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PubMed1987

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.

Adult↗
PubMed1987

Significant increase of urinary low-sulfated heparan-sulfate-related protein in patients with severe systemic scleroderma.

Radioimmunoassay with an antibody produced against urinary low-sulfated heparan-sulfate-related protein was devised and used to screen the heparan sulfate level in the urine of patients with systemic scleroderma. Patients with diffuse scleroderma, and patients also showing polymyositis/dermatomyositis had elevated values, whereas the value in patients with acrosclerotic scleroderma did not differ from that of the control population. In addition, an increase in this protein was associated with the positivity of anti-Scl-70 antibody. These findings suggest an important role for low-sulfated heparan sulfate in the pathobiology of severe systemic scleroderma.

Adult↗
PubMedDate not supplied

Antinuclear antibodies in progressive systemic sclerosis.

Sera from 84 patients with progressive systemic sclerosis (PSS) were tested for the presence of antinuclear antibodies by immunofluorescence on HEp2 cells and gel immunodiffusion. Fluorescent antinuclear antibodies were detected in 80 subjects with PSS (95%). Ninety-three percent of patients with CREST syndrome and 3% of those with diffuse scleroderma had a centromere staining. Precipitating antibodies were found in 57% of PSS sera and identified as anti-Scl 70 in 42 cases (50%). This specificity was found in 42 of 70 subjects with diffuse scleroderma (60%); another patient was positive for anti-nRNP antibodies, and 5 more sera from PSS patients showed precipitin lines of unknown specificity. No serum from 14 patients with CREST syndrome was positive for anti-Scl 70 antibodies. Significant relationships have been found between centromere staining and CREST syndrome (p less than 0.0005) and between the presence of anti-Scl 70 antibodies and the diffuse form of scleroderma (p less than 0.0005). The latter specificity is strongly associated with grainy speckled pattern on HEp2 fluorescence (p less than 0.0005). These data suggest that anti-Scl 70 antibodies and anti-centromere antibodies are useful markers for different subgroups of patients with PSS.

Adult↗
PubMed1978

Association of HLA antigen a9 with progressive systemic sclerosis (scleroderma).

Upon evaluation of 40 subjects with progressive systemic sclerosis (PSS), a significant association of HLA antigens A9 and Aw24 (a subgroup of A9) was found with diffuse scleroderma. HLA-Aw23 (a second subgroup of A9) was also increased in the patients, however, this was not statistically significant. Diffuse scleroderma appears to be one of the few diseases that shows an association with the HLA-A locus.

Epitopes↗
PubMedDate not supplied

Stimulation of lymphocyte reactivity by a low molecular weight cutaneous antigen in patients with progressive systemic sclerosis (scleroderma).

A low molecular weight cutaneous antigen was found to stimulate the release of macrophage migration inhibition factor from circulating lymphocytes of patients with diffuse scleroderma. The antigen had a molecular weight of approximately 3,500 and contained RNA and polypeptides, but no hydroxyproline. Lymphocytes from patients with the CREST syndrome, rheumatoid arthritis, and from normal controls did not respond to the antigen. An immune response to this antigen may be a factor in the pathogenesis of diffuse scleroderma.

Adult↗
PubMed1990

Direct quantitation of skin elasticity in systemic sclerosis.

A simple instrument, the "skin elastometer," was used to evaluate the elastic and plastic properties of volar forearm skin in 24 patients with systemic sclerosis and 24 healthy individuals matched for age, race and sex. Skin elastance in 17 patients with diffuse scleroderma was found to be significantly different from matched controls (p less than 0.001), and was associated with clinical skin scores independently determined by examination (r = 0.89, p less than 0.001). Seven patients with limited scleroderma (the CREST variant) had values for skin elastance which were intermediate between those of the patients with diffuse scleroderma and healthy persons. Plastic deformation of the stretched skin was similar in patients and controls. Quantitative measurement of skin elastance is a simple technique which may prove to be of value in the assessment of patients with systemic sclerosis.

Adult↗
PubMed1987

[Uric acid levels of the serum of healthy persons and patients with various rheumatic diseases].

Serum uric acid levels were investigated in a series of 1715 subjects. Of them 596 were patients with inflammatory rheumatic disorders, 162 gout patients, 236 with osteoarthrosis, 79 with systemic lupus erythematosus or diffuse scleroderma and 642 healthy subjects. On analyzing the results, very high uricemia values were found in the gout patients. Increased uricemia values were observed is patients with psoriatic arthritis and diffuse connective tissue disorders (systemic lupus erythematosus and diffuse scleroderma). Hyperuricemia was found in psoriatic arthritis, rheumatoid arthritis and in nosological entities classified under diffuse connective tissue disorders in 5.6 to 10.1% of patients. In the healthy examinees hyperuricemia was recorded in 3.8% of the cases.

Adolescent↗
PubMed1991

Endothelial and fibroblastic activation in scleroderma. The myth of the "uninvolved skin".

We studied the immunohistochemistry of the skin of scleroderma patients to determine the differences (if any) between clinically "affected" and "nonaffected" areas. We examined paired skin biopsy samples from clinically involved forearm skin ("affected") and clinically uninvolved proximal skin ("nonaffected") taken from 19 patients with diffuse scleroderma and from 15 normal control subjects. We stained the sections with antibodies to endothelial leukocyte-adherence molecule type 1 (ELAM-1; to detect endothelial activation) and to procollagen-1 (PC-1; to detect newly formed, unprocessed collagen). There was increased expression of ELAM-1 and PC-1 in sclerodermatous skin as compared with the controls, but there was no difference between clinically affected and nonaffected skin samples. In 10 of 11 patients whose condition was getting worse, endothelial and fibroblast activation preceded fibrosis. Endothelial and fibroblast activation are more widespread in the skin of scleroderma patients than is evident by inspection on physical examination. What appears to be "normal" skin in diffuse scleroderma is already pathologic, as shown by abnormal endothelial activation and procollagen production.

Adult↗
PubMed1989

Increased mast cell numbers in the sclerotic skin of porphyria cutanea tarda.

We quantitated numbers of mast cells in the sclerotic skin noted on the dorsa of the hands of 10 patients with porphyria cutanea tarda (PCT), and compared them with those of diffuse scleroderma and healthy controls. Mast cell counts in sclerodermoid skin of PCT patients were significantly greater than those in involved skin of 9 patients with diffuse scleroderma in its late stage and also greater than those in normal skin of 8 controls. When mast cell density was analyzed according to the depth of the dermis, an 84% increase was noted in the uppermost layer (0-0.2 mm in depth) and a 150% increase in the second uppermost layer (0.2-0.4 mm in depth) in the patients with PCT when compared with those in the corresponding sites of the controls. These results suggest a possible role of mast cells in the pathogenesis of sclerodermoid skin of PCT.

Adult↗
PubMed1992

Reactivity of anti-mitochondrial antibodies in primary biliary cirrhosis and systemic sclerosis.

Anti-mitochondrial antibodies (AMA) were detected by indirect immunofluorescence in the sera of 16 out of 17 (94%) patients with primary biliary cirrhosis (PBC). Immunoblotting experiments with mitochondrial polypeptides from the porcine liver as antigens revealed that three antigens were recognized by the sera from AMA-positive patients. These were a 70-kD protein recognized by nine out of 16 AMA-positive sera, a 50-kD protein recognized by 13 out of 16 AMA-positive sera and a 39-kD protein recognized by four out of 16 AMA-positive sera. The reactivity of these polypeptides was destroyed by brief exposure to trypsin. None of these antigens were recognized by any of the 30 control sera. These results show that the 70-kD, 50-kD and 39-kD proteins are the major mitochondrial autoantigens recognized by sera from patients with PBC. In addition, of 30 sera samples from patients with diffuse scleroderma, 13 reacted to the 70-kD and/or 50-kD antigens. Anti-centromere antibodies (ACA) were also detected in the sera of five of the 17 (29%) patients with PBC. The high prevalence of ACA in patients with PBC and the presence of anti-70- and 50-kD antibodies in patients with diffuse scleroderma provide evidence of an association between these two disorders.

Adult↗
PubMedDate not supplied

Clinical subsets of scleroderma: relevance of fluorescent and precipitating antinuclear antibodies.

Sera from 7 patients with localized and 35 with systemic scleroderma were studied for the presence of fluorescent antinuclear antibodies (FANA) (by indirect immunofluorescence on HEp-2 cells) and antibodies to extractable nuclear antigens (anti-ENA) (by immunodiffusion - ID - and counterimmunoelectrophoresis - CIE). In localized disease, antinuclear autoimmunity was limited to 1 FANA positive serum (14%); in systemic disease, the prevalence of FANA was 94% and that of anti-ENA ranged from 29% to 49% (by ID and CIE, respectively). The commonest ENA system, Scl-70, could be easily detected by CIE, in spite of the reported basic nature of the antigen. The anticentromere antibody occurred only in patients with acrosclerosis (7/26-27%), whereas the association of nucleolar + homogeneous FANA, as well as the anti-Scl-70, were found more frequently in diffuse scleroderma (9/9-100% and 6/9-67%, respectively). The presence of the anticentromere antibody excluded that of any anti-ENA, while a close association was found between nucleolar + homogeneous FANA and the anti-Scl-70. Pulmonary involvement was significantly more frequent in nucleolar + homogeneous FANA positive patients; moreover, in two cases the same pattern proved to predict the development of diffuse scleroderma.

Antibodies, Antinuclear↗
PubMed1988

The diagnosis and classification of scleroderma (systemic sclerosis).

Difficulty in the diagnosis of the disease scleroderma may occur at the early stage prior to the development of obvious skin sclerosis. A presumptive diagnosis may be made if Raynaud's phenomenon is accompanied by a positive 'neck test', 'scleroderma' capillary changes in the nailfolds or antinuclear antibodies. Definitive diagnosis may have to be delayed for several years from the onset of Raynaud's phenomenon until definite characteristic skin changes are seen. Ten cases in which an earlier diagnosis of scleroderma was not substantiated are listed. The earlier incorrect diagnosis would have been avoided by use of the methods described in this paper. Various terms have been used to denote subdivisions of scleroderma. These include acrosclerosis, diffuse scleroderma and CREST. We have used the terms Type 1, Type 2 and Type 3 based on the early extent of the skin sclerosis where Type 1 (limited extent) indicates sclerodactyly only, Type 2 (moderate extent) indicates sclerosis proximal to the metacarpophalangeal joints but excluding the trunk and Type 3 (extensive) indicates diffuse skin sclerosis including the trunk. The clinical value of this simple classification is reviewed and contrasted to other classifications which appear to be poorly defined and of limited use.

Adult↗
PubMed1990

Scleroderma overlap syndromes.

Overlap features with diffuse scleroderma are rare. More commonly, other connective tissue diseases have features usually seen in the systemic involvement of scleroderma. The limited form of scleroderma (CREST) has interesting associations with primary biliary cirrhosis, whereas mixed connective tissue disease evolves toward a scleroderma-like picture with advancing years.

Autoantibodies↗
PubMed1986

A cytogenetic analysis of twenty cases of systemic scleroderma.

Cytogenetic studies were performed on 20 patients with diffuse scleroderma who had not received recent or high doses of irradiation; 1,267 cells were examined. Neither the culture medium composition nor the disease had any significant effect on the frequency of structural chromatidic or chromosomal abnormalities.

Adult↗