PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “Scleroderma, Limited”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 37 records · Page 2Linked to original sources
PubMed1999

Association of clonally expanded T cells with the syndrome of primary biliary cirrhosis and limited scleroderma.

Clinical features of the CREST (calcinosis cutis, Raynaud's syndrome, esophageal dysmotility, sclerodactyly, and telangiectasias) syndrome are sometimes exhibited in patients with primary biliary cirrhosis (PBC), but the postulated autoimmune mechanisms behind these conditions are poorly understood. Clonally expanded T cells may play an important role in disease pathogenesis. In this study, overrepresentation of one T-cell receptor beta chain variable region, TCRBV3, was documented in patients with PBC and/or CREST. Overrepresentation of the TCRBV3 gene mRNA was demonstrated by semiquantitative reverse-transcriptase polymerase chain reaction (RT-PCR). T cells expressing TCRBV3 were analyzed by flow cytometry, were primarily CD8(+), and contained activated cells as assessed by expression of CD69. Clonally expanded T cells within this population were documented by both complementarity determining region 3 (CDR3) length polymorphism analysis and sequencing of T-cell receptor CDR3 cDNA. TCRBV3(+) clonal expansions were stable when followed for up to 5 years. The results of this study demonstrate that the T-cell repertoire of patients with PBC and CREST is characterized by expanded clonal populations of CD8(+) TCRBV3(+) T cells. These clonal expansions provide evidence that stimulation of clonal populations of CD8(+) T cells is associated with the clinical syndrome of PBC with CREST.

Adult↗
PubMed1992

[A case of Hashimoto struma coinciding with limited scleroderma].

In a female patient aged 43 years severe hypothyroidism was caused by Hashimoto struma. She had also cutaneous changes suggestive of collagen disease. On the basis of clinical manifestations, laboratory investigations and histological examinations coexistence was diagnosed of two autoimmunological diseases. Stress is laid on diagnostic difficulties and choice of management.

Adult↗
PubMed2004

Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease.

STUDY OBJECTIVES: To assess the efficacy and safety of continuous subcutaneous infusion of treprostinil, a stable prostacyclin analogue, for treating pulmonary arterial hypertension (PAH) in patients with connective tissue disease (CTD). DESIGN: Two multicenter, randomized, double-blind, placebo-controlled, prospective trials of treprostinil vs placebo in 470 patients with PAH. PATIENTS: A subset of 90 patients with PAH and CTD, including systemic lupus erythematosus, diffuse scleroderma, limited scleroderma, and mixed CTD/overlap syndrome. INTERVENTIONS: Patients received either treprostinil (initiated at 1.25 ng/kg/min, and titrated upward) or placebo via continuous subcutaneous infusion. The maximum dose of treprostinil allowed was 22.5 ng/kg/min. MEASUREMENTS: Six-minute walk (6MW) distance and dyspnea-fatigue scores were determined at baseline, and at 6 weeks and 12 weeks. Hemodynamic measures were obtained at baseline and at 12 weeks. RESULTS: At baseline, most patients had New York Heart Association class III symptoms. The mean baseline 6MW distance was 289 m (range, 60 to 448 m). The mean dose of treprostinil at week 12 was 8.4 ng/kg/min (range, 1.25 to 17.5 ng/kg/min). After 12 weeks, the change in cardiac index from baseline was + 0.2 +/- 0.08 L/min/m(2) in the treprostinil group and - 0.07 +/- 0.07 L/min/m(2) in the placebo group (p = 0.007). The pulmonary vascular resistance index decreased by 4 +/- 2 U x m(2) in the treprostinil group and increased by 1 +/- 1 U x m(2) in the placebo group (p = 0.006). The placebo-corrected median improvement from baseline in 6MW distance was 25 m in treprostinil-treated patients (p = 0.055); this improvement appeared to be dose related. Dyspnea fatigue scores also improved in the treprostinil group compared with the placebo group (p = 0.014). Adverse events included infusion site pain and typical side effects related to prostaglandins, and were tolerated by most patients. CONCLUSIONS: Continuous subcutaneous infusion of treprostinil in patients with PAH associated with CTD improved exercise capacity, symptoms of PAH, and hemodynamics.

Adolescent↗
PubMed2000

The frequency and significance of anticardiolipin antibodies in scleroderma.

OBJECTIVE: To determine the frequency and significance of anticardiolipin antibodies (aCL) in scleroderma. METHODS: We serially tested for aCL in a standardized fashion in patients with scleroderma who were outpatients and gave consent to enter this study. RESULTS: Sixty-three patients participated in this study. Thirty-six had diffuse scleroderma and 27 had limited scleroderma. Three (4.8%) were positive for aCL, of whom 2 had limited scleroderma. In only one patient aCL may have been clinically significant. This woman had limited scleroderma for years and had medium vessel occlusive disease with gangrene eventually requiring midfoot amputations and chronic warfarin treatment. She did not have features of systemic lupus erythematosus or other overlap conditions. The other 2 patients had no manifestations of the antiphospholipid antibody syndrome. CONCLUSION: aCL in scleroderma rarely manifest clinically. The range of frequency of positive aCL in the literature is from 0 to 63%. From our study and the literature, we cannot ascertain if the prevalence is different in limited and diffuse scleroderma.

Adult↗
PubMed2005

N-terminal connective tissue growth factor is a marker of the fibrotic phenotype in scleroderma.

BACKGROUND: Over-expression of connective tissue growth factor (CTGF) is a hallmark of fibrotic disease, including scleroderma. CTGF acts with the pro-fibrotic cytokine TGFbeta to promote sustained fibrotic responses in vivo. Elevated production of CTGF might be responsible for maintenance of the fibrotic phenotype in scleroderma. Assays of CTGF or of its fragments are potential non-invasive measures of the fibrotic response in scleroderma. AIM: To determine the utility of whole, N-terminal, and C-terminal CTGF as surrogate markers for fibrosis in scleroderma. DESIGN: Cross-sectional controlled study. METHODS: Plasma was collected prospectively from 47 scleroderma patients (26 diffuse scleroderma, 21 limited scleroderma) and 18 healthy controls. At the same time, dermal interstitial fluid was derived by a suction blister technique from the lesional skin of scleroderma patients, and from the forearm skin of healthy controls. Whole, N-terminal, and C-terminal CTGF were assayed by ELISA, using monoclonal antibodies specific for N- and C-terminal epitopes. RESULTS: N-terminal cleavage products of CTGF were present at elevated levels in the plasma and dermal interstitial fluid of scleroderma patients, compared to healthy controls. N-terminal CTGF levels in plasma and dermal interstitial fluid correlated with severity of skin disease and (negatively) with disease duration. Whole and C-terminal CTGF levels were low in blister fluid and plasma levels were not elevated in disease. DISCUSSION: These results support a role for CTGF in scleroderma-associated fibrosis and the utility of N-terminal CTGF as a marker of fibrosis.

Adult↗
PubMed1998

Different patterns of endothelial cell activation in renal and pulmonary vascular disease in scleroderma.

Abnormal endothelial cell function is implicated in the development of scleroderma, and in major life-threatening complications of the disease. The nature of the stimulus leading to abnormal endothelial cell function in scleroderma, scleroderma renal crisis, and scleroderma-associated pulmonary hypertension was investigated by measurement of soluble adhesion molecules, shed by activated endothelial cells, in sera from patients with these conditions. In scleroderma renal crisis, mean levels of soluble E-selectin (p < 0.05 limited scleroderma, p < 0.0001 diffuse scleroderma), sVCAM-1 (soluble vascular cell adhesion molecule-1) (p < 0.05 limited scleroderma, p < 0.05 diffuse scleroderma), and sICAM-1 (soluble intercellular adhesion molecule-1) (p < 0.0001 limited scleroderma, p < 0.0001 diffuse scleroderma) were raised, supporting a model of endothelial cell activation in this complication. Evidence for endothelial cell activation in scleroderma-associated pulmonary hypertension was inconsistent, with normal sE-selectin and normal sVCAM-1 in sera from patients with limited scleroderma and pulmonary hypertension. The endothelial cell phenotype in scleroderma-associated pulmonary hypertension may resemble that of unstimulated cells. The pulmonary vascular and renal vascular lesions associated with scleroderma may arise by distinct pathogenic mechanisms.

Acute Kidney Injury↗
PubMed2006

MCTD: is it rare in India?

Mixed connective tissue disease (MCTD) has been rarely reported from India. Thus, we did a retrospective analysis of cases of MCTD seen at our hospital during the last 13 years. We found 16 cases among 441 patients with connective tissue disease. All the 16 patients (15 females) of MCTD fulfilled classification criteria by Kasukawa and at least one of the other two (Sharp's and Alarcon-Sergovia). Raynaud's phenomenon, sclerodactyly, puffy fingers, esophageal hypomotility, and pulmonary disease were the most common manifestations. At a median follow-up of 12 months (1-172), 12 patients developed features of limited scleroderma and three patients had pulmonary hypertension.

Adult↗
PubMed2007

Fine specificity mapping of autoantigens targeted by anti-centromere autoantibodies.

Autoantibodies to centromeric proteins are commonly found in sera of limited scleroderma and other rheumatic disease patients. To better understand the inciting events and possible pathogenic mechanisms of these autoimmune responses, this study identified the common antigenic targets of CENP-A in scleroderma patient sera. Utilizing samples from 263 anti-centromere immunofluorescence positive patients, 93.5% were found to have anti-CENP-A reactivity and 95.4% had anti-CENP-B reactivity by ELISA. Very few patient samples exclusively targeted CENP-A (2.7%) or CENP-B (4.2%). Select patient sera were tested for reactivity with solid phase overlapping decapeptides of CENP-A. Four distinct epitopes of CENP-A were identified. Epitopes 2 and 3 were confirmed by additional testing of 263 patient sera by ELISA for reactivity with these sequences constructed as multiple antigenic peptides. Inhibition CENP-A Western blots also confirmed the specificity of these humoral peptide immune responses in a subset of patient sera. The first three arginine residues (aa 4-6) of CENP-A appear essential for antibody recognition, as replacing these arginines with glycine residues reduced antibody binding to the expressed CENP-A protein by an average of 93.2% (range 80-100%). In selected patients with serial samples spanning nearly a decade, humoral epitope binding patterns were quite stable and showed no epitope spreading over time. This epitope mapping study identifies key antigenic targets of the anti-centromere response and establishes that the majority of the responses depend on key amino-terminal residues.

Adult↗
PubMed2006

The development of connective tissue diseases in patients with autoimmune hepatitis: a case series.

OBJECTIVES: To study the prevalence of connective tissue diseases (CTD) in patients with autoimmune hepatitis (AIH). METHODS: We identified 11 cases of AIH over the past 7 years at our institution, through a systematic chart review of patients with this diagnosis. Their charts were reviewed for the development of systemic CTD. RESULTS: Three of the 11 patients with a definitive diagnosis of AIH developed systemic CTD. All were white: 2 women and 1 man, with an age range of 33 to 62 years, and with disease duration of 1 to 7 years. One patient developed systemic lupus erythematosus (SLE) with vasculitis and peripheral neuropathy. The second developed limited scleroderma and the third developed undifferentiated connective tissue disease (UCTD) and interstitial lung disease. There appear to be shared susceptibility alleles for AIH and CTD in addition to the shared positive autoantibodies. CONCLUSIONS: Patients with AIH may be at increased risk for developing systemic CTD. Conversely, a review of the literature reveals that patients with systemic CTD may be at increased risk of developing AIH. Patients with either AIH or CTD should be monitored for further development of concurrent autoimmune diseases.

Adult↗
PubMedDate not supplied

Psychotic episodes in an elderly woman with an anticentromere-positive scleroderma variant, IgA deficiency, and hypothyroidism.

A 66-year-old woman presented with a recurrent psychotic disorder with central manifestations that initially suggested a delusional disorder. Peripheral stiffness was at first attributed to a dystonic reaction secondary to neuroleptic treatment. The atypical presentation led to suspicion of collagen vascular disease. Both the psychotic disorder and peripheral involvement responded to cortisone. Findings are discussed in light of recent information on the pathogenesis and neuropsychiatric manifestations of systemic lupus erythematosus, progressive systemic scleroderma and limited scleroderma variants (CREST, unclassifiable connective tissue disease) with positive anticentromere titers, including a discussion of the vasculitis hypothesis of collagen vascular cerebral involvement.

Aged↗
PubMed2000

Pulmonary hypertension with limited cutaneous scleroderma (CREST syndrome).

A patient is described with a typical manifestation of pulmonary hypertension associated with limited cutaneous scleroderma, also known as CREST syndrome. The patient was treated with a calcium antagonist, oral anticoagulation and, because of evidence for parenchymal inflammation of the lung, with low-dose prednisone and cyclophosphamide. This treatment resulted in initial improvement of diffusion capacity and exercise tolerance, however, 1 year after diagnosis the patient died of progressive pulmonary hypertension.

Angiography↗
PubMed1999

Systemic sclerosis and antineutrophil cytoplasmic autoantibody-associated renal failure.

We report three patients who developed antineutrophil cytoplasmic autoantibody (ANCA)-associated crescentic glomerulonephritis, two of whom showed clinical features of limited scleroderma and one whose results of serological tests were suggestive of limited scleroderma without cutaneous features. All had anticentromere antibodies and antimyeloperoxidase antibodies. No patient showed the features of typical scleroderma renal crisis such as accelerated hypertension or microangiopathy. Our patients were normotensive at the time of onset of renal failure, and the clinical picture was characterised by only modest features of limited scleroderma. All three patients had crescentic glomerulonephritis at various stages of chronicity. One patient responded to immunosuppressive therapy with improvement in renal function; the other two patients rapidly developed end-stage renal failure. These patients and others recently described may represent a newly described form of scleroderma renal disease.

Aged↗