Clinical Significance of Antinuclear Antibodies in Japanese Patients with Systemic Sclerosis
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Abstract:
Clinical significance of specific antinuclear antibodies (ANA) in Japanese patients with systemic sclerosis was studied. The patients with systemic sclerosis were classified into four groups according to ANA: (1) anticentromere antibody-positive; (2) anti-Scl-70 antibody-positive; (3) anti-nRNP antibody-positive, and (4) others. The mean score (the number of positive signs in six selected specific signs) of the patients with anti-Scl-70 antibody was significantly higher than those of the other three groups. More frequent contracture of phalanges in the patients with anti-Scl-70 antibody less diffuse pigmentation in the patients with anti-nRNP antibody and less pulmonary fibrosis in the patients with anticentromere antibody were revealed. As shown above the detection of specific ANA in systemic sclerosis is clinically important.Keywords:
Scleroderma (fungus)
Clinical Significance
Abstract We describe a case of systemic scleroderma associated with Graves' disease and review six previously described cases associating the two diseases. Our case seems to be unique in that Graves' disease preceded the occurrence of systemic scleroderma.
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This study aimed to evaluate the differences in the clinical significance of antinuclear antibody (ANA) according to their titers and patterns in the diagnosis of systemic autoimmune diseases (AiD) in pediatric patients. Of the 2442 children who had undergone an ANA test, 473 (19.4%) were positive for ANA, of whom 33 (7.0%) were diagnosed with significant AiD. The positive predictive value (PPV) for significant AiD was considerably high on application of an ANA titer of ≥1:640, and the PPV of a dense fine speckled (DFS) pattern was significantly lower compared with those of speckled and homogenous patterns. The diagnostic value of ANA positivity for AiD is limited, and the clinical significance of the DFS pattern is relatively lower compared with that of other patterns, such as homogenous and speckled patterns, in children. It is necessary to approach the significance of ANA in children individually depending on titers and patterns.
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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.
Scleroderma (fungus)
CREST Syndrome
Immunofluorescence
Systemic scleroderma
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Scleroderma (fungus)
Systemic scleroderma
Immunofluorescence
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Two patients are reported with localized scleroderma who developed thrombocytopenia. One of these patients had a positive antinuclear antibody (ANA) and Anti-DNA antibodies tests. The occurrence of thrombocytopenia in localized scleroderma suggests an autoimmune mechanism, by the response to steroids and the presence of positive autoantibodies. This possible association emphasizes the need to look for hematologic disorders in patients with systemic, as well as localized scleroderma.
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Systemic scleroderma
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AbstractA number of different types of antinuclear antibodies have been characterized, and their clinical significance is beginning to become clear. The anti-DNA type correlates closely with renal injury and severity of clinical disease in systemic lupus erythematosus. Antiribonucleoprotein appears to be related to mixed connective tissue disease.
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Antinuclear antibodies (ANA), including anti‐DNA antibodies, and rheumatoid factors (RAT, Waaler‐Rose) were determined prospectively during a 3‐year period in 40 patients with localized scleroderma (LS) compared with 77 patients with generalized scleroderma (GS). ANA were increased in 25% of patients with LS, and in 47% with GS, anti‐DNA antibodies m 23% of patients with LS, and in 34% with GS. Thus, the anti‐DNA antibody level was lower compared with the known level in systemic lupus erythematosus. Rheumatoid factors were present in 6–7% of patients with LS, and in 14–15% of patients with GS. Increased antinuclear antibodies were not associated with any specific type of localized scleroderma, nor with internal disorders, and no case of clinical overlap to discoid or systemic lupus erythematosus was observed. However, six patients with localized scleroderma and complaints of arthralgia all presented increased antibodies, and one patient showed overlap to rheumatoid arthritis. It is suggested that increased ANA and anti‐DNA antibodies in localized scleroderma, associated with joint manifestations, represents a systemic component m this type of scleroderma, with activation of the immune system and similarities with generalized collagen diseases.
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Abstract. A case is presented of Paget's disease of the breast with underlying infiltrating carcinoma arising in a 35‐year‐old woman with systemic scleroderma. The tumor arose in an area of the skin affected by the systemic scleroderma 4 years after the onset of her systemic disease. The possibility of a causal relationship between these two processes is discussed and a brief review of the literature is presented.
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Systemic scleroderma
Breast carcinoma
Systemic therapy
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Scleroderma (fungus)
Systemic scleroderma
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Abstract When HeLa cells were used as the substrate for detection by the indirect immunofluorescence method, antinuclear antibodies were demonstrated in 16 of 22 (72.7%) sera from patients with localized scleroderma. When mouse kidney sections were used, the positive rate for antinuclear antibodies was 50% (11 of 22). In the 3 subgroups of localized scleroderma, frequencies of anti‐nuclear antibodies on HeLa cells were as follows: morphea, 50% (2 of 4), generalized morphea, 100% (6 of 6), linear scleroderma, 67% (8 of 12). Antibodies to centromere, Scl‐70, nuclear RNP, Sm, and SS‐B antigens were not detected in any patient with localized scleroderma. The high frequency of antinuclear antibodies in localized scleroderma sera suggests that localized scleroderma is a disease which, though different from diffuse scleroderma, also involves an immunologic abnormality.
Scleroderma (fungus)
Morphea
Immunofluorescence
Extractable nuclear antigens
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