method) were demonstrated in 83.3% of cases of mixed connective tissue disease, and Sm antibodies in 8% of SLE patients. It is worth stressing that in the presented material Sm antibodies were present only in association with RNP antibodies. Antibodies Ro and/or La were present most often in the sera of patients with SCLE, while Scl 70 antibodies were a marker of systemic sclerosis, more frequent in patients with diffuse scleroderma, while their demonstration in acroscleroderma suggested a more severe course of the disease. The study showed a high diagnostic and prognostic value of antibodies to soluble nuclear antigens in collagen diseases.     

Progressive Systemic Sclerosis Sine Scleroderma which Developed after Exposure to Epoxy Resin Polymerization

Progressive systemic sclerosis (PSS) sine scleroderma is well known as a special form of scleroderma. Because of its rarity, its pathogenesis has not yet been elucidated. We experienced a 33-year-old man who developed PSS sine scleroderma while working with epoxy resin polymerization. He had short white frenulum linguae, diffuse hyperpigmentation and facial telangiectasia, positive antinuclear antibody, and pulmonary dysfunction, but not acrosclerosis or sclerodactylia. Modest dermal collagen proliferation in the forearm skin confirmed PSS sine scleroderma. Epoxy resin polymerizer appears to be a potent causative agent for PSS sine scleroderma as well as for generalized morphea-like PSS.     

Low-dose UVA1 phototherapy for proximal and acral scleroderma in systemic sclerosis

Previous studies report the benefit of UVA1 phototherapy in treating acrosclerosis in patients with systemic sclerosis. We carried out a retrospective study to examine the effectiveness of UVA1 phototherapy in scleroderma affecting acral and proximal sites in patients with this disease. Patients with systemic sclerosis (diffuse type, n =5; limited type, n =3) underwent low-dose UVA1 radiation (30–40 J/cm²) thrice weekly. In all patients skin lesions improved, demonstrated by a fall in the modified Rodnan skin score ranging from 8 to 18 points in diffuse systemic sclerosis and 6 to 10 points in limited disease. This study would suggest that UVA1 phototherapy is effective for scleroderma affecting proximal and acral sites in patients with systemic sclerosis.     

Natural killer cell activity of peripheral blood mononuclear cells from patients with various forms of systemic scleroderma

Peripheral blood mononuclear cells from 63 patients with systemic scleroderma, including incipient or prodromal acrosclerosis, and from 20 healthy individuals were tested for natural killer (NK) cell activity and antibody-dependent cell cytotoxicity in a 4 h 51Cr release assay using K562 and L1210 cell lines respectively. In patients with systemic scleroderma natural killer cell activity was significantly decreased compared with the controls. NK cell activity was markedly lowered in patients with diffuse scleroderma and in transitional form acrosclerosis-diffuse scleroderma, and was normal in cases of acrosclerosis and/or CREST syndrome and in cases of prodromal or incipient scleroderma. Antibody-dependent cell cytotoxicity of mononuclear cells from the systemic scleroderma patients was within the normal range. The lowered natural killer cell activity correlated with the severity of systemic scleroderma, in terms of the extent of skin and organ involvement.