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PubMed1993

Myocarditis as a complication in scleroderma patients with myositis.

This report describes six diffuse scleroderma (PSS) patients with skeletal muscle myositis accompanied by severe myocarditis, diagnosed by CPK-MB elevation in conjunction with severe left ventricular (LV) hypokinesis. Although the myositis improved with steroid therapy in all patients, those treated with steroids alone died due to progressive LV failure. This experience suggests that the LV dysfunction in PSS patients with myositis may have an inflammatory component. Since the myocarditis may not be clinically apparent initially, it is suggested that CPK-MB fractionation and studies of LV function are undertaken in all PSS patients with myositis. The optimal treatment of this disorder, however, has yet to be determined.

Adult↗
PubMed2003

Systemic and cell type-specific gene expression patterns in scleroderma skin.

We used DNA microarrays representing >12,000 human genes to characterize gene expression patterns in skin biopsies from individuals with a diagnosis of systemic sclerosis with diffuse scleroderma. We found consistent differences in the patterns of gene expression between skin biopsies from individuals with scleroderma and those from normal, unaffected individuals. The biopsies from affected individuals showed nearly indistinguishable patterns of gene expression in clinically affected and clinically unaffected tissue, even though these were clearly distinguishable from the patterns found in similar tissue from unaffected individuals. Genes characteristically expressed in endothelial cells, B lymphocytes, and fibroblasts showed differential expression between scleroderma and normal biopsies. Analysis of lymphocyte populations in scleroderma skin biopsies by immunohistochemistry suggest the B lymphocyte signature observed on our arrays is from CD20+ B cells. These results provide evidence that scleroderma has systemic manifestations that affect multiple cell types and suggests genes that could be used as potential markers for the disease.

Adult↗
PubMed1986

A cytogenetic analysis of twenty cases of systemic scleroderma.

Cytogenetic studies were performed on 20 patients with diffuse scleroderma who had not received recent or high doses of irradiation; 1,267 cells were examined. Neither the culture medium composition nor the disease had any significant effect on the frequency of structural chromatidic or chromosomal abnormalities.

Adult↗
PubMed2006

Mycophenolate mofetil as first-line treatment improves clinically evident early scleroderma lung disease.

OBJECTIVE: To find an effective, safe immunosuppressive regimen as an alternative to cyclophosphamide (Cy) for the treatment of clinically evident diffuse scleroderma (dSSc)-associated alveolitis of recent onset. METHODS: Five consecutive patients with dSSc and recent-onset alveolitis were enrolled and treated with mycophenolate mofetil (MMF) and small (< or =10 mg/day) doses of predinisolone in this open-label trial. One patient with long-standing fibrosing alveolitis was later added to our cohort. Pulmonary function tests [carbon monoxide diffusing capacity (DLCO) and forced vital capacity (FVC)], pulmonary high-resolution computed tomography (HRCT) scans and clinical assessment were performed before and at specified time-points after enrolment. Cases of significant infections, leucopenia and abdominal pain were recorded. RESULTS: After 4-6 months of MMF therapy, DLCO improved significantly compared with pre-treatment (mean DLCO 75.4% vs 64.2% of predicted value, respectively, P = 0.033). Values of FVC also improved, with the difference almost reaching levels of statistical significance (mean FVC 76.2% vs 65.6% of predicted value, P = 0.057). Ground glass opacities cleared in three of four patients with recent-onset alveolitis and were reduced in one patient after 6-8 months of treatment. Breathlessness and cough improved by 3 months. A possible treatment failure was seen in one patient. However, in five patients functional and clinical improvement was sustained during the study period. No adverse events were recorded in this ongoing clinical trial. CONCLUSION: Our preliminary data suggest that in patients with dSSc and recent, clinically apparent alveolitis, early treatment with MMF and small doses of corticosteroids (CS) may represent an effective, well-tolerated and safe alternative therapy.

Adolescent↗
PubMed1997

[Association of amyotrophic lateral sclerosis with scleroderma. Study of 2 cases].

We report a clinical association of diffuse scleroderma and amyotrophic lateral sclerosis (ALS) in two patients. Scleroderma was diagnosed on skin, digestive, osteoarticular, pulmonary lesions and inflammatory syndrome. ALS was suspected on the association of diffuse amyotrophy, fasciculations, pyramidal tract involvement and electrophysiological data. Chronic medulla ischemia and or immune abnormalities are proposed as potential pathological mechanisms for ALS but fortuitous association can not be excluded.

Amyotrophic Lateral Sclerosis↗
PubMed1990

[Scleroderma].

Sclerodermas may occur in two basic forms: localized sleroderma (LSc) and systemic scleroderma (SSc). Pseudoscleroderma as well as overlap syndromes have to be differentiated from these two variants. From the clinical point of view, localized scleroderma can be subdivided into type I = plaque-like LSc (= morphea), type II = linear LSc, and type III = deep LSc. According to the degree of the cutaneous involvement, systemic scleroderma can likewise be classified into type I = sclerodactylia, type II = acrosclerosis, and type III = scleroderma with primary involvement of the trunk (diffuse scleroderma). In LSc, we never find systemic involvement; SSc, in contrast, is almost always associated with Raynaud's phenomenon, changes of the esophagus, as well as an increased titer of antinuclear antibodies (Hep-2 cell test). Only 23% of our patients with LSc showed elevated ANA titers. We present and discuss data of 56 patients with LSc and 52 patients with SSc. Evidence in the literature as well as our own findings suggest that the pathogenesis of LSc is different from that of SSc. The influence of various mediators and cytokines on the collagen metabolism might be regarded as a theoretical approach in order to develop new therapeutic regimens. This is even more important since there is still no efficient mode of treatment for neither localized nor systemic scleroderma.

Antibodies, Antinuclear↗
PubMed2005

Scleroderma in Australian aborigines.

Scleroderma (systemic sclerosis) has not been reported before in Australian Aborigines. We describe in detail a community middle-aged Aboriginal woman whose diffuse scleroderma terminated fatally with a renal crisis. Moreover, we have identified a further five Aboriginal patients on the South Australian Scleroderma Register (two with diffuse, two with limited and one with overlap scleroderma), a number consistent with that expected from the 2001 census data for our state. However, an analysis of all antinuclear antibody (ANA) requests from the Top End of Australia over a 6-year period revealed only two Aborigines with low titre anticentromere antibody (despite frequent occurrence of ANA with other specificities). Neither of these Aborigines had features of scleroderma. In conclusion, scleroderma does occur in indigenous Australians but further studies are needed to confirm the apparent infrequency of centromere-associated limited scleroderma (which is the commonest form of scleroderma in our Caucasian population).

Antibodies, Antinuclear↗
PubMed1983

Antinuclear antibodies in localized scleroderma.

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 antinuclear 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 patients 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.

Adolescent↗
PubMed1985

Scleroderma and pregnancy. Anaesthetic considerations.

The case of a pregnant patient with diffuse scleroderma who died following Caesarean section under general anaesthesia is presented. The patient's postoperative course was complicated by pulmonary oedema and pulmonary hypertension, sepsis, thrombocytopenia and renal failure. Aspects of the disease which possess anaesthetic implications are reviewed.

Adult↗
PubMed1980

[Endocrine changes in gestagen treatment in a patient with scleroderma diffusa].

In a male patient, treated with gestagens and corticosteroids for diffuse scleroderma, endocrine alterations over two years are followed. A decrease in gonadotrophin plasma levels and an increase of prolactin is observed. Levels of testosterone, DHEA, and 17-alpha-hydroxyprogesterone are lowered, while those of estradiol and androstenedione remain unchanged. The alterations may be explained by a suppression of pituitary function and following decrease of testicular (and adrenal) steroid secretion.

Adult↗
PubMed1995

Chromosome changes in lymphocytes of patients with scleroderma.

The authors have analyzed cytogenetically 28 cultured lymphocytes from females with Diffuse Scleroderma and 28 female controls between 30 and 70 years of age. Recurrent chromosome abnormalities were +8, +X, -X, and the PCD(X) phenomenon. Triplo X cells were significatively more frequent in patients than in controls. The incidence of +X and PCD(X) was significatively higher in the patients between 30 and 50 years of age, while the frequency of -X cells was higher in controls than in patients. None of these chromosome changes was correlated with the presence of anticentromere antibodies (ACA) in the patients' serum. Random structural chromosome abnormalities were also observed in the patients, but no break point clustering was observed. The incidence of chromosome breaks was significatively higher in patients than in controls. These data suggest a general tendency of females with Scleroderma to develop X polisomies and +X and the PCD(X) phenomenon may be considered Scleroderma related in younger patients.

Adult↗
PubMed2002

Scleroderma overlap syndromes.

Scleroderma is a connective tissue disease that causes fibrosis and vascular abnormalities, but that also has an autoimmune component. Many patients with scleroderma have a positive antinuclear antibody, and there can be family histories of other connective tissue diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis. Some patients have features of scleroderma and other autoimmune conditions. This article will review recent literature to help in the understanding of scleroderma with overlap features. Recent reports of scleroderma overlap with rheumatoid arthritis suggest distinct features of diffuse scleroderma with positive Scl-70, pulmonary fibrosis, and later seropositive erosive rheumatoid arthritis. SLE rarely occurs with scleroderma. Sjögren syndrome symptoms are common in scleroderma. In primary Sjögren syndrome, anticentromere antibody positive patients have more Raynaud phenomenon. Antibodies that occur in scleroderma that are thought to be specific are present in other connective tissue diseases. For instance, Scl-70 antibody is reported in as many as 35% of patients with scleroderma but can be present in 25% of patients with SLE. Myositis or myopathy can be features of scleroderma. Scleroderma overlap with polymyositis is frequently anti-PM Scl antibody positive, whereas anti-Jo-1 does not normally occur in the overlap of scleroderma and polymyositis but is usually exclusively positive in polymyositis with arthritis and alveolitis. A better prognosis is found with PM Scl antibody in myositis. Vasculitis is not a typical feature of scleroderma, but has been reported. Eosinophilic fasciitis is rare, and the onset could be associated with simvastatin.

Arthritis, Rheumatoid↗
PubMed1990

Muscle involvement in the scleroderma syndromes.

Muscle involvement was identified in 14 patients with scleroderma or a connective tissue disease overlap syndrome with predominant features of scleroderma. Patients presented with symmetrical proximal weakness indistinguishable from other inflammatory myopathies. Creatine kinase and electromyography were useful to demonstrate muscle involvement. Muscle histopathology demonstrated primarily the vasculopathy of scleroderma or polymyositis in similar numbers of patients. Scleroderma vasculopathy and polymyositis generally occur without specificity to diffuse scleroderma, the calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia syndrome, or an overlap syndrome with arthritis. Polymyositis also occurs when the vasculopathy of scleroderma involves other organ systems.

Adult↗
PubMed1992

Significance of plasma endothelin-1 levels in patients with systemic sclerosis.

Endothelin-1 (ET-1) is a novel potent vasoconstrictor peptide discovered in the supernatant fraction of cultured endothelial cells. We measured plasma levels of ET-1 using a sensitive sandwich enzyme immunoassay. Plasma concentrations of ET-1 in 31 patients with systemic sclerosis (SSc) (1.90 +/- 0.47 pg/ml) were higher than those (1.31 +/- 0.10 pg/ml) in 25 age and sex matched healthy subjects. Patients with SSc with diffuse scleroderma had higher levels of ET-1 compared with those with limited scleroderma. Plasma ET-1 levels correlated inversely with carbon monoxide diffusing capacity (DLco). Measurement of plasma ET-1 levels may be useful as a predictor of prognosis of SSc.

Adult↗
PubMedDate not supplied

[Prevalence and clinical significance of cathepsin G antibodies in systemic sclerosis].

OBJECTIVES: To evaluate the prevalence and clinical significance of cathepsin G antibodies in patients affected with systemic sclerosis (SSc, scleroderma). METHODS: 115 patients affected by SSc, 55 (47,8%) with diffuse scleroderma (dSSc) and 60 (52,2%) with limited scleroderma (lSSc), were tested for cathepsin G antibodies by ELISA method. Moreover these sera were evaluated by indirect immunofluorescence (IIF) on ethanol and formalin fixed human neutrophils. RESULTS: By means of the ELISA method 16 (13,9%) patients were found to be sera positive for anti-cathepsin G, 2 (12.5%) of which showed a perinuclear fluorescence pattern (P-ANCA) and 4 (25%) an atypical ANCA staining, while 10 (62,5%) were negative on IIF. The IIF on scleroderma sera revealed 5 (4,3%) P-ANCA and 18 (15,7%) atypical ANCA patterns. The anti-cathepsin G antibodies significantly prevailed in scleroderma sera (p=0.02) when their frequency was compared with that of healthy controls; while they were not significantly associated to any clinical or serological features of SSc patients. CONCLUSIONS: The anti-cathepsin G antibodies were significantly frequent in scleroderma sera; however, no clinical correlations were found. Thus, the significance of their presence in SSc still needs to be clarified.

Adult↗
PubMed1984

[Prognosis and treatment of scleroderma].

The prognosis and treatment of scleroderma depend on the clinicopathological form of the disease: superficial, localised scleroderma is only a minor cosmetic problem; circumscribed, deep forms of scleroderma may cause troublesome symptoms which justify steroid therapy. Circumscribed and diffuse scleroderma are similar to systemic scleroderma for which prognostic criteria have been identified. There is a poor prognosis in men, in extensive skin involvement and in intestinal, cardiac or renal involvement. Limited skin disease and anti-centromere antibodies are associated with a good prognosis. The absence of an animal model and the uncertainty as to the underlying cause, explain the therapeutic problems. The authors discuss the respective value of a number of "specific" drugs, including D-penicillamine which seems to be the most hopeful line of treatment. Continuous follow-up of this chronic condition remains essential for the diagnosis and symptomatic management of visceral complications.

Adrenal Cortex Hormones↗
PubMed1993

Large vessel occlusive disease associated with CREST syndrome and scleroderma.

OBJECTIVES: To report the cases of three patients with CREST syndrome and one patient with diffuse scleroderma who had severe macrovascular disease and only minimal vascular risk factors. METHODS: The medical histories, physical examinations, and results of clinical investigations were reviewed in four patients. RESULTS: These four patients had severe morbidity from macrovascular disease of the arms and legs in the presence of minimal underlying vascular risk factors. These patients represent 11% of the women with scleroderma seen at our hospital since 1974. This is a greater than threefold increase above the expected proportion of symptomatic vascular disease seen in population studies. In the patients with CREST syndrome, large vessel disease was first seen more than 10 years after the onset of Raynaud's phenomenon, which was the first manifestation of the disease. A pathological specimen of the ulnar artery from one patient showed severe luminal narrowing by an acellular material with no evidence of atheroma. CONCLUSIONS: These cases suggest an association of both the CREST syndrome and scleroderma with macrovascular disease.

Aged↗
PubMed2003

Risk of cancer in patients with scleroderma: a population based cohort study.

BACKGROUND: Previous studies have suggested an increased risk of cancer among patients with scleroderma. OBJECTIVE: To study a population based cohort of patients with scleroderma in South Australia. METHODS: Subjects with scleroderma were identified from the South Australian Scleroderma Registry established in 1993. All subjects on the scleroderma registry were linked to the South Australian Cancer Registry to identify all cases of cancer until 31 December 2000. Standardised incidence ratios (SIRs) for cancer for subjects with scleroderma were determined using the age- and sex-specific rates for South Australia. RESULTS: In 441 patients with scleroderma, 90 cases of cancer were identified, 47 of which developed after inclusion on the scleroderma registry. The SIRs for all cancers among these patients were significantly increased (SIR=1.99; 95% confidence interval (95% CI) 1.46 to 2.65) compared with the cancer incidence rates for South Australia. The SIRs for lung cancer (SIR=5.9; 95% CI 3.05 to 10.31) were also significantly increased. The SIRs for all cancers among the subgroups with diffuse scleroderma (SIR=2.73; 95% CI 1.31 to 5.02) and limited scleroderma (SIR=1.85; 95% CI 1.23 to 2.68) were significantly increased. CONCLUSIONS: This population based cohort study provides evidence that scleroderma is associated with cancer, and in particular, lung cancer. In addition, both diffuse and limited forms of scleroderma are associated with a similarly increased risk of cancer.

Adolescent↗