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PubMed1990

[Electrocardiographic disorders in diverse clinical variations of scleroderma].

The electrocardiograms of 130 patients affected by scleroderma, 117 with systemic sclerosis (67 with diffuse and 50 with limited form) and 13 with localized scleroderma were analyzed. ECG was normal in 44.6% of cases, respectively in 39.3% with systemic sclerosis and in 92.3% with localized scleroderma. The more frequent electrocardiographic abnormalities were supraventricular and ventricular premature beats, right bundle branch block, left anterior hemiblock, low QRS voltages, ST-T wave abnormalities and Q or QS aspects. All the abnormalities above resulted to be more frequent in the patients with diffuse systemic sclerosis in comparison to the patients with limited systemic sclerosis. Our results confirm that the cardiac involvement appears to be much more serious in the diffuse systemic sclerosis.

Adolescent↗
PubMed1987

Systemic sclerosis (scleroderma): clinical, genetic, and serologic subsets.

Immunogenetic markers, autoantibodies, and clinical features were studied in 47 patients, 35 Caucasian and 12 black, with systemic sclerosis. Twenty-two had generalized scleroderma, while 25 had limited skin involvement. HLA-DR1 (RR = 2.1, p = 0.08) and DR5 (RR = 2.1, p = 0.08) were increased in Caucasian patients vs controls as was the supertypic specificity HLA-DRw52 (RR = 2.8, p = 0.02, pc = 0.04). HLA-DR6.1 was increased in black patients vs controls (RR = 15.4, p = 0.008, pc = 0.088). There were no significant increases in any of the complement allotypes in either racial group. Anticentromere antibody was noted in 10 patients, all Caucasian; 7 had limited disease. Anti-Scl-70 was noted in 4 patients; all had generalized disease (p = 0.036). HLA-DR2 was present in all anti-Scl-70 positive patients (RR = 22.5, p = 0.006). Our results suggest that clinical subsets of systemic sclerosis can be defined by genetic and serological markers.

Adult↗
PubMed2005

Incidence and clinical correlation of anticentromere antibody in Thai patients.

Anticentromere antibodies (ACA) are useful in assessing and classifying patients with mild variant of systemic sclerosis called calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias (CREST) syndrome. From their prognostic significance, we are interested in the prevalence and disease correlation in Thai patients. A total of 3,233 serum samples of patients with any musculoskeletal symptoms were sent for antinuclear antibody determination at Ramathibodi Immunology Laboratory Service between the years 1998 and 2001. Forty sera (1.23%) were ACA positive. These sera were from 27 patients with autoimmune diseases and 13 with nonautoimmune diseases. Among autoimmune group, scleroderma was the most common diagnosis (33.3%) with limited sclerosis being the most frequent variant. The percentages of autoimmune disease were almost the same among the low-titer (1:40) and the high-titer (1:640) groups. The study suggests that the prevalence of ACA in Thai patients is low. The presence of ACA detected in patients with vague musculoskeletal symptoms does not suggest a diagnosis of CREST syndrome. Even high-titer ACA can be found in nonautoimmune diseases.

Adult↗
PubMed1991

[Diabetic hand syndrome in juvenile diabetes].

Patients with Type I diabetes may develop a scleroderma-like syndrome, including limitation of joint mobility. This syndrome, cherioarthropathy, is considered a complication of diabetes, but its cause is unknown. We examined 30 Jewish and 13 Arab patients in our juvenile diabetes clinic for skin and joint involvement. Signs of cherioarthropathy in both hands were found in 13 children (30.2%); all had skin changes and 6 (13.5%) also had articular involvement of the hands. There was no correlation between the presence of cherioarthropathy and the patient's age or the duration of diabetes. The syndrome was significantly more frequent among Arabs (8/15, 61.5%) than Jewish children (5/30, 16.6%), p less than 0.01. There was a indirect correlation between incidence of cherioarthropathy and adequacy of glycemic control, but no difference in glycemic control between Arab and Jewish children. This may indicate a genetic factor in the development of cherioarthropathy in juvenile diabetes.

Child↗
PubMed2003

[Pulmonary artery hypertension associated with connective tissue diseases].

MOST IMPORTANT: Pulmonary hypertension (PH) is a severe, potentially life-threatening complication of connective tissue diseases, among which scleroderma is first line. The aim of this paper was to review the literature and report our experience with this particular complication of connective tissue diseases. In our centre of pulmonary vascular diseases, connective tissue diseases represent the third cause of PH. RESULTS: Scleroderma and particularly its limited cutaneous variant, the CREST syndrome, is the most common connective tissue disease affected by pulmonary hypertension. It can be related to a specific lung parenchymal involvement (hypoxic PH), to an isolated pulmonary vascular involvement or to a cardiac dysfunction secondary to specific myocardial lesions. DIAGNOSIS: Echocardiography is an excellent examination to detect pulmonary hypertension. However, right heart catheterisation is necessary to confirm the diagnosis of pulmonary hypertension and to test vasoreactivity with a potent vasodilator such as nitric oxide (NO). REGARDING TREATMENTS: Oral calcium channel blockers are indicated in patients who are responders to acute NO tests. Treatment with continuous intravenous prostacylin is obviously an improvement, at least functionally, although it appears less effective than in primary PH. With the new subcutaneous, oral and inhaled vasodilatators (prostaglandin and endothelin receptor antagonists), a few cases of improvement of PH with intensive immunosuppressive therapy were observed, essentially during systemic lupus erythematosus and Sharp syndrome. IN PRACTICE: PH is a severe complication of connective tissue diseases. Early detection of this complication should allow an earlier and more aggressive therapeutic approach in these patients, before irreversible vascular lesions occur.

Adult↗
PubMed2005

[Systemic sclerosis--clinical course and treatment possibilities].

Systemic sclerosis is an autoimmune disease, characterized by fibrosis of the skin and internal organs, such as esophagus, heart, lungs, and kidneys. Three main forms of systemic sclerosis are conspicuous: diffuse systemic sclerosis, limited systemic sclerosis and scleroderma sine sclerosis. Respective variants differ in skin sclerosis exacerbation, progress and prognosis; whereas organ involvement, osseous and articular alterations are comparable in all forms of the disease. Due to the fact that etiology of systemic sclerosis is not known, no specific therapy has been developed. The therapy is based on influencing main pathogenetic factors, such as peripheral circulation disturbances, abnormal immune activation and increase in collagen accumulation. An overview of clinical course of systemic sclerosis and new therapeutic modalities is provided.

Connective Tissue↗
PubMed2000

Lung transplantation and systemic sclerosis.

We performed lung transplantation in nine patients with Scleroderma related lung disease. Patient characteristics included: 7 (78%) females, 6 (67%) with limited and 3 (33%) with diffuse Scleroderma. Pulmonary fibrosis was present in 7 (78%) and pulmonary hypertension in 4 (44%). All patients were carefully screened by the Johns Hopkins and University of Maryland Scleroderma Center and only referred for transplantation when concomitant renal insufficiency (creatinine clearance < or = 50 ml/min), aspiration, and skin brakdown were excluded. When compared to a similar group of transplant patients with nonscleroderma lung disease (primary pulmonary fibrosis), there was no significant difference in post-transplant survival at four years (76.2 +/- 0.15% vs. 69.2% +/- 0.12%), mean annual incidence rate for acute rejection (0.14 +/- 0.14 vs. 0.47 +/- 0.13) and infection (viral 0.17 +/- 0.17 vs. 0.29 +/- 0.11) (bacterial 0.17 +/- 0.17 vs. 1.4 +/- 0.4) (fungal 0.99 +/- 0.69 vs. 0.36 +/- 0.16) or serum creatinine (1.55 +/- 0.34 mg/dl vs. 1.15 +/- 0.09 mg/dl). We conclude that lung transplantation is viable option for carefully selected patients with scleroderma related lung disease.

Contraindications↗
PubMed1994

Familial CREST syndrome.

The familial occurrence of scleroderma is uncommon particularly the limited (CREST) form. We describe 2 families in which such an association occurred. Family pedigree 1 consists of 2 of 3 sisters with CREST scleroderma. Both affected sisters shared HLA types and C4 allotypes including DR5, found more frequently in patients with scleroderma. The unaffected sister did not share this MHC allele. Family pedigree 2 includes a grandmother and grandson with CREST scleroderma as well as a family member with Raynaud's phenomena alone. We conclude that familial occurrence of scleroderma may be associated with shared class II MHC antigens.

Adult↗
PubMed2002

Molecular aspects of scleroderma.

Scleroderma is a complex disease characterized by activation of the immune system, small-vessel vasculopathy, and fibrosis of the skin and other organs. This review is limited to the discussion of scleroderma fibroblast biology and the cellular and molecular mechanisms that contribute to the abnormal deposition of collagen. Selected aspects of abnormal extracellular matrix (ECM) regulation by scleroderma fibroblasts are critically reviewed. These include the role of transforming growth factor-beta (TGF-beta) and connective tissue growth factor (CTGF) and their receptors in the fibrotic process in scleroderma and the overview of the transcription factors involved in regulation of the human alpha2 (I) collagen (COL1A2) gene.

Animals↗
PubMed2000

Autologous hemopoietic stem-cell transplantation for children with refractory autoimmune disease.

Autologous stem cell transplantation (ASCT) has been proposed as a possible treatment for severe autoimmune diseases such as rheumatoid arthritis (RA), multiple sclerosis (MS), systemic sclerosis, and systemic lupus erythematosus (SLE). To date, more than 250 patients with various autoimmune disorders have undergone an ASCT since 1996. Among them, there is a very limited number of children. This review summarizes the experience with ASCT for pediatric rheumatic diseases. Most reported cases concern juvenile idiopathic arthritis (JIA). Experience with ASCT for childhood SLE, Scleroderma, or Dermatomyositis is very limited. To date, 12 children with severe systemic or polyarticular JIA, all with progressive disease activity despite the use of corticosteroids, MTX, CsA, or Cyclophosphamide were treated in our center with ASCT. Rheumatologic follow-up at 3-month intervals up to 36 months showed a marked decrease in arthritis severity as expressed by the core-set criteria for juvenile chronic arthritis (JCA) activity. However, these children remain at risk for severe viral infections due to the prolonged lymfopenia. ASCT in this severely ill patient group induces a very significant and drug-free remission of the disease, but carries a significantly risk of developing fatal MAS.

Adolescent↗
PubMed2003

Fabrication of a sectional impression tray and sectional complete denture for a patient with microstomia and trismus: a clinical report.

This article describes techniques used to fabricate mandibular and maxillary sectional trays and a folding maxillary complete denture for a patient with limited oral opening caused by scleroderma. For the foldable denture, the anterior teeth had to be arranged on a second base and the hinge fitted at a location higher than the denture base. These 2 factors increased the thickness of the denture and limited the volume of the tongue. Nevertheless, a single-piece denture base provided the patient with ease in placement and removal of the denture.

Dental Impression Technique↗
PubMed1984

[Clinical value of the estimation of plasma converting enzyme activity].

Plasma angiotensin I-converting enzyme "activity" (CEA) was estimated as its enzymatic effect on the synthetic substrate HHL in normal subjects and patients with untreated sarcoidosis, alcoholic decompensated liver cirrhosis and scleroderma. CEA was above the upper limit of normal in 60% of sarcoidosis cases and 30% of cirrhotics; it was within normal in scleroderma. The assessment of the influence of chloride concentration on CEA showed that maximum was obtained for a concentration of 300 mM. In addition the inhibitory effect of angiotensin I and the converting enzyme inhibitors SQ 14225 and MK 422 was demonstrated together with the action of high concentrations of penicillamine. The inhibitory influence of these substances was similar when added to the plasma of normal or sarcoidosis subjects.

Adult↗
PubMed2000

Low-dose broad-band UVA in morphea using a new method for evaluation.

Until recently, various therapies for localized scleroderma have been used with limited success. Recently, phototherapy, with or without psoralen, was proposed as a successful treatment modality. The aim of this study was to evaluate the effect of broad-band low-dose ultraviolet A (UVA) phototherapy in patients with localized scleroderma, using a new method for evaluation. Twelve patients complaining of morphea were exposed to UVA irradiation at a dose of 20 J/cm2 3 times per week for 20 sessions. Selected covered plaques served as internal controls. The efficacy of therapy was judged clinically by sequential inspection and palpation. In biopsy specimens from exposed and covered plaques stained with hematoxylin and eosin (H & E) and Masson trichrome stains, the concentration of collagen per dermal surface area was measured with the use of a computerized image analyzer. All patients reported remarkable softening of skin lesions, confirmed by sequential palpatory assessment. A significant reduction in the mean concentration of collagen per surface area was detected in the plaques exposed to UVA (the P value being 0.007, P<0.01), whereas in the covered plaques the difference was not statistically significant (the P value being 0.10, P>0.05). The conclusion is that low-dose broad-band UVA phototherapy is a very effective and safe treatment modality for localized scleroderma.

Adolescent↗
PubMed1989

Marker antibodies in scleroderma and polymyositis: clinical associations.

Sera of 34 patients with progressive systemic sclerosis and of 11 patients with polymyositis/dermatomyositis (PM/DM) were analyzed by the immunoblotting technique for the presence of marker antibodies. The presence of anti-centromere, anti-Topoisomerase-I (anti-Topo-I) and anti-Jo-1 antibodies was found to be highly specific for the CREST syndrome, diffuse scleroderma and PM/DM, respectively, but only of limited sensitivity (78, 44 and 45%, respectively). Anti-Topo-I positive diffuse scleroderma patients had a more severe disease (digital pitting scars and renal insufficiency) than anti-Topo-I negative diffuse scleroderma patients. Anti-Jo-1 was associated with interstitial lung disease. Longitudinal studies showed a constant antibody pattern. Our results confirm the clinical usefulness of these marker antibodies.

Antibody Specificity↗
PubMed1992

The absence of factor B activation products in scleroderma.

Previous investigators have reported the presence of complement activation products in scleroderma. Using an assay consisting of isoelectric focussing followed by immunofixation (IEF/IF) for alternative pathway activation product Ba, and an ELISA for C3d that we developed for evaluating lupus plasmas, we assayed 48 plasmas from patients with diffuse cutaneous scleroderma, 16 patients with the limited CREST variant, 2 patients with mixed connective tissue disease (MCTD) and 4 patients with Raynaud's disease. Ba was not detected in any patient's plasma. Only one plasma from a patient with CREST contained elevated levels of C3d. We cannot reconcile the absence of B activation products in our patients with scleroderma with the results of the previous report, and conclude that further studies are necessary.

Adult↗
PubMed2000

Systemic sclerosis sine scleroderma: demographic, clinical, and serologic features and survival in forty-eight patients.

OBJECTIVE: To describe the demographic, clinical, and laboratory features and natural history of patients with systemic sclerosis sine scleroderma (ssSSc), and to compare them with those of patients with SSc and limited cutaneous involvement (IcSSc). METHODS: The University of Pittsburgh Scleroderma Databank served as the data source. Patients were divided into those who had no skin thickening (ssSSc) and those who had skin thickening only distal to elbows or knees and/or of the face (IcSSc) during their disease course. These two groups were compared with regard to demographic characteristics, clinical, laboratory, and serologic features, and survival rates. Chi-square and Student's t-test analyses were performed, and Fisher's exact test was used as appropriate. RESULTS: Of 555 consecutive patients without diffuse cutaneous SSc, 48 (9%) had ssSSc and 507 (91%) had IcSSc. The ssSSc patients had a mean total disease duration of 18.6 years (15.1 years before study entry and 3.5 years of followup after study entry), and had not developed IcSSc or another connective tissue disease. Other than the absence of skin thickening, the ssSSc group had no significant differences in individual internal organ involvements, laboratory features, serum autoantibody type, or survival rate compared with patients with IcSSc. Within the category of lung involvement, patients with ssSSc had a significantly greater frequency of dyspnea with mild exertion or at rest, and a tendency toward reduced carbon monoxide diffusing capacity (<70% of predicted normal) and primary pulmonary arterial hypertension. Patients with IcSSc had significantly more frequent individual manifestations of digital pitting scars, digital-tip ulcers, telangiectasia, and calcinosis than those with ssSSc, in part related to increased time of observation. Puffy fingers and finger joint contractures were detected significantly more often in IcSSc patients. CONCLUSION: Systemic sclerosis sine scleroderma should be included in the spectrum of SSc with limited cutaneous involvement and should not be considered a distinct or separate disorder.

Adolescent↗