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PubMed1996

Scleroderma renal crisis.

Renal crisis occurs in systemic sclerosis patients with rapidly progressive diffuse cutaneous thickening early in their disease. SRC is characterized by malignant hypertension, hyperreninemia, azotemia, microangiopathic hemolytic anemia, and renal failure. This complication, which in the past has been almost uniformly fatal, is now successfully treated in most cases with ACE inhibitors. This therapy has improved survival, reduced requirement for dialysis, and in those on dialysis has often allowed discontinuation of this procedure 6 to 18 months later. Prompt diagnosis and early, aggressive initiation of therapy with ACE inhibitors will result in the most optimal outcome. Chronic nonrenal crisis renal insufficiency is unusual and rarely progresses to significant renal dysfunction.

Humans↗
PubMed1975

[Therapeutic action of coagulation factor XIII in scleroderma. 20 cases].

Clotting factor XIII was used in 13 patients suffering from scleroderma, 17 with generalised scleroderma and 3 with localised scleroderma. The duration of treatment varied between 15 days and 6 months. The effect was marked in 7 cases and more moderate in 5 others. It took the form of increased suppleness of the skin, improved joint mobility and, to a lesser degree, an improvement in vasomotor disturbances of the extremities. Oesophageal abnormalities on X-ray were never altered. The return to normal of alveolar-capillary diffusion seen in two cases requires futher confirmation. The action of factor XIII in scleroderma may be explicable by the formation of transamidation bonds between the alpha chains of the collagen molecule, similar to those obtained on fibrin.

Adolescent↗
PubMed2001

Blockade of endogenous transforming growth factor beta signaling prevents up-regulated collagen synthesis in scleroderma fibroblasts: association with increased expression of transforming growth factor beta receptors.

OBJECTIVE: To elucidate the role of transforming growth factor beta (TGFbeta) in the increased expression of the collagen gene in scleroderma fibroblasts. METHODS: Dermal fibroblasts from 10 patients with diffuse systemic sclerosis (SSc) of recent onset and from 10 healthy individuals were studied. The production of active and total (active + latent) TGFbeta1 levels from cultured dermal fibroblasts was measured using a TGFbeta1 enzyme-linked immunosorbent assay system. Expression of the TGFbeta type I and type II receptor proteins in dermal fibroblasts was determined by immunoblotting, and the level of expression of human alpha2(I) collagen messenger RNA (mRNA) was evaluated by Northern blot analysis. The transcriptional activity of the human alpha2(I) collagen gene was examined with chloramphenicol acetyltransferase (CAT) assays using the -772 COL1A2/CAT construct. RESULTS: SSc fibroblasts expressed increased levels of TGFbeta type I and type II receptors but secreted amounts of TGFbeta similar to those secreted by normal fibroblasts. The blockade of TGFbeta signaling with anti-TGFbeta antibodies or a TGFbeta1 antisense oligonucleotide abolished the increased mRNA expression, as well as the up-regulated transcriptional activity of the human alpha2(I) collagen gene in SSc fibroblasts. CONCLUSION: These results suggest that TGFbeta plays a crucial role in the pathogenesis of SSc and raise the possibility of a therapeutic approach with anti-TGFbeta antibodies or a TGF11 antisense oligonucleotide.

Antibodies↗
PubMed1996

Progressive systemic sclerosis sine scleroderma which developed after exposure to epoxy resin polymerization.

Progressive systemic sclerosis (PSS) sine scleroderma is well known as a special form of scleroderma. Because of its rarity, its pathogenesis has not yet been elucidated. We experienced a 33-year-old man who developed PSS sine scleroderma while working with epoxy resin polymerization. He had short white frenulum linguae, diffuse hyperpigmentation and facial telangiectasia, positive antinuclear antibody, and pulmonary dysfunction, but not acrosclerosis or sclerodactylia. Modest dermal collagen proliferation in the forearm skin confirmed PSS sine scleroderma. Epoxy resin polymerizer appears to be a potent causative agent for PSS sine scleroderma as well as for generalized morphea-like PSS.

Adult↗
PubMed1993

Effect of cold stress on coronary sinus blood flow in patients with scleroderma.

The mechanism of cardiac involvement in scleroderma is not known. Histologic studies of the myocardium in patients who died of scleroderma revealed that half had myocardial damage. Characteristic involvement ranges from focal contraction-band necrosis to diffuse fibrosis despite the absence of obstructive coronary artery disease of the major epicardial vessels. These findings suggest that scleroderma heart disease might result from episodic reduction of coronary blood flow due to abnormalities of coronary vasomotor tone. Studies using cold pressor thallium 201 myocardial perfusion scans support this hypothesis. Our study measured coronary blood flow during hand immersion in ice water at cardiac catheterization to determine whether patients with scleroderma had an abnormal coronary blood flow response. Coronary sinus blood flow was measured using a thermodilution method. Five patients with scleroderma were compared with 5 control subjects. All patients and controls had normal coronary angiograms. The coronary blood flow at baseline in the scleroderma and the control group (130 +/- 33 mL/min and 86 +/- 27 mL/min, respectively) was not significantly different. During the cold pressor test, both groups had a small, insignificant increase in coronary blood flow from baseline to 60 seconds (130 to 144 mL/min, scleroderma patients; 86 to 89 mL/min, control subjects). Our findings suggest that the cold pressor test does not cause an abnormal increase in coronary vasomotor tone or an absolute reduction in coronary blood flow in patients with scleroderma, as previously suggested by thallium 201 cold pressor studies.

Adult↗
PubMed1993

Esophageal physiology and pathophysiology.

This article presents the normal physiology of esophageal peristalsis. It discusses current approaches to the diagnosis and treatment of primary disorders of the esophagus, including achalasia, nutcracker esophagus, diffuse esophageal spasm, as well as the secondary disorder, scleroderma.

Esophageal Motility Disorders↗
PubMed2000

Permanent sacral nerve stimulation for fecal incontinence.

OBJECTIVE: To characterize the longer-term therapeutic response of permanent sacral nerve stimulation for fecal incontinence and to delineate suitable indications and the mode of action. SUMMARY BACKGROUND DATA: A single report of permanent sacral nerve stimulation in three patients followed up for 6 months showed marked improvement in fecal continence. Acute evaluation has shown that the effect may be mediated by altered rectal and anal smooth muscle activity, and facilitation of external sphincter contraction. METHODS: Five women (age 41-68 years) with fecal incontinence for solid or liquid stool at least once per week were followed up for a median of 16 months after permanent implantation. All had passive incontinence, and three had urge incontinence. The cause was scleroderma in two, primary internal sphincter degeneration in one, diffuse weakness of both sphincters in one, and disruption of both sphincters in one. RESULTS: All patients had marked improvement. Urgency resolved in all three patients with this symptom. Passive soiling resolved completely in three and was reduced to minor episodes in two. Continence scores (scale 0-20) improved from a median of 16 before surgery to 2 after surgery. There were no early complications, and there have been no side effects. One patient required wound exploration at 6 months for local pain, and a lead replacement at 12 months for electrode displacement. The quality of life assessment improved in all patients. The resting pressure increased in four patients, but there was no consistent measured physiologic change that could account for the symptomatic improvement. CONCLUSIONS: In patients with sphincter degeneration and weakness, and possibly in those with sphincter disruption, sacral nerve stimulation markedly improves fecal incontinence.

Adult↗
PubMed1984

Motor disorders of the esophagus: diagnosis and treatment.

Esophageal motor disorders may be clearly primary, as in achalasia or diffuse esophageal spasm (DES), or clearly secondary, as in scleroderma or intrathoracic malignancy. In patients with gastroesophageal reflux, abnormal motility of the esophageal body and stomach, and lower esophageal spasm (LES) appear to predispose patients to reflux. It is possible that esophagitis caused by refluxed gastric material then further impairs motility, propagating the injury. Therapeutically, appropriate use of recently available medications, such as calcium channel blockers and metoclopramide, and new applications of previously available agents, such as hydralazine and bethanechol, have improved our ability to relieve symptoms and at times restore more normal motility.

Bethanechol Compounds↗
PubMed1991

Current topics in the diagnosis and treatment of scleroderma.

Scleroderma has been viewed as one of the most discouraging diffuse connective tissue diseases to manage. In the last 10 years, we have seen major advances in our understanding of its pathogenesis. New techniques for earlier diagnosis widen the window of opportunity for therapeutic intervention. A multitude of possible disease-modifying therapies are under study. Advances in molecular biology, including increased understanding of the cellular messages controlling the process of fibrosis, provide additional hope for better treatment. Clinical research in this disease is inhibited by limited numbers of patients and the difficulties encountered in evaluating progression and response to treatment. All patients with suspected or newly diagnosed scleroderma should be evaluated at a medical center actively engaged in the research of this disease. Such research is likely to accelerate the gains made during the last decade.

Adult↗
PubMed1996

Raynaud's phenomenon.

Raynaud's phenomenon is a common clinical problem occurring in 3% to 5% of the general population. The first symptom of scleroderma is often Raynaud's phenomenon, which is associated with a diffuse small vessel vasculopathy and ischemia and reperfusion injury to skin and other organs targeted in this disease. Current studies support the concept that Raynaud's phenomenon is secondary to a local defect in the regulation of regional blood flow. New evidence demonstrates that there is a profound sensitivity to alpha 2-adrenoceptors mediated vasoconstriction in scleroderma vessels. Traditional treatment of Raynaud's phenomenon is cold avoidance and the use of vasodilators. Oral prostaglandins have shown promise as therapeutic agents.

Clinical Trials as Topic↗
PubMed1994

Fasciitis (not scleroderma) following prolonged exposure to an organic solvent (trichloroethylene).

We describe 2 cases of diffuse fasciitis with eosinophilia (DFE) associated with prolonged exposure to the industrial solvent trichloroethylene (TCE). The medical and personal histories, examinations, and laboratory and pathological investigations were reviewed and summarized. The 2 case reports, representing the first and 2nd cases of DFE associated with TCE, were compared with 8 reported cases of systemic sclerosis associated with TCE and suggest a direct association between TCE exposure and the development of fasciitis (DFE).

Aged↗
PubMed1977

Colchicine in the treatment of scleroderma.

We treated 10 patients with well established scleroderma with colchicine in the highest tolerated dose for one year. We could determine no clinical or laboratory improvement in any patient. Progression of disease was suggested by the development of new skin ulcers and telangiectasia, by an increase in musculoskeletal complaints, and by a significantly diminished ability to make a fist. In addition, there was deterioration of pulmonary function as demonstrated by a statistically significant fall in FEV1, and diffusing capacity. Colchicine was not of value in the treatment of scleroderma in this group of patients.

Adult↗
PubMedDate not supplied

Autoantibodies to RNA-polymerases in Italian patients with systemic sclerosis.

OBJECTIVE: To assess the frequency and clinical correlates of the systemic sclerosis-related autoantibodies to RNA polymerases in Italian patients. METHODS: Sera from 115 patients with systemic sclerosis (SSc) and 10 patients with systemic sclerosis-overlap syndromes recruited from a single center in northern Italy were investigated for antibodies to RNA polymerase I, II, and III by means of immunoprecipitation using 35S-labeled HeLa cell antigen extract. Twenty-five normal volunteers and 91 patients with different connective tissue diseases were studied as a control group. RESULTS: Antibodies to RNA-polymerases were found in 14/115 SSc patients (12.1%). None of the normal controls and none of the patients with other connective tissue diseases, including overlap syndromes, were positive. Antibodies reacting with RNA-polymerase I and III (+/- RNA-polymerase II) were found in 9/115 patients (7.8%) and were mutually exclusive with respect to other scleroderma-related autoantibodies. Isolated anti-RNA polymerase II reactivity was found in 5 patients and was associated with anti-topoisomerase I antibodies in 4 cases. Anti-RNA-polymerase I and III antibodies were associated with diffuse cutaneous involvement and male gender. Only two patients from our series had scleroderma renal crisis, and one of them had anti-RNA polymerase antibodies. CONCLUSIONS: Anti-RNA-polymerase antibodies appear to be less frequent in Italian patients than in Caucasian patients from the United Kingdom or USA. This might be associated with the lower frequency of scleroderma renal crisis.

Adult↗
PubMed2003

Scleroderma renal crisis.

Renal crisis occurs in patients who have systemic sclerosis with rapidly progressive diffuse cutaneous thickening early in their disease. SRC is characterized by malignant hypertension, hyperreninemia, azotemia, microangiopathic hemolytic anemia, and renal failure. SRC was almost uniformly fatal, but in most cases it can now be successfully treated with ACE inhibitors. This therapy has improved survival, reduced the requirement for dialysis, and often allowed for the discontinuation of dialysis 6 to 18 months later. Prompt diagnosis and early, aggressive initiation of therapy with ACE inhibitors will result in the most optimal outcome.

Acute Kidney Injury↗
PubMed2001

Pulmonary-renal syndrome in systemic sclerosis.

BACKGROUND AND OBJECTIVE: Renal failure, pulmonary hypertension, and interstitial lung disease are major causes of morbidity and mortality in systemic sclerosis (SSc). However, the concomitant occurrence of pulmonary hemorrhage associated with acute renal failure in SSc has been rarely described. The present study is the first analysis of pulmonary-renal syndrome in SSc. PATIENT AND METHODS: We present a 44-year-old woman with SSc who died of a fulminant course of acute renal failure associated with diffuse alveolar hemorrhage. We termed this uncommon and fatal complication of SSc scleroderma-pulmonary-renal syndrome (SPRS). A search of the English-written literature yielded reports of 10 additional similar cases. These patients, together with our present case, form the basis of the present analysis. RESULTS: The average age of the patients with SPRS was 46 years. The majority of the patients (80%) were women, and most had diffuse SSc. SPRS occurred an average of 6.4 years after disease onset and was associated with prior fibrosing alveolitis and/or D-penicillamine treatment. Interestingly, normotensive renal failure seems to characterize the scleroderma patients, because 9 of 11 (82%) had normal blood pressure. SPRS bears a poor prognosis: all of the 11 patients (100%) died within 12 months of admission. However, only 60% of the 5 patients for whom we have treatment data received corticosteroids. CONCLUSIONS: Pulmonary-renal syndrome is a rare but fatal complication of SSc. Because the treatment data are scarce and the prognosis is poor, aggressive treatment with pulse corticosteroids, cyclophosphamide, and possibly plasmapheresis is suggested.

Acute Kidney Injury↗
PubMed2001

Oesophageal pressure-cross-sectional area distributions and secondary peristalsis in relation to subclassification of systemic sclerosis.

The aim of the present study was to correlate the severity of oesophageal motor dysfunction with the severity of cutaneous disease in systemic sclerosis (SS). Patients were divided into three groups based on the degree of skin involvement: type I, acrosclerosis distal to the wrist; type II, scleroderma extending above the wrist in proximal direction; type III, diffuse cutaneous systemic sclerosis. Impedance planimetry employing distensions with pressures up to 5 kPa with the concomitant measurement of oesophageal cross-sectional area (CSA) was used in combination with standard oesophageal manometry. Measurements were made at 7 and 15 cm above the lower oesophageal sphincter (LOS). Thirty patients (16 type I, six type II and eight type III patients) and 23 normal controls were included. LOS pressure was lower in SS patients than in normal patients, with the lowest values in type III. The CSAs were higher in SS patients than in controls at both sites (P < 0.001). The CSAs at the distal site were highest in type III, as compared to type I and II (P < 0.03). The CSA at the highest induced pressure (5.0 kPa) was 613 +/- 45, 719 +/- 79, and 808 +/- 115 mm2 in types I, II and III, respectively. No differences in CSA were found at the proximal site between the three types of SS. The distensibility did not differ between SS and normal patients at the distal site. The distensibility was lowest in SS patients (P < 0.001) at the proximal distension site. The distensibility did not vary with the type of SS at either site. Significant differences in contraction frequency of the secondary peristalsis as function of wall tension were demonstrated between the SS patients and controls at the distal site (P < 0.05). No differences were found at the proximal site. The contraction frequency and amplitude at the distal and proximal sites did not differ among the three types. In conclusion for most parameters studied, SS patients differed from normal patients. Among SS types, the most pronounced changes were found in type III.

Adult↗
PubMed1980

Diffuse fasciitis with eosinophilia.

In 1974 Shulman described two patients who had a scleroderma-like thickening of the skin in association with eosinophilia. Biopsies revealed striking thickening of the fascia between the subcutaneous tissue and muscle, with intense infiltration of plasma cells and lymphocytes. There was no evidence of myositis or of the visceral manifestations of systemic sclerosis. Reports of similar patients have since appeared; we report a further patient thought to have this condition.

Adult↗
PubMed1990

Diffusing capacity of the lung and nifedipine in systemic sclerosis.

Lung involvement in systemic sclerosis may be due in part to a functional abnormality of the pulmonary vasculature. To investigate the possible role of a pulmonary vasospastic process in this disorder, 21 non-smoking patients who had no evidence of cardiac disease or pulmonary hypertension were evaluated with pulmonary function tests prior to administration of nifedipine, 30 minutes after a single oral dose of nifedipine (20 mg), and after 4 weeks of treatment with nifedipine (10 mg 3 times daily). Treatment with nifedipine did not significantly change any of the pulmonary function values, except for the carbon monoxide diffusing capacity (DLCO). The linear trend between the individual DLCO values at baseline and their changes immediately following the initial 20-mg dose of nifedipine (r = -0.603, P = 0.02) and after 4 weeks of treatment (r = -0.636, P = 0.01) showed that the lower the DLCO value at baseline, the greater the improvement caused by nifedipine. These findings support the hypothesis of a potentially reversible pulmonary vasospasm in systemic sclerosis and suggest that nifedipine may be useful in the treatment of lung disease in these patients; however, further studies are needed.

Adult↗