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PubMed1988

The diagnosis and classification of scleroderma (systemic sclerosis).

Difficulty in the diagnosis of the disease scleroderma may occur at the early stage prior to the development of obvious skin sclerosis. A presumptive diagnosis may be made if Raynaud's phenomenon is accompanied by a positive 'neck test', 'scleroderma' capillary changes in the nailfolds or antinuclear antibodies. Definitive diagnosis may have to be delayed for several years from the onset of Raynaud's phenomenon until definite characteristic skin changes are seen. Ten cases in which an earlier diagnosis of scleroderma was not substantiated are listed. The earlier incorrect diagnosis would have been avoided by use of the methods described in this paper. Various terms have been used to denote subdivisions of scleroderma. These include acrosclerosis, diffuse scleroderma and CREST. We have used the terms Type 1, Type 2 and Type 3 based on the early extent of the skin sclerosis where Type 1 (limited extent) indicates sclerodactyly only, Type 2 (moderate extent) indicates sclerosis proximal to the metacarpophalangeal joints but excluding the trunk and Type 3 (extensive) indicates diffuse skin sclerosis including the trunk. The clinical value of this simple classification is reviewed and contrasted to other classifications which appear to be poorly defined and of limited use.

Adult↗
PubMed1993

[Mechanisms of cardiac involvement in systemic scleroderma, apropos of 3 cases].

We studied myocardial manifestations of systemic sclerosis in three patients. Two patients were symptomatic. A transthoracic echocardiography, a coronary angiography, a cardiac catheterization, thallium scans with dipyridamol completed by thoracic cold exposure and endomyocardial biopsy with immunohistological study were performed. None of them have coronary stenosis. In the three cases abnormalities of myocardial perfusion were detected, two of them have fibrosis. The immunohistological study always showed an anusual expression of HLA DR on cardiac fibroblasts.

Aged↗
PubMed1984

[Electron microscopy study of pathological collagen fibril formation in systemic scleroderma].

Electron microscopy of the skin biopsies from patients with systemic sclerodermia revealed the pathologic alterations of cellular and intracellular structures. The disease is characterized by focal lysis of nuclear, organoid and cytoplasmic membranes of the fibroblastic cells with the most essential alteration of cell plasmalemma. The cellular fragments (parts of cytoplasma, cisterns of the rough endoplasmic reticulum, mitochondria, nuclear debris) are found between the extracellular bundles of collagen fibrils. The pathologic forms of collagen aggregation (globular forms, non-homogeneous fibrils of the quaternary structure and transversally-bound microfibrillar aggregates) appear along with the fibrillar quaternary structures. Abnormal forms of fibrillar collagen are reluctant for deaggregation and lysis and facilitate the progression of fibrosis.

Autoimmune Diseases↗
PubMed1990

[Effect of cisapride on esophageal motility in healthy probands and patients with progressive systemic scleroderma].

Prokinetic agents might be useful in patients with progressive systemic sclerosis (PSS) who have disturbed function of the lower esophageal sphincter and impaired acid-clearance of the tubular esophagus. We therefore compared, by means of esophageal manometry, the effect of 20 mg cisapride orally vs. placebo in 12 patients with progressive systemic sclerosis and proven esophageal dysfunction as well as in 10 healthy volunteers in a double-blind, prospective trial. An increase of the lower esophageal resting pressure from 18.1 +/- 2.4 mm Hg to 23.9 +/- 8.1 mm Hg* after cisapride administration was observed in healthy volunteers, and from 10.9 +/- 3.2 mm Hg to 13.6 +/- 4.0 mm Hg* in the PSS patients. The amplitudes of peristaltic waves in the distal part of the esophagus were increased by cisapride from 83.8 +/- 10.6 mm Hg to 95.6 +/- 15.5 mm Hg* in volunteers and from 28.9 +/- 12.8 mm Hg to 36.8 +/- 16.2 mm Hg in patients (*:P less than 0.05). These results indicate that cisapride has a therapeutic rationale in the treatment of esophageal dysfunction in PSS; further clinical investigations are justified.

Adult↗
PubMed1991

Serological profiles as prognostic clues for progressive systemic scleroderma: the Italian experience.

Ninety-one patients with progressive systemic sclerosis have been examined both clinically and serologically in order to have a better prognostic insight. Three main serological profiles have been isolated. The patients with anticentromere antibodies (ACA) represented one third of the cases, developed skin sclerosis rather later and rarely exhibited ankyloses and ulcerations. The esophagus was commonly involved while the lung, heart and kidneys were not. ACA-positive patients were not identified with the CREST syndrome, as the latter disclosed other profiles with the same frequency. Patients with anti-Scl-70 antibody represented one fourth of the cases and had the fastest progression, developing sclerosis in less than 5 years after the onset of Raynaud's phenomenon. Ankyloses and lung fibrosis, as well as joint, heart and kidney involvement, were found in most of them. Patients with anti-SSA/Ro antibodies were uncommon, but corresponded to a severe subset, having a fast progression and a constant involvement of the lung. Probably due to the rougher definition of their serology, patients with antinuclear, antispeckle-patterned and anti-Ku antibodies or without any detectable antibody could be defined less easily and corresponded to an intermediate position between ACA- and anti-Scl-70-positive patients. Though it is probably premature to trust it completely, a serological classification may provide the prognostic clues clinical classifications cannot.

Adult↗
PubMedDate not supplied

[Autoimmune theory of pathogenesis of systemic scleroderma].

Recent studies indicate there is bidirectional traffic of cells during the normal human pregnancy. Fetal cells have been found to persist in the maternal peripheral blood for many years after pregnancy (microchimerism). It will be noted that fetal cells in the blood and skin of SSD patients occur more frequently and in substantially higher concentrations than in healthy subjects. Many autoimmune disorders afflict women but in SSD, Sjogren's disease, and primary biliary cirrhosis case rates are at their highest at the post-childbearing age. The well-known condition of chimerism, graft-versus-host disease, has clinical similarities to some autoimmune diseases, notably systemic sclerosis (scleroderma). Results of reported studies as well as our data secured in the investigation of 190 patients with scleroderma lend support to the hypothesis put forward that microchimerism may be involved in the pathogenesis of scleroderma. If this hypothesis is confirmed new treatment modalities will be offered to scleroderma patients.

Adult↗
PubMed1995

[Mechanisms of cardiac involvement in systemic scleroderma. Apropos of 3 cases].

To investigate myocardial manifestations in progressive systemic sclerosis, we studied three patients with transthoracic echocardiography, cardiac catheterization with coronary angiography, thallium scans with dipyridamol completed by thoracic cold exposure and endomyocardial biopsy with immunohistology. Two patients were symptomatic. In the three cases, abnormalities of myocardial perfusion were detected without coronary stenosis. Two patients had myocardial fibrosis with a coronary spasm in one case. The immunohistological study always showed an unusual expression of HLA class II antigen on cardiac fibroblasts.

Aged↗
PubMed1986

[Systemic scleroderma. Contribution of esophageal manometry].

Oesophageal motor function was studied by oesophageal manometry in 48 patients with progressive systemic sclerosis: 25 with proximal scleroderma and 23 with diffuse scleroderma. Oesophageal lesions were noted in 70% (74% in diffuse scleroderma; 64% in proximal scleroderma). Classical manometric signs of scleroderma were found in only 31% of patients. Peristaltic modifications might begin at the junction of the two muscular coats, since a four centimeter long aperistaltic suspended area was noted in that region in 20% of patients, especially in the proximal scleroderma group. Oesophageal motility and low lower oesophageal sphincter pressure account for the gastro-oesophageal reflux and may compromise respiratory function, as suggested by the high frequency of concurrent oesophageal and respiratory dysfunction in diffuse scleroderma. Systematic prevention of gastro-oesophageal reflux should perhaps be advocated as soon as abnormalities in oesophageal motility are diagnosed.

Adolescent↗
PubMed2001

[Effects of acute pharmacological blockade of the renin-angiotensin system on intrarenal hemodynamics in patients with systemic lupus erythematosus and systemic scleroderma].

AIM: To evaluate effects of an ACE inhibitor captopril on intrarenal hemodynamics during acute test in patients with lupus nephritis (LN) and chronic sclerodermic nephropathy (CSN). MATERIAL AND METHODS: Renal hemodynamics was studied using ultrasound duplex scanning of renal vessels (UDS) on Vingmed SD-100 unit in 5 patients with SLE with renal affection, 5 patients with scleroderma systematica (SS) with renal affection. The hemodynamic parameters were measured before and 1 hour after administration of 50 mg captopril. RESULTS: LN were found to have low arterial perfusion. CSN patients exhibited low intrarenal blood flow in high resistance of small vessels. Captopril improved intrarenal hemodynamics (enhancement of linear and volume blood flow) in LN patients; in CSN renal artery dilated and resistance of small vessels reduced while renal arterial perfusion increased. CONCLUSION: Improvement of intrarenal hemodynamics in LN and CSN patients in response to captopril allows to recommend administration of ACE inhibitors in SLE and SS with renal damage. Ultrasonic duplex scanning of renal vessels provides detailed information about the condition of intrarenal vessels without invasion.

Angiotensin-Converting Enzyme Inhibitors↗
PubMed1993

Effects of cytokine application on glucocorticoid secretion in an animal model for systemic scleroderma.

We previously reported on an altered immune-endocrine feedback loop via the hypothalamo-pituitary-adrenal (HPA) axis in Obese strain (OS) chickens afflicted with spontaneous autoimmune thyroiditis. These animals are deficient in plasma corticosterone increase after antigenic challenge or application of cytokine-containing conditioned medium of mitogen-stimulated spleen cells (CM). To investigate whether the impaired ability to respond to cytokines with glucocorticoid-increasing factor (GIF) activity, e.g. interleukin 1 (IL 1), is restricted to OS chickens as a model for an organ-specific autoimmune disease, we extended our experiments to another autoimmune-prone animal strain, the chickens of the University of California at Davis line 200 (UCD-200). These animals develop an inherited inflammatory fibrotic disease that closely resembles human progressive systemic sclerosis (scleroderma). Application of GIF-containing CM to UCD-200 chickens leads to a transient increase in glucocorticoid serum levels within 1-2 hours comparable to that of controls. But, while corticosterone levels in the latter returned to normal baseline levels after 4 hours, they were still elevated in autoimmune chickens. Although the peak of the glucocorticoid hormone serum concentrations was equal to that of controls, UCD-200 had to secrete twice as much adrenocorticotropic hormone to achieve this corticosterone serum level due to an apparent hyporesponsiveness of the adrenal gland to this secretagogue. The altered cytokine-induced glucocorticoid secretion is found in early as well as in chronic, sclerotic stages of the disease. Cellular alterations in the peripheral blood of UCD-200 chickens during the prolonged elevated corticosterone section, i.e. between 2-4 hours after CM application, are characterized by a significant decrease in the percentage of CD4+ and CD8+ cells. Furthermore, a significant increase in B cells up to 24 hours with a maximum after 1 hour was found. The proliferative response to the mitogen concanavalin A of peripheral mononuclear cells was inversely correlated to the serum corticosterone level, showing a permanent decrease of 80-90% after 1-4 hours in autoimmune animals. This functional alteration in UCD-200 was accompanied by an 80% decrease in serum interleukin 2 (sIL 2) activity 4 hours after CM application. Twenty-four hours later an eight-fold increase in sIL 2 rebound activity was found, indicating that the inhibitory effect of corticosterone in UCD-200 chickens is not long-lasting.

Adrenocorticotropic Hormone↗
PubMed1993

Activation of dermal connective tissue in scleroderma.

Systemic scleroderma is an acquired disorder which typically results in fibrosis of the skin and internal organs. The pathogenesis of systemic scleroderma is characterized by three distinct processes: microvascular alterations including capillary endothelial cell injury, perivascular inflammatory reaction in dermis, and excessive accumulation of collagen in the dermal layer of lesional skin. In this review, molecular mechanisms resulting in activation of collagen synthesis by dermal fibroblasts in scleroderma are discussed. Specifically, the role of inflammatory cells and the cytokines/growth factors produced by these cells in the pathogenesis of scleroderma is emphasized. The possibilities for prevention and resolution of tissue fibrosis on the basis of these observations are also discussed. Understanding the pathogenetic mechanisms of scleroderma at a molecular level is likely to provide possibilities for development of more specific therapeutic modalities for this and other fibrotic disorders.

Cell Adhesion Molecules↗
PubMed1991

Expression of intercellular adhesion molecule-1 (ICAM-1) in the skin of patients with systemic scleroderma.

The expression and tissue distribution of intercellular adhesion molecule-1 (ICAM-1) in skin biopsies from 12 patients with systemic (SSc) and localized (LS) scleroderma was studied and compared to the biopsies from patients with lupus erythematosus (LE) and normal individuals. In normal human skin ICAM-1 expression was restricted to the vascular endothelium, infiltrating mononuclear cells (MNC), and to few individual keratinocytes. In the inflammatory stage of SSc, however, the expression of ICAM-1 was dramatically increased at the site of MNC infiltrates and could also be detected on fibroblast-like cells lying well apart from these infiltrates in the deep dermis. In contrast, in LS ICAM-1 was expressed mainly at the sites of MNC infiltrates. In LE ICAM-1 expression was confined to the keratinocytes, endothelial cells, and mononuclear cells in the upper parts of the dermis. Analysis of serial tissue sections from patients with SSc demonstrated also colocalization of staining of ICAM-1 around blood vessels with LFA-1-positive lymphocytes. Increased expression of ICAM-1 in the dermis of patients with SSc may represent an important mechanism by which MNC become localized and retained at a site of connective tissue inflammation, leading to the activation of fibroblasts.

Adult↗