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PubMed1996

Clinical and laboratory features of patients with scleroderma and silicone implants.

We reviewed the available clinical and laboratory data from 56 patients with scleroderma and silicone implants from the English medical literature and 19 cases which have not been previously reported. The average age of onset of scleroderma was 43.6 +/- 10 years (range 20-73). Patients had silicone implants for an average of 9 +/- 4 years prior to the development of scleroderma (range 1-32). Most patients had limited scleroderma (41%). Twenty three percent had intermediate scleroderma and 36% had diffuse scleroderma. Clinical findings included: Raynaud's phenomenon in 77%, esophageal dysfunction in 53%, and pulmonary involvement in 47%. Cardiac and renal involvement were uncommon. Antinuclear antibodies by immunofluorescence were found in 83 percent of patients. The immunofluorescence pattern was speckled in 53%, centromere in 31% and nucleolar in 9%. Other antibodies (Scl-70, RNP, SSA/Ro, PM-Scl) were found in only a small proportion of patients. A clinical, serologic and immunogenetic comparison of patients with silicone implants and scleroderma and patients with idiopathic scleroderma is needed to better understand the pathogenesis of this disorder.

Adult↗
PubMed2001

Esophageal hypomotility in systemic sclerosis: close relationship with pulmonary involvement.

PURPOSE: Esophageal motility was assessed in patients with systemic sclerosis (SSc) by scintigraphy and compared with (i) extent of scleroderma, (ii) duration of disease, (iii) index of anti-topoisomerase I antibody (topo I), and (iv) pulmonary involvement. METHODS: A multiple-swallow test was performed in 47 patients with SSc in the supine position with 99mTc-DTPA. A region of interest on the entire esophagus was defined and the retention ratio (RR) was calculated from a time-activity curve. RESULTS: Patients with diffuse scleroderma had higher RRs than those with limited scleroderma (48.8% vs. 30.0%; p < 0.05). There was no correlation between the RRs and the duration of disease. Patients with positive topo I had higher RRs than those who were negative (53.8% vs. 29.7%; p < 0.05). Patients with reduced % diffusion capacity for carbon monoxide (%DLCO) had higher RRs than those with normal %DLCO (40.5% vs. 19.6%; p = 0.03). Patients with reduced % vital capacity (%VC) had higher RRs than those with normal %VC (54.6% vs. 25.0%; p < 0.005). Patients with pulmonary fibrosis had higher RRs than those who were negative (58.5% vs. 20.3%; p < 0.00005). CONCLUSION: Esophageal dysfunction in patients with SSc showed a correlation with the extent of scleroderma, positive topo I, and pulmonary involvement. The RR can be an objective clinical marker for the severity of organ fibrosis.

Adolescent↗
PubMed2003

Acute myocarditis associated with adenoviral infection in a patient with scleroderma.

We describe a 40-year-old man with limited scleroderma who presented with acute heart failure following a flu-like illness. He was known to have incomplete left anterior bundle branch block, initial isolated pulmonary hypertension with enlarged right atrium, and no pulmonary fibrosis. He received therapy for acute heart failure and was transferred to a scleroderma centre for specific treatment of scleroderma cardiomyopathy. Investigations showed raised inflammatory markers and diffuse hyperechogenic thickening of the myocardium on echocardiography. Contrast-enhanced (Gd-DOTA) cardiovascular magnetic resonance imaging (CV-MRI) showed multiple areas of non-homogeneous delayed hyperenhancement in the left ventricle, suggestive of myocarditis. Antiadenovirus IgM antibodies were detected with a titer consistent with recent infection. Six weeks later a repeat Gd-DOTA CV-MRI showed an almost complete resolution of the areas of hyperenhancement and there was a significant reduction in the adenovirus antibody titer with serological conversion to IgG. To our knowledge this is the first report of viral myocarditis in scleroderma. Infections are important causes of morbidity and mortality in this disease and should always be included in the differential diagnosis of cardiac symptoms. We propose that contrast-enhanced CV-MRI is valuable in a non-invasive diagnosis of heart disease in patients with scleroderma.

Acute Disease↗
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↗
PubMed2001

Scleroderma pericardial disease presented with a large pericardial effusion--a case report.

Scleroderma pericardial disease is usually silent and benign. The incidence of pericardial involvement in scleroderma is about 50% according to autopsy results, but symptomatic pericarditis manifests in about 16% of patients with diffuse scleroderma and in about 30% of patients with limited scleroderma. The clinically evident pericardial effusion is rare in scleroderma, although it can be detected in about 41% of patients with echocardiography. In majority of the patients, the pericardial effusion is small and not associated with symptoms. The pericardial effusion manifests usually after the manifestation of the other clinical and serologic features of scleroderma. A case of scleroderma is reported that presented with a large pericardial effusion, which antedated the other clinical and serologic features of scleroderma. The pericardial involvement in scleroderma is reviewed.

Humans↗
PubMed2001

Treatment of systemic sclerosis.

Systemic sclerosis is an extremely variable disease in its manifestations and consequently, treatment needs to be individualized depending on the specific problems that each patient has. Limited scleroderma patients have a prolonged duration of Raynaud's phenomenon and puffy fingers before they develop any skin thickening, digital ulcers or gastrointestinal symptoms. They are likely to present with all the classic manifestations of scleroderma. Diffuse scleroderma patients have a much more acute systemic onset with marked whole hand swelling and may initially have only subtle skin thickening. A good understanding of the differences between the natural history of limited and diffuse scleroderma will enable the physician to treat present problems and anticipate future ones more effectively. One should determine which major subset and organ systems are involved before deciding on the appropriate therapy. Advances in organ-specific therapy, particularly calcium channel antagonists in Raynaud's phenomenon, proton pump inhibitors in esophageal reflux, intravenous iloprost and endothelin receptor antagonists in pulmonary hypertension, and ACE inhibitors in renal crisis, have decreased morbidity and mortality in patients with scleroderma. Studies of aggressive therapies to prevent or improve pulmonary fibrosis are in progress. Further clinical experience in wound healing, gastrointestinal malabsorption and physical therapy for loss of motion has helped patients to have a more comfortable life. In recent years, a significant number of controlled clinical trials have been performed and there has been improved understanding of the best way to perform studies and of which patients are most likely to respond to therapy. Penicillamine, methotrexate, photopheresis, relaxin, interferons, and cyclosporine have all been studied in controlled trials with variable outcomes. Although an overall remittive therapy has not yet been determined, new, potentially useful agents are being investigated.

Antirheumatic Agents↗
PubMed1985

Sclerodermoid changes of porphyria cutanea tarda: possible relationship to urinary uroporphyrin levels.

From 1950 to 1982, fifteen patients were seen at the Mayo Clinic with a diagnosis of sclerodermoid changes of porphyria cutanea tarda. Fourteen patients had changes similar to scleroderma limited to the skin, and one patient had scleroderma-like skin changes accompanied by visceral abnormalities. Both light-exposed and unexposed areas of the body were affected. Areas of involvement included the chest, the V-shaped area of the neck, and the back, face, and shoulders. In six patients, morpheaform changes represented the presenting cutaneous sign of porphyria cutanea tarda. Follow-up examination, after treatment that included abstinence from alcohol and phlebotomy, revealed that the sclerodermoid skin changes had disappeared in six patients and improved in four. Generally, the degree of improvement of the sclerodermoid changes was proportional to the reduction of the urinary uroporphyrin levels toward normal (p = 0.02).

Adult↗
PubMed1993

Systemic sclerosis (scleroderma) associated with discoid lupus erythematosus.

Six patients with systemic sclerosis (SS) and discoid lupus erythematosus (DLE) were studied to determine whether such cases have some common clinical and laboratory findings. DLE preceded SS in all cases. Three patients had diffuse scleroderma with lung and esophagus involvements and the others limited scleroderma. Three patients had anti-topoisomerase-I and antiribonucleoprotein antibodies, 2 had either of them and the remaining anticentromere antibodies. Four had DLE located on the scalp, leading to alopecia. The other 2 had DLE on the face and extremities. No case fulfilled criteria for systemic lupus erythematosus (SLE). The present cases with SS and DLE, but without SLE, indicate that this type of systemic-cutaneous collagen disease overlap does exist and may be not so rare.

Adult↗
PubMed1985

Scl-86, a marker antigen for diffuse scleroderma.

More than 300 sera from patients with a connective tissue disease were analyzed with the immunoblotting technique. The presence of autoantibodies against an 86,000-mol wt marker antigen for diffuse scleroderma (Scl-86) was found in 14 out of 33 patients with scleroderma. The presence of anti-Scl-86 antibodies seemed to correlate with the diagnosis of diffuse scleroderma since they were found in 13 out of 22 diffuse scleroderma patients and in only one out of 11 patients with limited scleroderma. All scleroderma sera (33 patients' sera and 13 reference sera) were also tested for the presence of anti-Scl-70 antibodies. It was found that all anti-Scl-70 positive sera (n = 25) contained anti-Scl-86 antibody as well, suggesting a relationship between these two antigens. However, the Scl-86 antigen was shown to be an extremely insoluble nonchromosomal protein, resistant to boiling in sodium dodecyl sulfate. This contrasts with the Scl-70 antigen, which has been described as a thermolabile, soluble antigen present in the chromatin fraction. Together, our results are consistent with the idea that Scl-70 is a degradation product of Scl-86. The Scl-86 antigen is present in freshly prepared rabbit thymus, spleen, and liver nuclei as well as in nuclei from various cultured cell lines, but is not detectable in extractable nuclear antigen from rabbit thymus. In a limited retrospective study, the anti-Scl-86 antibodies were found in two sera from patients with Raynaud's phenomenon before the development of diffuse scleroderma. Therefore, it is possible that screening of patients' serum for this antibody might predict the development of diffuse scleroderma.

Animals↗
PubMed1993

Treatment of systemic sclerosis.

Although there have been no major breakthroughs in scleroderma therapy, new treatments have been tested in patients with systemic sclerosis, including both interferon alfa and interferon gamma. These biologic agents can reduce collagen synthesis, which is a rational target for scleroderma therapy. Debate about the use of photopheresis continues, and it was suggested in a recent editorial that photopheresis is no better than D-penicillamine in the treatment of scleroderma and is more expensive. Cyclosporine appears to have frequent renal toxicity when used to treat scleroderma. Outcome measurements have been concentrated on in scleroderma trials. Several types of scleroderma classifications were compared, and the classification of diffuse and limited scleroderma was strongly related to disease severity. Skin score was systematically compared with mapping the surface area of involved skin, and the skin score was found to be more reliable. A possible prognostic indicator in scleroderma is high-resolution pulmonary computed tomography, which is sensitive in early detection of scleroderma-associated interstitial lung disease. Classification of Raynaud's phenomenon into primary and secondary forms has been proposed, and further testing of the criteria and long-term follow-up is necessary to validate this classification. Over the past year, treatment of vasospasm with prostacyclin analogues has been efficacious with iloprost but not with low-dose oral cicaprost. Tissue plasminogen activator is not beneficial in the treatment of Raynaud's phenomenon. A report of radical microarteriolysis for the treatment of refractory Raynaud's phenomenon seems promising, warranting further investigation.

Clinical Trials as Topic↗
PubMed1995

[Systemic sclerosis. A rare connective tissue disease with manifestations in many organs].

Systemic sclerosis (scleroderma) is a rare connective tissue disease which can affect most human organs. Systemic sclerosis is divided into two groups: one diffuse scleroderma form, which is often more serious, with extensive organ involvement, and a limited scleroderma form with good prognosis. During a period of 20 years from 1974 to 1994, 14 patients were diagnosed as having systemic sclerosis in a population of approximately 150,000. Five were classified as having the diffuse form, and nine as having the limited form. Ten patients were found to have antinuclear antibodies. All patients had Raynaud's phenomena and scleroderma. Involvement of the gastrointestinal tract/lungs and joint-tendon sheets were found in six and eight patients respectively. Muscles, heart and kidneys were involved in three of the patients. Our results correspond with those described in the literature.

Adult↗
PubMed2002

[Anti-neutrophil cytoplasmic autoantibody-associated rapid progressive glomerulonephritis complicated with both limited and diffuse scleroderma].

We report two patients with scleroderma, 73-year-old female and 67-year-old female, who developed anti neutrophil cytoplasmic autoantibody (ANCA) associated rapid progressive glomerulonephritis (RPGN). Both patients have had a long history of scleroderma (23 and 14 years, respectively) when ANCA-associated glomerulonephritis occurred. In the first patient, scleroderma was localized in both fingers. She has been followed-up as CREST syndrome rather than systemic sclerosis. The complaints on admission were leg edema and left chest pain in the first patient, and a pyrexia and dyspnea in the second patient. Both patients showed pulmonary manifestation (pleural effusion in the first patient, interstitial pneumonia and alveolar hemorrhage in the second patient, respectively) and rapid progressive glomerulonephritis. Both patients died in spite of corticosteroid therapy. Autopsy findings in the second patient demonstrated crescentic glomerulonephritis and alveolar hemorrhage. Our cases demonstrated that MPO-ANCA associated glomerulonephritis could be associated with limited scleroderma as well as systemic scleroderma. In these condition, the prognosis will be poor if scleroderma seemed to be stable.

Aged↗
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

A comparison of nesiritide vs. epoprostenol in a patient with precapillary pulmonary hypertension due to scleroderma complicated by postcapillary pulmonary hypertension.

To the best of our knowledge, acute decompensated left-sided heart failure with preserved left ventricular ejection fraction in a patient with scleroderma has not been previously reported. We describe a patient with severe pulmonary hypertension due to limited scleroderma in whom nesiritide led to marked reductions in pulmonary arterial and capillary wedge pressure as well as resolution of symptoms and pulmonary edema. Subsequent epoprostenol use was associated with an increase in pulmonary capillary wedge pressure and a recurrence of pulmonary edema. Thus, nesiritide may be the preferred agent in scleroderma patients with severe pulmonary hypertension and preserved left ventricular systolic function since epoprostenol may lead to adverse hemodynamic effects.

Antihypertensive Agents↗
PubMed1995

Left ventricular wall motion abnormalities in 80 patients with systemic sclerosis.

Segmental analysis of left ventricular wall motion was performed in 80 patients with systemic sclerosis by echocardiography. Half of the cases showed normal wall motion. Eighteen of the 57 patients with limited cutaneous systemic sclerosis (31.6%) exhibited hypokinetic wall motion abnormalities, while five of the 23 cases with diffuse cutaneous systemic sclerosis (21.7%) had similar findings. The three cases showing akinesis also belonged to the limited scleroderma subgroup. Our findings show that hypokinetic wall motion abnormalities can be detected in a remarkably high proportion of cases with limited cutaneous systemic sclerosis.

Adult↗
PubMed1998

Iloprost as cyclic five-day infusions in the treatment of scleroderma. An open pilot study in 20 patients treated for one year.

OBJECTIVE: To evaluate the safety and efficacy of cyclic intravenous iloprost therapy in diffuse or limited scleroderma. METHODS: Twenty patients, 14 women and 6 men with a mean age of 47.8 +/- 8.2 years, were given iloprost infusions for six hours a day during five consecutive days, at a rate of 0.5 to 2 ng/kg/min. The course was repeated every three months for one year. Efficacy was evaluated based on a scleroderma skin lesion score, an ischemic lesion score, a well-being self-assessment score, and lung function tests including measurement of the diffusing capacity of the lung for carbon monoxide. Safety was assessed based on adverse event collection. RESULTS: The scleroderma skin lesion and ischemic lesion scores decreased significantly over the one-year treatment period, from 37.1 +/- 16.5 to 10.2 +/- 6.9 (P < 0.001) and from 31.8 +/- 19.1 to 2.2 +/- 2.0 (P < 0.05), respectively. The well-being self-assessment score also showed a significant improvement, from 71.4 +/- 16.5 to 15.0 +/- 6.6 (P < 0.001). The diffusing capacity for carbon monoxide was decreased in 11 patients at baseline and showed a slight, non significant increase in these patients after the treatment period. No serious or persistent side effects were recorded. CONCLUSION: Cyclic intravenous iloprost therapy was associated with improvements in skin changes and in general health, as well as with a slight increase in the diffusing capacity of the lung for carbon monoxide. Our data suggest that iloprost may act on some of the pathogenetic mechanisms of scleroderma in a way that improves the course of the disease.

Female↗
PubMed1996

Case report: insulin-dependent diabetes mellitus in childhood associated with scleroderma.

A 14-year-old boy presented with Type I diabetes mellitus and subsequently developed pancreatic exocrine insufficiency and systemic sclerosis (SSc). His diabetes had been diagnosed when he was about 5 years old, after the onset of ketoacidosis. Insulin treatment was provided from then until the time he was treated in our department. Exocrine pancreatic insufficiency, which occurred at age 9, was treated with pancreatic extracts. Cystic fibrosis was excluded after a chloride sweat test. The diagnosis of SSc was confirmed at age 14 on the basis of skin sclerosis, sclerodactyly and oesophageal and pulmonary involvement and then at age 18 by the occurrence of Raynaud's disease. Thus, this patient demonstrated a rare and previously unreported association of Type I diabetes mellitus and systemic scleroderma. Limited joint mobility and skin abnormalities are frequent in childhood diabetes mellitus but should not be misdiagnosed as systemic scleroderma.

Adolescent↗