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PubMed1980

Diversity of antinuclear antibodies in progressive systemic sclerosis. Anti-centromere antibody and its relationship to CREST syndrome.

Antinuclear antibodies were demonstrated in the sera of 43 of 45 (96%) patients with progressive systemic sclerosis (22 of 24 with diffuse scleroderma and 21 of 21 with the CREST syndrome variant). This high percentage of positive reactions resulted from the use of a tissue culture substrate (HEp-2) to detect antinuclear antibodies by the indirect immunofluorescent method. Patterns of nuclear staining included diffuse fine speckles, large coarse speckles, nucleolar and centromere staining. When organ sections such as mouse kidney were used as substrate for the detection of antinuclear antibodies, nucleolar staining and centromere staining were the two patterns most frequently overlooked. Three types of antibodies appeared to be highly specific for scleroderma: antibody to Scl-70 antigen, antibody to centromere, and antinucleolar antibody. The anti-centromere antibody appeared to be highly selective for the CREST variant of progressive systemic sclerosis.

Antibodies, Antinuclear↗

PubMed1996

VCAM-1 expression on endothelium in lesions from cutaneous lupus erythematosus is increased compared with systemic and localized scleroderma.

The cutaneous lesions in systemic sclerosis (SSc) and lupus erythematosus (LE) are pathologically distinct and may display separate cell adhesion receptors. We have scored lesional skin for the presence of cell adhesion molecules that may influence inflammatory and fibrotic processes in five patients with LE, six patients with diffuse scleroderma and four patients with morphoea. The immunohistological distribution, and the number and intensity of cells staining, were recorded for VCAM-1, ICAM-1, E-selectin, alpha 2 to alpha 6 and beta 2 integrins and HLA-DR. VCAM-1 staining intensity was increased on endothelium from lesions in LE compared with SSc (P = 0.05). Low-level VCAM-1 and E-selectin expression was present on endothelium from uninvolved skin including that from patients with morphoea. HLA-DR expression was increased on infiltrating mononuclear cells (P < 0.05) and keratinocytes in LE (P < 0.05) and the number of fibroblasts staining for ICAM-1 was increased in lesions from patients with SSc, although this did not reach statistical significance. Overall, with respect to endothelial adhesion events, our findings support an important role for VCAM-1 in sustaining chronic inflammation in cutaneous LE.

Adult↗

PubMed1984

The anticentromere antibody: disease specificity and clinical significance.

Serum samples from 539 subjects were screened for the presence of the anticentromere antibody on a human laryngeal carcinoma (HEp-2) cell line (Antibodies, Inc.). The antibody was present in 61 patients (11%), most of whom had features of limited scleroderma or the CREST syndrome (calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia), either independently or in association with primary biliary cirrhosis. The antibody was rarely found in patients with rapidly advancing or diffuse scleroderma. The anticentromere antibody is therefore a useful prognostic indicator in patients with early scleroderma, as it may help to predict what pattern of scleroderma will evolve. Screening for this antibody should be conducted in all patients with Raynaud's phenomenon, primary biliary cirrhosis, and scleroderma. Other previous studies have indicated a similar disease specificity and prognostic importance of this antibody.

Antibodies, Antinuclear↗

PubMed1977

Histocompatibility antigens in progressive systemic sclerosis (scleroderma).

A study was undertaken to determine the distribution of major histocompatibility (HLA) antigens in progressive systemic sclerosis (PSS) (scleroderma). A total of 106 patients with PSS and 208 normal controls were tested for the presence of 18 different HLA antigens by the microcytotoxicity technique. These patients were equally divided between patients with classical diffuse scleroderma (53 patients) and those with the CREST syndrome variant of the disease (53 patients). When the P values were multiplied by 18, to correct for the number of antigens studied, no significant alteration in the frequency of any HLA antigen was found for the entire group of scleroderma patients or for either of the two subpopulations.

Calcinosis↗

PubMed1988

Modulatory effect of sera from scleroderma patients on lymphocyte-induced angiogenesis. II. Effector cells for the enhancing effect of acroscleroderma patients' sera.

In our previous study, using the modified lymphocyte-induced angiogenesis assay, we found that incubation of normal human peripheral blood mononuclear cells (PBMCs) with sera from patients with acroscleroderma, but not with diffuse scleroderma, markedly enhanced their angiogenic capability. The results of the present work suggest that this enhancement is mediated by lymphocytes bearing receptors for the Fc portion of IgG and belonging mainly to the CD4+ T-cell subset.

Adult↗

PubMed1983

HLA-DR antigens in progressive systemic sclerosis (scleroderma).

Typing at the HLA-DR locus was performed in 125 North American white patients with progressive systemic sclerosis (PSS). We could not confirm associations of PSS with the HLA-DR5 antigen or the HLA-B8/DR3 haplotype. A weak association of the DR1 antigen and PSS with diffuse scleroderma (27.5% vs 11.5% in local controls (corrected p less than 0.05) was observed. HLA-DR1 was significantly associated with the presence of anticentromere antibodies (p less than 0.05), and this combination was found more frequently in individuals with the CREST syndrome variant of PSS. This study failed to identify a clear-cut association between PSS and a genetic factor linked to the major histocompatibility complex.

Adolescent↗

PubMed1982

Histocompatibility antigens in progressive systemic sclerosis (PSS; scleroderma).

Patients with progressive systemic sclerosis (PSS; scleroderma) were typed for the HLA-A, -B, and -DR antigens. No significant differences in the frequencies of any HLA-A or -B antigen were found. In the subgroup of patients with PSS and diffuse scleroderma (PSS-DS), the frequency of Bw35 was increased (0.30 vs 0.17 in controls; p less than 0.005, corrected P greater than 0.2). Although patients with PSS-DS also had an increased frequency of DR1 antigen (0.27 vs 0.12 in local controls; P less than 0.005, corrected P less than 0.05), no association between Bw35 and DR1 antigens could be detected. We found no increase in the frequencies of the DR3 or DR5 antigens in patients with PSS. However, in a subset of PSS patients with pulmonary fibrosis, an increase in DR3 and a decrease in DR4 antigens (P less than 0.005) were observed. Serum antibodies to centromere occurred more frequently in DR1-positive than DR/-negative patients (0.46 vs 0.18; P less than 0.005). This study of a large number of patients with PSS failed to confirm previously reported associations of PSS with the HLA-B8/DR3 haplotype of HLA-DR5 antigen.

Adolescent↗

PubMed1989

[Electrocardiographic and echocardiographic evaluation of cardiac changes in systemic scleroderma].

Cardiac abnormalities were studied using the clinical, electrocardiographic and echocardiographic methods in 66 patients with systemic scleroderma. Abnormal ecg was seen in 41 patients (62.1%), most frequently in form ventricular extrasystole (21.2%), incomplete block of the right bundle branch (12.1%), left ventricular and right atrial hypertrophy (10.6%) each, and the pattern suggesting the history of past myocardial infarction (7.6%). Abnormal echocardiographic results were found in 38 patients (57.6%). The most usual changes, differentiating that group from the controls, were: sluggish diastolic movement of the posterior wall of the left ventricle (40.7%), pericardial effusion (37.0%), elongation of the isovolumic relaxation time diastole phase (99.3 s.m.v. 81.1 ms, p less than 0.001) and right ventricular dilatation. There were no significant differences between the scleroderma and the control group as regards the indices of the contractility of the left ventricle: ejection fraction (EF), velocity of circumferential fibres shortening (VCF), diastolic size of the left ventricle and of the left atrium. The prevalence of the electrocardiographic and echocardiographic abnormalities in particular types of scleroderma (diffuse, acroscleroderma, severe acroscleroderma, CREST) was roughly similar: 50-76%. Finding of the impaired diastolic rather than systolic function of the left ventricle and of the similar prevalence of the cardiac abnormalities in the particular types of scleroderma is new and contradictory to the commonplace opinions.

Adult↗

PubMed1997

Scleroderma and pregnancy.

Pregnancy in systemic sclerosis may be uneventful, with both good maternal and fetal outcomes. Because scleroderma is a multisystem disease and complications do occur, however, careful antenatal evaluations, discussion of potential problems, and participation in a high-risk obstetric monitoring program is very important to optimize the best outcome. Because women with diffuse scleroderma are at greater risk for developing serious cardiopulmonary and renal problems early in the disease, they should be encouraged to delay pregnancy until the disease stabilizes. All patients who become pregnant during this high-risk time should be monitored extremely carefully. Although there are some suggestions that there are increases in infertility and miscarriages before disease onset, recent studies show that these issues probably do not have major impact for women with established scleroderma who plan to become pregnant. The high risk of premature and small infants may be minimized with specialized obstetric and neonatal care, however. Renal crisis in scleroderma is the only truly unique aspect of these pregnant, which, unlike blood pressure elevation in nonscleroderma pregnancies, must be treated aggressively with ACE inhibitors. Other pregnancy problems may not be unique to scleroderma, but because it is a chronic illness, any complication carries higher risks for both mother and child. Careful planning, close monitoring, and aggressive management should allow women with scleroderma to have a high likelihood of a successful pregnancy.

Female↗

PubMed1988

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.

Anti-Inflammatory Agents↗

PubMed1985

Autoantibodies in childhood scleroderma.

The clinical presentation of scleroderma in childhood is even more varied than in adult life. This study of 19 children shows that antinuclear antibodies (ANA) detected on the HEp2 cell substrate are just as common as in the adult disorders. All seven children with diffuse scleroderma or acrosclerosis were ANA positive, as were eight of the 12 with more localised disease. Antinucleolar antibodies were particularly frequent (58% overall), but anticentromere antibody was not observed. Enzyme-linked immunosorbent assays (ELISAs) for antibodies to collagen types I-V, in contrast to adult scleroderma, gave normal results in all childhood cases.

Antibodies, Antinuclear↗

PubMed1995

Immunogenetic analysis of 5 families with multicase occurrence of scleroderma and/or related variants.

OBJECTIVE: To investigate the relative contribution of the major histocompatibility (MHC) gene complex in the etiopathogenesis of familial scleroderma and/or its variants, in 5 Australian families, each with 2 affected members. METHODS: Affected individuals and consenting first degree relatives were examined and had blood collected for histocompatibility leukocyte (HLA) class I, class II antigen, and complement C4 typing. An antinuclear (ANA) profile screen on each family member was performed. RESULTS: Family 1, had 2 affected siblings (scleroderma, CREST), each anticentromere positive (> 1/640 titer), with identical HLA haplotypes. A brother, who was HLA identical to his affected sisters was clinically normal and ANA negative. In Family 2, there were no HLA haplotype similarities between the 2 sisters affected with diffuse scleroderma. Both were ANA positive (> 1/640). A 3rd sister was HLA identical to the proband but was clinically normal and ANA negative. In Family 3, affected siblings (CREST, morphea) had identical HLA haplotypes. In Family 4, both mother (CREST) and one of her 2 daughters (scleroderma) had anticentromere antibodies (1/2560). The unaffected daughter, not sharing either of her sister's haplotypes, was normal and ANA negative. In Family 5, 2 sisters (CREST, CREST) were HLA identical. CONCLUSION: A female predominance in familial scleroderma was observed. There was no common HLA haplotype between different families affected with scleroderma or its disease variants. Within families (except in one case, where the possibility of crossover exists or a question of paternity) affected siblings shared both HLA haplotypes. The development of disease was not totally accounted for by HLA genes, since family members with the same HLA haplotypes as the proband, were not affected. It appears that genes within the MHC complex are required but are not sufficient for the development of systemic sclerosis.

Adolescent↗

PubMed1992

Hemolytic uremic syndrome in a patient with systemic sclerosis treated with cyclosporin A.

The case is presented of a 48-year-old female suffering from diffuse cutaneous systemic sclerosis (diffuse scleroderma) since 8 years, who went into renal failure as part of hemolytic uremic syndrome following 3 weeks' treatment with 3.8 mg/kg cyclosporin A. Hemolytic uremic syndrome has previously been described in transplant patients receiving cyclosporin A. There are also four cases reported in the literature of renal failure developing in middle aged females with diffuse cutaneous systemic sclerosis after short-term use of low dosage cyclosporin A treatment. It is suggested, that it may be wise not to use cyclosporin A to this category of patients, in which it can not be ruled out, that even a low dose therapy may trigger the rapid onset of scleroderma renal crisis or as in our case provoke hemolytic uremic syndrome.

Acute Disease↗

PubMed1990

[Anti-Scl-70 antibodies in systemic scleroderma].

We looked for anti-Scl-70 and anti-centromere antibodies in 109 patients (26 men and 83 women). Mean age was 43 +/- 15 years. Forty patients had systemic sclerosis according to ARA criteria. The extension of cutaneous involvement was defined by using Barnett and Coventry criteria: 12 patients were type I (sclerodactyly), 20 type II (acrosclerosis) and 8 type III (diffuse scleroderma). Among the 12 patients with type I, there were 8 cases of CREST syndrome defined as follows: presence of sclerodactyly, Raynaud's phenomenon and 2 of the 3 following criteria: oesophageal dysmotility, calcinosis, telangiectasia. Other organ involvement was recorded. Control patients had idiopathic Raynaud's phenomenon (n = 22), other connective tissue diseases (n = 20), and miscellaneous diseases (n = 28). Ninety-nine patients were prospectively included in this study. Patients' sera were stored at -20 degrees C. Ten previously stored sera obtained from patients with systemic sclerosis were also analyzed. Immunological tests were performed simultaneously and with no information on the diagnosis. When antinuclear antibodies were detected by indirect immunofluorescence, double immunodiffusion and immunoblotting were performed. Anti-Scl-70 antibodies were detected in systemic sclerosis only: 1 of 12 type I, 11 of 20 type II and 4 of 8 type III. One serum negative by immunodiffusion was positive using immunoblotting. We found that the specificity of anti-Scl-70 antibodies for systemic sclerosis was 100 p. 100 and their sensitivity 40 p. 100. There was a correlation between the presence of anti-Scl-70 antibodies and the presence of antinuclear antibodies (p less than 0.05) and the extent of cutaneous involvement (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1985

Modulatory effect of sera from scleroderma patients on lymphocyte-induced angiogenesis.

Sera from 22 patients with progressive systemic sclerosis were tested for the ability to modify the angiogenic capability of normal human mononuclear cells. The sera from patients with acrosclerosis, including the abortive form (CREST syndrome: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias), markedly enhanced this capability compared with sera from both healthy donors and patients with severe acrosclerosis and diffuse scleroderma. The enhancing effect of sera from patients with acrosclerosis decreased and/or disappeared in cases where the patient's acrosclerosis was chronic and severe. Thus, this test may be of diagnostic value in distinguishing various subgroups of systemic sclerosis.

Adult↗

PubMed1992

[Clinical significance of anti-nucleolar antibodies detected by immunoprecipitation method in patients with systemic sclerosis].

We have characterized a clinical significance of anti-U3RNP, anti-7-2RNP, anti-RNA polymerase I and anti-PM-Scl antibody, autoantibodies to nucleolar proteins detected by immunoprecipitation method in patients with systemic sclerosis (SSc). In 248 patients with SSc, anti-U3RNP antibody was positive in 9 (3.6%), anti-7-2RNP antibody was positive in 7 (2.8%) and anti-RNA polymerase I antibody was positive in 3 (1.2%). But none of 248 patients was positive for anti-PM-Scl antibody. Anti-U3RNP antibody positive SSc patients showed significantly lower frequency of joint and lung involvements, compared with anti-U3RNP antibody negative SSc patients. Anti-7-2RNP antibody was found only in patients with limited scleroderma. The anti-7-2RNP antibody could be detected before appearance of skin thickening, so this indicate the usefulness of detecting anti-7-2RNP antibody in the early stage of SSc. Two of 3 anti-RNA polymerase I antibody positive patients were classified as diffuse scleroderma. All anti-RNA polymerase I antibody positive patients had high incidence of internal organ involvements including lung, heart and kidney, so two of these patients died of heart failure. These data showed the close clinical association of antigenic specificities of anti-nucleolar antibodies analysed by immunoprecipitation method, and indicated the usefulness of detecting these anti-nucleolar antibodies in subgrouping of patients with SSc.

Adult↗

PubMed1989

Serum aminoterminal propeptide of type III procollagen in progressive systemic sclerosis and localized scleroderma.

Sera from 31 patients with progressive systemic sclerosis (PSS), 5 patients with widespread localized scleroderma (LS), and 3 patients with lichen sclerosus et atrophicus were analyzed for aminoterminal propeptide of type III procollagen (PIIINP) using a radioimmunoassay based on human propeptide. Thirty-eight per cent of the patients with PSS had levels above normal range, including all of the 3 patients with diffuse scleroderma. The same applies to 4 of 5 patients with widespread localized LS, while PIIINP in all 3 patients with lichen sclerosus et atrophicus were within normal levels. In patients with acrosclerosis, elevated PIIINP seems to be correlated to rapid progression and extension of lesions. A significant increase in PIIINP was found in a patient following discontinuation of prednisone and cyclophosphamide, while the present investigation did not allow judgement of effects of treatment with either penicillamine or cyclosporin A.

Humans↗

PubMed1990

Specificity of anti-Scl-70 antibodies in scleroderma: increased sensitivity of detection using purified DNA topoisomerase I from calf thymus.

A large scale purification of DNA topoisomerase I from calf thymus was obtained using the combinations of 62% ammonium sulfate precipitation, Biorex 70, hydroxylapatite and phenylagarose chromatography. Silver staining of the preparation separated by sodium dodecyl sulfate-polyacrylamide gel electrophoresis revealed 2 bands of apparent molecular weight of 70 and 60 kDa. Immunoblotting with a serum of anti-Scl-70 specificity also recognized both proteins. Anti-Scl-70 antibodies were detected in 24/32 (75%) patients with diffuse scleroderma by ELISA and 28/32 (87%) had the ability to neutralize the topoisomerase I activity. All of 14 sera with anticentromere antibodies did not react with DNA topoisomerase I by both assays. The presence of anti-Scl-70 antibodies remained disease specific despite the increase in sensitivity of both assays.

Animals↗