The titers and complement-activating abilities of anticentromere antibody in systemic sclerosis, other connective tissue diseases, and other related conditions

The anticentromere antibody is considered to be a useful serologic marker for the CREST syndrome. But this antibody also appears in other related conditions less frequently. We classified 29 patients with anticentromere antibodies into 3 groups: (1) 16 patients with systemic sclerosis or Raynaud's phenomenon alone; (2) 7 patients with other connective tissue diseases; (3) 6 patients with other conditions. Ig class reactivities and complement-fixing abilities of anticentromere antibody were measured by the indirect immunofluorescence test. The whole Ig titers were high (1025 or more) in all patients belonging to group 1. However, the properdin-fixing anticentromere antibody titers of these patients were relatively low (256 or less). In contrast, the patients in group 2 and 3 were shown to have higher C3- and properdin-activating abilities which were determined by the ratios of the titers of C3- and properdin-fixing anticentromere antibody to the IgG titers although the whole Ig titers of these patients were widely distributed. These data suggest that the patients who have low whole Ig titers and/or high properdin-fixing titers do not belong to the scleroderma spectrum and that the patients without clinical features of scleroderma have high C3- and properdin-activating abilities.     

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.     

Glucocorticoids in autoimmune connective tissue diseases

ABSTRACT: Glucocorticoids (GCs) are occasionally required for the cutaneous manifestations of autoimmune connective tissue diseases. In general, good therapeutic alternatives with fewer side effects than GCs are available, including anti-inflammatory agents such as antimalarials or dapsone, or immunosuppressives such as azathioprine or methotrexate, and these can serve as GC-sparing agents or can substitute for the use of GCs. When GC use cannot be avoided, it is important to implement a number of recommendations and improvements in dosing and prevention of side effects in order to optimize care. These include using appropriate and adequate doses of GCs initially, appropriate tapering regimens, and proper monitoring, prophylaxis, and treatment for infections, osteoporosis, avascular necrosis, hyperglycemia, hypertension, hyperlipidemia, and glaucoma.     

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.     

Calcinosis cutis in autoimmune connective tissue diseases

Calcinosis cutis is a chronic condition involving insoluble calcified deposits of the skin and subcutaneous tissue. It is commonly associated with autoimmune connective tissue diseases and can be a source of pain and functional disability. The likelihood of developing calcinosis varies among the autoimmune connective tissue diseases, with systemic sclerosis and dermatomyositis being the most commonly associated. Identification of therapy for this challenging disorder has been hampered by a paucity of large controlled trials. Although there is no uniformly effective treatment for calcinosis cutis, several surgical and medical therapies have demonstrated varying degrees of benefit in the treatment of calcinosis, including surgical excision, laser therapy, extracorporeal shock wave lithotripsy, diltiazem, minocycline, colchicine, and topical sodium thiosulfate, along with others. Recommendations for the diagnosis and therapy of calcinosis cutis in patients with autoimmune connective tissue diseases are discussed.     

Update on connective tissue diseases in dermatology

Recent advances in understanding the pathogenesis of autoimmune diseases, including lupus erythematosus, dermatomyositis, and scleroderma, have allowed for reorganization of the classification of these disorders. With these novel stratifications, early identification of rheumatic skin diseases with systemic implications and consistency in designing and executing therapeutic trials will be enhanced. This review will provide a compilation of updates on epidemiology, pathology, evaluation, and classification with a predominant focus on therapeutics, reflecting the growth is this area.     

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.     

未分化结缔组织病的研究进展

未分化结缔组织病是临床实际存在的一种疾病状态,目前发病机制尚不清楚。未分化结缔组织病的临床特征为具有自身免疫病的症状和体征,只有一种自身抗体阳性,不符合任何结缔组织病的诊断标准。演变是未分化结缔组织病疾病过程中最为突出的特点,30％可演变为结缔组织病,70％维持未分化结缔组织病的状态。经未分化结缔组织病演变而来的结缔组织病,病情相对轻微,重要器官损害较少。疾病特点和演变结果提示,未分化结缔组织病是自身免疫性疾病疾病谱中重要一员。     

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.     

Autoimmune connective tissue diseases in the COVID-19 pandemic

Autoimmune connective tissue diseases are a heterogeneous group of clinical entities sharing a common feature-an impairment of structural components like collagen and elastin, arising by autoimmune mechanisms. Because most patients are on a long-term immunosuppressive therapy, which renders them vulnerable to infections, a new challenge appears in front of physicians in the coronavirus disease 2019 (COVID-19) era. Immune mechanisms are substantial for the control and ceasing of viral infections, and their impairment may cause serious complications; however, data from immunosuppressed transplant patients do not reveal a higher frequency or diseases’ severity in those infected by COVID-19. Several immunotherapies used to treat autoimmune connective tissue diseases favorably modulate the immune response of severe acute respiratory syndrome coronavirus (SARS-CoV-2)–infected patients.The present review highlights the problems of susceptibility, severity, and therapeutic options in patients with autoimmune connective tissue diseases during the COVID-19 pandemic. The relationship between autoimmune connective tissue diseases and COVID-19 infection is explained with antiviral protection genes expression, hypercytokinemia, and lymphohistiocytosis/macrophage activation mechanisms. Recommendations concerning therapy for prevention during the pandemic period or in case of concomitant COVID-19 infection are also presented. Clinical trials are ongoing regarding COVID-19 therapy blocking the cytokine response. © 2021 Elsevier Inc. All rights reserved.     

Serum lysosomal hydrolases in various forms of scleroderma

The activities of several lysosomal hydrolases (beta-galactosidase, beta-hexosaminidase, alpha-mannosidase, beta-glucuronidase and acid phosphatase) were determined in serum and blood lymphocytes from patients affected with scleroderma. Statistical comparisons between means of patient and control groups (Student's t test) showed a significant difference in serum beta-galactosidase and acid phosphatase between patients and controls (serum beta-galactosidase: 36.5 +/- 22 nmol/h/ml in the scleroderma group versus 24 +/- 13 nmol/h/ml in the control group; serum acid phosphatase 853 +/- 345 nmol/h/ml in the scleroderma group versus 634 +/- 295 nmol/h/ml in the control group). These differences were significant (p less than 0.01). Both enzyme activities were also significantly increased in the group with systemic scleroderma, but the difference was less in the group with localized scleroderma (this is discussed in terms of statistics and pathophysiology). The other enzyme activities determined were not significantly modified. The validity of these results is discussed, together with their diagnostic value and with the pathophysiological hypotheses put forward to explain the high levels found.