Acid lysosomal hydrolases in systemic sclerosis and other connective tissue diseases

The activities of five lysosomal hydrolases were determined fluorometrically in the serum of patients with systemic sclerosis (PSS), systemic lupus erythematosus (SLE), dermatomyositis (DM), rheumatoid arthritis (RA), or Raynaud's disease (RD). In PSS the β-galactosidase activity was significantly increased compared with controls and the other connective tissue diseases. The β-N-acetyl-glucosaminidase was significantly increased in PSS, SLE and DM. In PSS both enzymes were more active in the early stage of the disease than later. These changes of enzyme pattern seem to be a relatively reliable marker for the differential diagnosis of PSS compared to other connective tissue diseases, especially for RD, in which the β-galactosidase activity was significantly decreased. Further work is required to determine whether these polysaccharide-degrading acid hydrolases play a role in the pathogenesis of PSS.     

The ribonucleic acid (RNA) skin test in systemic lupus erythematosus and other connective tissue diseases

A series of 65 patients with different autoimmune diseases was examined using different RNA-solutions for intradermal skin tests. Clinically positive results were obtained most often in patients with mixed connective tissue disease but quite often also in patients with systemic lupus erythematosus and progressive systemic sclerosis or with some symptoms of an automimmune nature. The histological examination of the biopsies from the test sites revealed that there was no correlation between the clinically positive tests and the histological criteria usually used as a sign of a positive test.     

系统性红斑狼疮以外的其他结缔组织病与妊娠

结缔组织病常为多系统损害的自身免疫性疾病,且好发于生育年龄,该文对常见结缔组织病与妊娠的相互影响、疾病合并妊娠的治疗等方面进行综述,以指导临床,提高孕妇和胎儿的生存率.     

Immunofluorescence studies in progressive systemic sclerosis (scleroderma) and mixed connective tissue disease

Immunofluorescence (IF) investigations of the skin were performed in thirty patients with progressive systemic sclerosis (scleroderma) and eight patients with mixed connective tissue disease (MCTD). The results show that speckled epidermal nuclear immunoglobulin deposition occurs not only in MCTD but also in true scleroderma. Granular IgM deposition at the dermo-epidermal junction of light-exposed skin was detected in both groups of patients, but six of eight MCTD patients also showed a granular IgM band in non-exposed skin. Antinuclear antibodies (ANA) were demonstrated in the sera of 96% and 100% of patients with scleroderma and MCTD respectively. The pattern of nuclear IF staining in scleroderma included dense fine speckles, large coarse speckles, threads, nucleolar and centromere staining. In MCTD, by contrast, the ANA staining pattern consisted of threads. The significance of ANA titres and immunological specificities for the in vivo reaction of serum ANA with epidermal nuclear antigens is discussed.     

Collagen components in the duodenal and rectal mucosa in progressive systemic sclerosis and other diseases

Small intestinal mucosa from 14 patients suffering from progressive systemic sclerosis and 22 patients with various other diseases was analysed for collagen components. There was no significant difference in the concentration of hydroxyproline, hydroxylysine and proline between the two groups. Rectal mucosa from 11 progressive systemic sclerosis patients, 5 patients with ulcerative colitis and 7 patients with various other diseases was analysed for collagen components. No significant difference was demonstrated in the concentration of hydroxyproline, hydroxylysine and proline between progressive systemic sclerosis patients and patients with various other diseases, but in patients with ulcerative colitis the concentration of hydroxyproline, hydroxylysine and proline were found to be significantly lower than in the two other groups.     

自噬在结缔组织疾病中的作用研究进展

细胞自噬是一种通过清除体内功能紊乱的蛋白或受损的细胞器,维持内环境稳态的重要降解过程.自噬还调控多种细胞生物学行为,包括细胞凋亡、代谢、炎症反应、抗原提呈、病原体清除等,与众多疾病密切相关.结缔组织病是一种病因不十分清楚,常伴有免疫学功能异常的一组疾病.而细胞自噬又被认为是调节机体免疫功能的重要机制之一,参与了T、B淋巴细胞的激活及增殖,成为连接固有免疫及适应性免疫的桥梁.因此,通过对细胞自噬在结缔组织疾病中作用机制的研究,为临床医生认识和治疗相关皮肤疾病提供理论依据.     

Elevation of IgG levels is a serological indicator for pulmonary fibrosis in systemic sclerosis with anti-topoisomerase I antibodies and those with anticentromere antibody

It is unclear whether any clinical and laboratory features are associated with pulmonary fibrosis (PF) in systemic sclerosis (SSc). We assessed these features using a database of 29 patients with SSc and anti-topoisomerase I antibodies and 68 patients with SSc and anticentromere antibody (ACA). Clinical features were not associated with the incidence of PF in patients with SSc and anti-topoisomerase I antibodies, although severe skin sclerosis was correlated with the presence of PF in patients with ACA. Serum IgG levels were often raised in patients with SSc and PF. Serum IgG levels in patients with PF were significantly higher than those in patients without PF, and were negatively correlated with percentage vital capacity and percentage diffusing capacity of the lung for carbon monoxide. In addition, serum IgG levels were correlated with serum interleukin-6. Thus, serum IgG levels are associated with PF in patients with SSc and anti-topoisomerase I antibodies and in patients with SSc and ACA.     

Diagnostic significance of autoantibodies in connective tissue diseases

Autoantibodies have become an important diagnostic tool for the diagnosis of connective tissue diseases (CTD) and for defining certain subgroups of these diseases. According to the reationship between clinical symptoms and the specificity of autoantibodies they can be classified into two major groups: (1) marker antibodies which are highly specific for a particular disease entity and (2) symptom specific autoantibodies associated with certain clinical expressions or subgroups. Titers of autoantibodies can vary from low to high but do usually not correlate with the status of disease activity. In order to interpret the significance of autoantibodies in CTD and to predict the course of disease it is necessary not only to identify single autoantibody specificities but to consider the combination in which they appear.     

Diagnostic significance of autoantibodies in connective tissue diseases

Autoantibodies have become an important diagnostic tool for the diagnosis of connective tissue diseases (CTD) and for defining certain subgroups of these diseases. According to the reationship between clinical symptoms and the specificity of autoantibodies they can be classified into two major groups: (1) marker antibodies which are highly specific for a particular disease entity and (2) symptom specific autoantibodies associated with certain clinical expressions or subgroups. Titers of autoantibodies can vary from low to high but do usually not correlate with the status of disease activity. In order to interpret the significance of autoantibodies in CTD and to predict the course of disease it is necessary not only to identify single autoantibody specificities but to consider the combination in which they appear.     

Chromosomal breakage in systemic sclerosis and related disorders.

Chromosome aberrations such as gaps and breaks of one or both chromatids, acentric fragments, dicentrics, ring chromosomes and other abnormal chromosomes are observed in lymphocyte and fibroblast cultures as well as in direct bone marrow preparations from patients with systemic sclerosis. A serum factor producing chromosome breaks in mitoses from healthy donors was observed in 37 of 42 scleroderma patients. The biochemical nature of this breakage factor is still undefined. Increased breakage is also noted in a high percentage of healthy family members of scleroderma patients. It is also a common feature of related disorders such as lupus erythematosus, dermatomyositis, periarteritis nodosa and rheumatoid arthritis. An increase in chromosome breaks and rearrangements is also present in NZB mice developing spontaneously an autoimmune disorder that has been extensively studied by workers interested in lupus erythematosus. The similarity of the cytogenetic findings provides the opportunity to use these mice as an experimental model to investigate relationships between immunological perturbations and chromosomal aberrations.     

Enhanced antibody titers to an oxidized DNA base in inflammatory and neoplastic diseases

Reactive oxygen species (ROS) produced by phagocytic cells induce oxidative stress during chronic inflammation. ROS play a role in the pathogenesis of a broad range of diseases including autoimmune, cardiac and neoplastic abnormalities. We found that sera of patients with a variety of inflammatory dermatoses contain elevated levels of antibodies (Ab) binding to an oxidized DNA base derivative, 5-hydroxymethyl-2'deoxyuridine (HMdU) coupled to bovine serum albumin, as determined by the enzyme-linked immunosorbent assay. Patients with immune complex diseases and a history of neoplasm elaborated the highest titers of anti-HMdU Ab. Titers from sera of psoriatic subjects were lower than from the aforementioned groups but were still significantly elevated (p < 0.001) above those of healthy controls. Treatment of inflammatory dermatoses with systemic antiinflammatory and cytotoxic drugs significantly lowered the titers [p < 0.005 (immune complex) or p < 0.001 (psoriasis and neoplastic) diseases], suggesting that this assay may be of value in monitoring the response to therapy in these diseases.     

结缔组织病患者念珠菌深部定植的危险因素分析

目的 了解结缔组织病患者念珠菌深部定植的发生率及相关危险因素.方法 对153例结缔组织病患者和63例健康人的咽拭、中段尿、肛拭标本进行真菌培养,采用Logistic相关回归分析模型,对患者念珠菌深部定植的危险因素进行相关性分析.结果 患者组的念珠菌深部定植率35.29%显著高于健康人组7.94%,菌种分析以白念珠菌最常见.患者组血红细胞计数降低,尿蛋白增多,血清补体(CH50、C3、C4)水平降低,糖皮质激素使用每日均量较高以及广谱抗生素使用与结缔组织病患者念珠菌深部定植有显著相关性;而性别、年龄、身高、体质量、病程、有结缔组织病家族史、既往脏器损害、血白细胞及中性粒细胞计数低下、血小板计数低下、尿红细胞与白细胞异常增多、糖皮质激素使用总量与疗程、某些免疫抑制剂(环磷酰胺、硫唑嘌呤等)的使用、雷公藤的使用总量与疗程、窄谱抗生素的使用等与结缔组织病患者念珠菌深部定植无显著相关性.结论 结缔组织病患者念珠菌深部定植率显著高于健康人,控制相关危险因素将减少念珠菌深部定植的发生.     

Dermoscopy findings of nail fold capillaries in connective tissue diseases

Nail fold (video) capillaroscopy is a well-established technique to assess patients with Raynaud's phenomenon, in whom specific abnormalities of capillaries are predictive of underlying systemic sclerosis and its related diseases (scleroderma spectrum disorder). The typical abnormalities are also found in patients with dermatomyositis and those findings are useful for the assessment of vascular injury and the evaluation of therapeutic effect in patients with scleroderma spectrum disorder and dermatomyositis. Recently, it has been suggested that dermoscopy can replace the capillaroscopy in significant part for detection of nail fold capillary abnormalities. In this review, I summarized the established capillaroscopy findings in connective tissues diseases and tried to apply the findings of dermoscopy to the findings and classification of capillaroscopy.