Systemic Scleroderma

Tuffanelli, Denny L.; Winkelmann, R. K.

doi:10.1001/archderm.1961.01580150005001

Cited by 503 publications

(70 citation statements)

References 19 publications

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“…1 years. Patients were classified, according to Tuffanelli & Winkelmann's criteria [1], into one of the following three groups: GM (n 18), linear scleroderma (n 26), and morphea (n 16). Furthermore, patients with GM were divided into two types: eight patients with multiple plaque type (i.e.…”

Section: Patients and Seramentioning

confidence: 99%

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Autoantibodies to mitochondrial 2-oxo-acid dehydrogenase complexes in localized scleroderma

Fujimoto

Sato

Ihn

et al. 1996

Clinical and Experimental Immunology

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SUMMARYSera from patients with localized scleroderma frequently produce cytoplasmic staining by indirect immunofluorescence, although the antigen remains to be determined. We studied the prevalence, antigen specificity and associated clinical characteristics of anti-cytoplasmic antibodies in localized scleroderma. Serum samples from 60 patients with localized scleroderma were examined by indirect immunofluorescence analysis and immunoblotting. By immunofluorescence analysis on HEp-2 cell substrate, seven of 60 (12%) patients were shown to be positive for anti-cytoplasmic antibodies. Among these, six patients with generalized morphea had anti-mitochondrial antibodies as shown by immunoblotting: they showed reactivity with the E2 component of pyruvate dehydrogenase complex (PDC), with protein X, and with the E2 component of ® -oxo-glutarate dehydrogenase complex, while two of them showed reactivity with PDC-E1®. One of these patients who was positive for anti-PDC-E1® antibody showed laboratory abnormalities, suggesting the presence of primary biliary cirrhosis. The age of disease onset was significantly higher in these six patients than in those without anti-mitochondrial antibodies. Furthermore, five of them were classified into generalized morphea with multiple plaque lesions but without linear lesions (multiple plaque type). These observations suggest that major antigens for anti-cytoplasmic antibodies in patients with localized scleroderma are mitochondrial enzymes, 2-oxo-acid dehydrogenase complexes. Patients with anti-mitochondrial antibodies may comprise a unique subset of localized scleroderma designated multiple plaque type of generalized morphea of older onset.

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Section: Patients and Seramentioning

confidence: 99%

“…The absence of Raynaud's phenomenon, acrosclerosis, and internal involvement differentiates localized scleroderma from systemic sclerosis. Furthermore, the onset of systemic sclerosis is usually in middle age, while localized scleroderma tends to affect children or young adults [1][2][3].…”

Section: Introductionmentioning

confidence: 99%

Autoantibodies to mitochondrial 2-oxo-acid dehydrogenase complexes in localized scleroderma

Fujimoto

Sato

Ihn

et al. 1996

Clinical and Experimental Immunology

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“…1 -3 When cerebral ischemia is a manifestation of scleroderma, it is generally associated with evidence of renal failure and severe hypertension. 4 - 5 We describe a patient with CREST syndrome (a variant of scleroderma characterized by calcinosis cutis, Raynaud's phenomenon, esophageal reflux, sclerodactyly, and telangiectasia) and CNS vasculitis who was treated with high-dose immunosuppressive therapy, resulting in dramatic clinical improvement.…”

Section: Scleroderma and Central Nervous System Vasculitismentioning

confidence: 99%

Scleroderma and central nervous system vasculitis.

Pathak

Gabor

1991

Stroke

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We describe a patient with scleroderma (CREST syndrome) and central nervous system vasculitis. While angjography demonstrated segmental symmetrical arterial narrowing characteristic of vasculitis, results of leptomeningeal biopsy were normal. There was no evidence of systemic vasculitis, renal failure, or malignant hypertension previously thought to be required to explain central nervous system dysfunction in patients with scleroderma. Signs and symptoms attributable to vasculitis were reversible with aggressive immunosuppressive therapy. (Stroke 1991^2:410-413)

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“…Morphologically, localized scleroderma is classified into 3 variants: morphea, linear scleroderma, and generalized morphea (1). Morphea is usually characterized by 1 or a few circumscribed sclerotic plaques with an ivory-colored center and a surrounding violaceous halo.…”

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confidence: 99%

Antihistone antibodies in patients with localized scleroderma

Sato

Ihn

Soma

et al. 1993

Arthritis & Rheumatism

106

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Objective. To study the prevalence and antigen specificity of antihistone antibodies (AHA) in localized scleroderma.Methods. Forty-nine serum samples from patients with localized scleroderma were examined by an enzyme-linked immunosorbent assay (ELISA) and by immunoblotting.Results. By ELISA, AHA were demonstrated in 47% (23 of 49) of patients with localized scleroderma and in 87% (13 of 15) of patients with generalized morphea. Immunoblotting revealed that the predominant antigens were histones H1 and H3. The presence of AHA correlated with that of anti-single-stranded DNA antibody.Conclusion. Some of the major antigens for antinuclear antibodies in patients with localized scleroderma are histones.Localized scleroderma is a connective tissue disorder that is limited to the skin and the subcutaneous tissues underlying the cutaneous lesions. This disease differs from systemic sclerosis in that it is not accompanied by Raynaud's phenomenon, acrosclerosis, or involvement of the internal organs, and the From the

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Systemic Scleroderma

Cited by 503 publications

References 19 publications

Autoantibodies to mitochondrial 2-oxo-acid dehydrogenase complexes in localized scleroderma

Autoantibodies to mitochondrial 2-oxo-acid dehydrogenase complexes in localized scleroderma

Scleroderma and central nervous system vasculitis.

Antihistone antibodies in patients with localized scleroderma

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