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Results for “Scleroderma, Systemic”

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PubMed1979

Biogenic amines derived from tryptophan in systemic and cutaneous scleroderma.

Blood levels of serotonin (5-HT) and urinary excretion of 5-hydroxyindole-acetic acid (5-HIAA), tryptamine (T), and indole-acetic acid (IAA) were determined in 39 cases of systemic scleroderma and in 7 cases of very severe cutaneous scleroderma. The T/IAA ratio was normal and serotonin elevated in mild and rather severe acrosclerosis alike, i.e. in systemic scleroderma with pronounced vascular involvement. The T/IAA ratio was increased and serotonin normal in severe acroscleroderma, representing an intermediate form or transition to diffuse scleroderma, in severe diffuse scleroderma, and in severe cutaneous scleroderma. The T/IAA ratio and serotonin level were both elevated in a few cases of systemic scleroderma and in severe generalized morphea with concomitant vascular changes. The findings suggest impaired monoamine oxidase (MAO) activity in scleroderma with consequent accumulation in the organism of biogenic amines derived from tryptophan. An increased T/IAA ratio seems to be of prognostic significance in scleroderma, suggesting an adverse course of the disease.

Colorimetry↗
PubMed2000

A case of linear scleroderma and myasthenia gravis.

We report a case of a 28-year-old woman with myasthenia gravis who developed linear scleroderma seven years later. Myasthenia gravis and scleroderma are rarely found in direct association with each other; there are only five such reported cases, all of which were systemic scleroderma patients. Although localized and systemic scleroderma are distinct entities, autoimmunity is believed to be involved in the pathogenesis of both. Myasthenia gravis and scleroderma may occur coincidentally, but an autoimmune predisposition seems to be the more likely underlying cause, as evidenced by an increased incidence of autoantibodies and autoimmune diseases.

Adult↗
PubMed1998

Localized scleroderma.

Localized scleroderma can be divided into three main subtypes: morphea, linear scleroderma, and generalized morphea. Plaque morphea usually has a good prognosis. Variants of morphea, including guttate morphea and atrophoderma of Pasini and Pierini, are seen. Linear scleroderma, whether involving an extremity or the face, is often associated with serological abnormalities. Cosmetic and functional prognosis may be poor. Therapy is usually ineffective. Generalized morphea may be difficult to differentiate from systemic scleroderma. However, progression to systemic scleroderma is uncommon.

Adult↗
PubMed1991

Altered T cell subpopulations and lymphocytes expressing natural killer cell phenotypes in patients with progressive systemic sclerosis.

Scleroderma (progressive systemic sclerosis [PSS]) is known to be associated with abnormal T cell immunoregulation. In the present study, we evaluated lymphocyte phenotypes in patients with PSS and normal control subjects by flow cytometry and monoclonal antibodies for total T (CD3), T suppressor (CD8), T helper (CD4), T helper-inducer (CDw29), T suppressor-inducer (CD45R), human leukocyte antigen, DR+B (CD19), DR+T, and natural killer subsets, HNK-1 (CD57) and NKH-1 (CD56) cells. Patients with PSS compared to normal subjects had significantly lower percentages of CD3+ (p less than 0.005) and CD8+ (p less than 0.05) (similar to several patients with rheumatoid arthritis also evaluated), as well as CD45R (p less than 0.05), T+DR+ (p less than 0.05), and NKH-1 (CD56) (p less than 0.0005) cells. Patients with PSS with late-limited or generalized disease had lower percentages of CD8+, CD19, NKH-1+, and CDw29, but higher percentages of CD4+, HNK-1, and CD45R cells compared to patients with early stage disease, but these results were not statistically significant. These unique alterations in patients with PSS may prove to be useful in monitoring the stage of disease activity for therapy and further define immunologic defects.

Adult↗
PubMed1995

Elevated levels of platelet derived growth factor and transforming growth factor-beta 1 in bronchoalveolar lavage fluid from patients with scleroderma.

OBJECTIVE: Systemic sclerosis (scleroderma, SSc) frequently affects the lungs, and interstitial pulmonary fibrosis is one of its major complications. The pathophysiology of SSc lung disease s poorly understood, but recent studies document an inflammatory process resembling that of idiopathic pulmonary fibrosis with increased numbers of activated alveolar macrophages and granulocytes in bronchoalveolar lavage (BAL) fluid). We determined levels of 2 potentially important mediators of fibroproliferative repair in BAL fluid from patients with SSc. METHODS: Using Western blot and ELISA techniques we measured levels of platelet derived growth factor (PDGF) and transforming growth factor-beta (TGF-beta in BAL fluid from patients with SSc and healthy controls. The mitogenic effect of these cytokines on SSc lung myofibroblasts was determined by [3H]thymidine incorporation. RESULTS: SSc BAL fluid contains significantly elevated levels of PDGF-AA and PDGF-BB. Where TGF-beta 1 was significantly elevated in SSc lavage fluid, the amount of TGF-beta 2 was significantly less than that observed in normal lavage fluid. Myofibroblasts cultured from SSc lavage fluid exhibited enhanced [3H]thymidine incorporation upon exposure to the growth factors present in SSc BAL fluid: PDGF and TGF-beta 1. SSc lung myofibroblasts pretreated with TGF-beta 1 exhibited an enhanced mitogenic effect upon stimulation by PDGF, due in part to the induction of the PDGF alpha receptor. CONCLUSION: Our studies support a role for PDGF and TGF-beta 1 in the pathogenesis of SSc lung disease.

Bronchoalveolar Lavage Fluid↗
PubMed2006

Fibulin-4 is a target of autoimmunity predominantly in patients with osteoarthritis.

Autoimmunity to chondrocyte-producing proteins has been reported in patients with osteoarthritis (OA) as well as in those with rheumatoid arthritis (RA). To answer whether or not OA-specific autoimmunity exist, we performed screening of chondrocyte-producing autoantigens by two-dimensional electrophoresis and Western blotting with each of 20 OA and 20 RA serum samples. We identified an apparently OA-specific autoantigen spot with a molecular mass of 52 kDa and a Isoelectric point of 4.1 as fibulin-4 by mass fingerprinting. By preparing recombinant proteins of fibulin-4, we determined prevalence of the autoantibodies to fibulin-4 in 92 patients with OA, 67 patients with RA, 40 patients with systemic lupus erythematosus, and 43 patients with systemic scleroderma. As a result, the IgG type anti-fibulin-4 autoantibodies were detected in 23.9% of sera from patients with OA, in 8.9% of sera from patients with RA, in 2.5% of sera from patients with systemic lupus erythematosus, and in 9.3% of sera from patients with systemic scleroderma. Furthermore, we immunized DBA/1J, ICR, BALB/c, and C57BL/6 mice with the recombinant fibulin-4 proteins to investigate arthritogenecity of fibulin-4. As a result, mild synovitis was detected in all of the four strains. In addition, we demonstrated expression of fibulin-4 in chondrocytes at both mRNA and protein levels in vivo and in vitro by RT-PCR, Western blotting, and immunohistochemistry. Taken together, fibulin-4, expressed in chondrocytes and recognized as an autoantigen mainly in OA rather than in RA, may play pathogenic roles in OA.

Adult↗
PubMed1994

Clinical aspects of systemic and localized scleroderma.

Continued efforts are being made to better define the clinical course, disease subsets, and predictors of outcome in scleroderma. Data suggest that the course of the skin disease is triphasic, with the most active thickening phase in the first 12 months of disease. The presence of specific autoantibodies may predict clinical course more precisely than any clinical feature. Antipolymerase I, II, and III antibodies seem specific for scleroderma and, if present, may predict aggressive disease. Early detection of lung involvement provides an opportunity to select patients who may be responsive to drug treatment. Renal crisis in scleroderma is still important and may occur in the absence of significant signs of cutaneous fibrosis. Renin plasma levels do not appear helpful in predicting renal crisis. Significant gastrointestinal reflux disease with delayed acid clearance and esophagitis is associated with aperistalsis of the lower esophagus. Evidence for widespread structural and functional abnormalities of the microvascular circulation have been reemphasized. The psychosocial impact of scleroderma has been studied, demonstrating both the importance of depression and the need for social support. The etiology of localized scleroderma remains unknown despite efforts to link these lesions to Borrelia burgdorferi infection.

Autoantibodies↗