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PubMed2003

The efficacy of oral cyclophosphamide plus prednisolone in early diffuse systemic sclerosis.

Pharmacological treatment of diffuse systemic sclerosis (SSc) directed at the tissue fibrosis has generally been ineffective. Many immunosuppressive drugs have been tried as therapy for SSc, regardless of the disease subtype and/or stage. The aim of this study was to show the efficacy and the toxicity of oral cyclophosphamide and prednisolone therapy on the prevention of fibrosis-based tissue damage in the early stages of the diffuse SSc. Twenty-seven patients with early diffuse SSc were treated with oral cyclophosphamide (1-2 mg/kg/day) plus oral prednisolone (40 mg/every other day) between the years 1995 and 1998. The results regarding the efficacy and toxicity of cyclophosphamide were compared with those of 22 early SSc patients who had been treated with oral D-penicillamine between 1992 and 1995. All the patients were evaluated using clinical and laboratory parameters every 6 months for 2 years. There was a significant improvement on the skin score, maximal oral opening, flexion index, predicted forced vital capacity (FVC) and carbon monoxide diffusing capacity (DLCO) in the cyclophosphamide group. The decrease in skin score in the cyclophosphamide group started earlier than in the D-penicillamine group. No life-threatening or irreversible adverse reaction was observed. This open study supports the use of oral cyclophosphamide plus prednisolone therapy to prevent fibrosis and its complications in the early stages of diffuse SSc.

Administration, Oral↗
PubMed1998

Diversity of myocardial involvement in systemic sclerosis: an 8-year study of 95 Japanese patients.

BACKGROUND: Myocardial fibrosis has been reported to be an important complication of systemic sclerosis (SSc). Left ventricular (LV) hypertrophy has also been reported, but its significance is unclear. Correlations between cardiac complications and clinical or serologic subsets of SSc have not been previously evaluated by long-term observational studies. METHODS: We correlated the findings of echocardiography, electrocardiography, and autopsy with clinical and serologic features in 95 patients with SSc who were monitored for up to 8 years. RESULTS: Septal thickness determined by echocardiography was significantly greater in patients with anti-nuclear ribonucleoprotein (nRNP) antibodies than in patients with anti-Scl70 or anticentromere antibodies. Fractional shortening was reduced in patients with diffuse cutaneous involvement or anti-Scl70 antibodies. Ten patients had cor pulmonale, which was confirmed by echocardiography. Four of them had diffuse cutaneous involvement and advanced pulmonary fibrosis, whereas four had anti-nRNP antibodies and mild or no fibrosis. Septal hypertrophy was detected in 12 patients, 10 of whom had limited cutaneous involvement and six had anti-nRNP antibodies. Diffuse or regional LV wall hypokinesia was seen in seven patients, of whom six had anti-Scl70 antibodies and five had diffuse cutaneous involvement. Six patients with cor pulmonale, five with abnormal LV wall motion, and three with septal hypertrophy died during the follow-up period. Autopsy revealed LV hypertrophy in three with septal hypertrophy, disarray and irregular hypertrophy of myocytes in one of them, and extensive myocardial fibrosis in one with diffuse LV wall hypokinesia. CONCLUSIONS: Septal hypertrophy and cor pulmonale caused by pulmonary vasculopathy were associated with limited cutaneous involvement or anti-nRNP antibodies, whereas abnormal LV wall motion and cor pulmonale caused by advanced pulmonary fibrosis were mostly seen in patients with diffuse cutaneous involvement or anti-Scl70 antibodies.

Adult↗
PubMed2006

The role of DMARDs in systemic sclerosis therapy.

The aim of this review is to evaluate the evidence for disease-modifying anti-rheumatic drugs (DMARDs) for treatment of systemic sclerosis (SSc). In the previously published trials, DMARD therapy was usually initiated for severe skin thickening, organ involvement and alveolitis. These studies suggest beneficial effects of methotrexate, azathioprine, ciclosporine A and cyclophosphamide therapy in SSc patients. However, many of these data were derived from retrospective analyses with low numbers of patients, short-term follow-up and often without an appropriate control group. Finally, some of these studies led to inconsistent results. At the present time there is no DMARD therapy of proven efficacy in SSc. Immunosuppressive therapy should only be considered in patients with early diffuse disease, overlap syndromes or pulmonary fibrosis. Current expert recommendations suggest a therapy with methotrexate for skin thickening or cyclophosphamide for acute alveolitis. However, more clinical trials with larger numbers of patients with recent onset SSc are needed.

Antirheumatic Agents↗
PubMed1976

[Lung biopsy in interstitial pulmonary processes].

The value of open lung biopsies for the diagnosis of diffuse interstitial lung fibrosis is discussed. Except for few cases, e.g. granulomatous processes, needle biopsies are not a reliable method. In diffuse lung fibroses of unknown etiology which are not related to systemic diseases such as collagen diseases, combined electronmicroscopy and EDAX (Energy Dispersive X-ray Analysis) may reveal pneumoconiotic lesions due to unperceived mineral-dust exposure.

Biopsy↗
PubMedDate not supplied

An evaluation of some inflammatory, coagulative and immune factors in progressive systemic sclerosis.

Thirty-five patients affected by Progressive System Sclerosis (PSS) (20 acrosclerosis and 15 diffuse sclerosis) were subjected to the following laboratory tests: Beta 2 microglobulin (B2m), Fibronectin, C3c, C4, Fibrinogen, Factor VIII Coagulant (F. VIII:C), Factor VIII related Antigen (F. VIII:Ag), IgG, ESR, Antithrombin III (AT III), alpha 1 Antitrypsin (alpha 1AT) and alpha 2 Macroglobulin (alpha 2M). The results showed that no difference was observed between the normal group and the groups of PSS patients with respect to alpha 1AT, alpha 2M, and AT III. In contrast the B2m, ESR, IgG, F. VIII:Ag results were significantly higher in the PSS patients, while Fibrinogen and C3c results were slightly higher, but no high enough to be of statistical significance. The two PSS patient subgroups did not produce significant results, only the C4 values were lower in the Diffuse Sclerosis subgroup. From these results it seem that the tests used are not very useful indifferentiating the two subgroups of the PSS patients.

Adult↗
PubMed2006

Unique characteristics of systemic sclerosis sine scleroderma-associated interstitial lung disease.

STUDY OBJECTIVES: To describe the characteristics of systemic sclerosis sine scleroderma (ssSSc)-associated interstitial lung disease (ILD) presenting as idiopathic interstitial pneumonia (IIP). DESIGN: Retrospective review of six patients with ssSSc-associated ILD diagnosed after referral for evaluation of IIP. MEASUREMENT AND RESULTS: All patients were white, their mean age was 56 years (range, 37 to 86), and gender was evenly divided. Sclerodactyly, skin thickening, and digital edema were absent in all patients. All patients had scattered telangiectasia, and four patients had Raynaud phenomenon with abnormal nailfold capillaroscopy findings. All described gastroesophageal reflux, and three patients had esophageal dysmotility by esophagography. All had restrictive pulmonary physiology and a reduced diffusion capacity. High-resolution CT revealed nonspecific interstitial pneumonia (NSIP) or usual interstitial pneumonia (UIP) radiographic patterns. Of the three patients who underwent surgical lung biopsy, two patients had NSIP and one patient had UIP pathologic patterns. Five patients had asymptomatic pericardial effusions and elevated pulmonary artery pressures by echocardiography. All patients had nucleolar-staining anti-nuclear antibodies (ANAs), and one patient was anti-Scl-70 positive. All five anti-Scl-70-negative patients were anti-Th/To positive, and the anti-Scl-70-positive patient was anti-Th/To negative. CONCLUSIONS: In the presentation of an IIP, the presence of a nucleolar-staining ANA, telangiectasia, Raynaud phenomenon with abnormal capillaroscopy findings, gastroesophageal reflux, or pericardial disease suggests underlying systemic sclerosis. These findings should aid clinicians in the evaluation and treatment of patients with otherwise undefined ILD.

Adult↗
PubMed2000

Evidence of cerebral hypoperfusion in scleroderma patients.

OBJECTIVES: To investigate regional cerebral blood flow by (99m)Tc-hexamethylpropylenamineoxime (HMPAO) single photon emission computed tomography (SPECT) in a series of 40 patients (mean age 58.5+/-11.5 yr) affected by systemic sclerosis (SSc) in comparison with age-matched healthy controls. METHODS: Subjects affected by concomitant severe pathologies that might interfere with the interpretation of the SPECT results were excluded. SPECT findings were correlated with the severity of peripheral microvascular involvement, as assessed by nailfold videocapillaroscopy (NVC). Whenever possible, patients underwent magnetic resonance imaging (MRI) of the brain. RESULTS: Twenty-one SSc patients (52%) showed hypoperfusion in two or more regions of interest (ROIs) at the SPECT analysis. MRI was available in 14 of these patients, and was shown to be altered in eight of them (57%). One patient with both abnormal SPECT and abnormal MRI was affected by mild cognitive impairment. Transcranial Doppler sonography was normal in all but one of these patients with hypoperfusion. Nineteen patients exhibited a normal brain SPECT scan, but the MRI was shown to be altered in 3/12 of them (25%). No significant differences were found between the group of SSc patients showing hypoperfusion and those showing a normal SPECT scan regarding age, the duration of disease, the presence of vascular risk factors or damage of other organs typically involved in the disease, and the severity of peripheral microvascular involvement (NVC). CONCLUSIONS: Focal or diffuse cerebral hypoperfusion was found in more than half of the neurologically asymptomatic SSc patients studied, paralleling the incidence of altered brain MRI. The hypoperfusion was not linked to ageing and possibly reflects the cerebral location of the microangiopathic process characterizing the disease.

Adult↗
PubMed2001

European multicentre study to define disease activity criteria for systemic sclerosis. I. Clinical and epidemiological features of 290 patients from 19 centres.

OBJECTIVE: To investigate the existence of differences among European referral centres for systemic sclerosis (SSc) in the pattern of attendance and referral and in the clinical and therapeutical approaches. METHODS: In 1995 the European Scleroderma Study Group initiated a multicentre prospective one year study whose aim was to define the disease activity criteria in SSc. During the study period each participating European centre was asked to enroll consecutive patients satisfying American College of Rheumatology criteria for SSc and to fill out for each of them a standardised clinical chart. Patients from various centres were compared and differences in epidemiological, clinical, and therapeutical aspects were analysed. RESULTS: Nineteen different medical research centres consecutively recruited 290 patients. The patients could be divided into two subgroups: 173 with the limited (lSSc) and 117 with the diffuse (dSSc) form of the disease. The clinical and serological findings for the series of 290 patients seemed to be similar to data previously reported. However, when the data were analysed to elicit any differences between the participating centres, a high degree of variability emerged, in both epidemiological and clinical features and in the diagnostic and therapeutic approaches to the disease. CONCLUSIONS: The clinical approach to SSc, not only in different countries but also in different centres within the same country, is not yet standardised. To overcome this problem, it will be necessary for the scientific community to draw up a standardised procedure for the management of patients with SSc. This would provide a common research tool for different centres engaged in research on this complex disease.

Adolescent↗
PubMed2004

Cytokine and chemokine levels in systemic sclerosis: relationship with cutaneous and internal organ involvement.

Systemic sclerosis (SSc) is a connective tissue disorder characterized by excessive collagen deposition in the skin and internal organs. Several cytokines and chemokines have been implicated in the induction of fibrosis, but a definitive relationship between specific cytokines and organ involvement has not been established yet. Serum samples, PBMC and T cell lines (TCL) obtained from 54 patients affected by SSc and 20 healthy donors (HD) were examined by ELISA for Interferon-gamma (IFN-gamma ), interleukin (IL)-4, IL-6, IL-10, IL-18, Transforming growth factor (TGF)-beta1, Tumour necrosis factor (TNF)-alpha, sCD30, Macrophage derived chemokine (MDC), Monocyte chemoattractant protein (MCP)-1, Macrophage inflammatory protein (MIP)-1alpha and Regulated on activation normal T-cell expressed and secreted (RANTES). In all the SSc serum samples, we found significantly increased levels of IL6, TNFalpha and MCP-1 but reduced amounts of gamma-IFN and MDC. IL6, IL10, IL18, MIP-1alpha and TNFalpha measured in supernatants from PHA-stimulated PBMC and IL6, MCP-1 and RANTES in supernatants from stimulated TCL were also increased in patients. MDC was decreased in all the biological SSc sources studied. TGF-beta1, IL10, and sCD30 were produced at a significantly lower level by SSc TCL. Serum IL6 and sCD30 levels were significantly increased in dc-SSc patients compared to lc-SSc as were levels of MCP-1 produced by PBMC and IL10 from TCL. We observed a strict relationship between pulmonary fibrosis and IL10, MCP-1 (both from TCL) and serum IL6. Kidney involvement was related to serum MCP-1 levels and IL18 production from PBMC. Oesophageal involvement correlated with MDC production from PBMC and IL10 synthesis by TCL. We showed that IL-6, IL-10, MDC and MCP-1 are variably associated with internal organ involvement and allow the discrimination between limited and diffuse forms of the disease.

Cell Line↗
PubMed2003

[Soluble receptors of cytokines in sera of patients with systemic sclerosis--clinical correlation].

The aim of this study was to examine the levels of some soluble receptors of cytokines in sera from patients with systemic sclerosis (SSc) and to evaluate their role as parameters of disease activity and severity. Fifty-four patients with SSc were included in the study. Concentrations of the soluble receptors: sIL-2R, sTNF alpha RI and sIL-6R were determined by an enzyme-linked immunosorbent assay-ELISA. Controls were healthy age- and sex-matched volunteers. The study demonstrated that soluble receptors levels: sII-2R, sIL-6R and sTNF alpha RI were elevated in SSc patients and correlated with progress and severity of the disease. The most important correlations were found for sIL-2R levels, which were increased in 66.7% of patients. The raised levels of sTNF alpha RI were found in 32.4% of patients, and sIL-6R in 23.7% of patients. The concentrations were elevated in diffuse form of SSc and in patients with rapid systemic progression.

Adult↗
PubMedDate not supplied

[Significance of speckled staining of epidermal nuclei in direct immunofluorescence. 10 cases (author's transl)].

This study involved 10 patients having, in direct immunofluorescence, with covered healthy skin, a speckled IgG (+/-IgM) staining of the epidermal nuclei. The chief clinical signs seen and their respective percentages were: Raynaud's syndrome (80%), arthralgia (80%), diffuse or localised alopoecia (60%), muscular disease (40%), swollen fingers (40%), sclerodactylie (20%), cutaneous sclerosis extending beyond the extremities (30%), cutaneous signs of lupus erythematosus (30%), renal involvement (10%). In nine cases out of ten there were circulating anti-ENA antibodies at high levels, divided into anti-RNP antibodies (7/10), anti-Sm antibodies (1/10) and anti-RNP and anti-Sm antibodies (1/10). Diagnoses were divided into: lupus erythematosus (3/10), systemic scleroderma (3/10), Sharp's mixed connective tissue disease (MCTD) (3/10) and non-classified connective tissue disease (1/10). The combination of speckled staining of epidermal nuclei and circulating anti-ENA antibodies cannot be considered to be specific of any particular type of connective tissue disease and the prognosis of the disease does not appear to differ from that of the usual prognosis of connective tissue disease with anti-ENA antibodies.

Adolescent↗
PubMed1991

Immunological markers of the subsets of systemic scleroderma and its overlap.

The studies performed repeatedly in 298 cases of systemic scleroderma (SSc) disclosed high prevalence of antinuclear antibody (in about 95% of the cases) with the use of HEp-2 cells as the substrate for indirect immunofluorescence method. Anticentromere antibody (ACA) was found in 25% of acrosclerosis cases, but in 50% of patients with cutaneous involvement limited to the digits, i.e. almost without cutaneous indurations. The visceral involvement was comparable in cases positive and negative for ACA, and a follow-up of these patients showed that the course, although protracted, was not always mild as previously believed. Scl 70 antibody proved to be a most characteristic marker of SSc, appearing in about 87% of diffuse variety and in above 50% of acrosclerosis cases. The most important finding was the detection of the coexistence of ACA and Scl 70 antibody in 10 of 180 cases studied by three methods: indirect immunofluorescence, double immunodiffusion, (for Scl 70) and immunoblotting with recombinant kinetochore antigen for ACA and recombinant Topoisomerase I antigen for Scl 70 antibody. These antibodies, believed to be mutually exclusive, are most often missed due to masking effect on HEp-2 cells by the stronger antibody, usually Scl 70. Its detection proved to be possible by evaluation of different serum dilutions and several cell mitoses, and confirmed by immunoblotting with recombinant antigens. Of practical significance are the clinical correlations between Pm-Scl antibodies, showing homogeneous nucleolar staining on HEp-2 cells, and overlap or atypical SSc cases with concomitant muscle involvement. This antibody was found to have a surprisingly high association with atypical SSc or scleromyositis of children.

Antibodies, Antinuclear↗
PubMed1996

Signal-averaged electrocardiography and echocardiography in the evaluation of myocardial involvement in progressive systemic sclerosis.

To assess the myocardial involvement in progressive systemic sclerosis we evaluated the presence of late potentials by signal-averaged electrocardiography (signal-averaged ECG) and the left ventricular function by M-mode, two dimensional and Doppler echocardiography. Fifteen outpatients, 7 with diffuse progressive systemic sclerosis and 8 with CREST syndrome variant, without clinical or electrocardiographic evidence of cardiac disease were studied and compared with 18 normal subjects. Late potentials occurred in 5 out of 15 progressive systemic sclerosis patients (33%) with a significant difference versus controls (P < 0.05) and were present only in the patients with diffuse progressive systemic sclerosis (P < or = 0.001 vs. controls). All progressive systemic sclerosis patients showed a normal left ventricular systolic function. Abnormal left ventricular filling was found in 9 progressive systemic sclerosis patients (5 with diffuse progressive systemic sclerosis and 4 with CREST). A more severe impairment of the mean values of diastolic function indexes was found in diffuse progressive systemic sclerosis than in CREST. In all diffuse progressive systemic sclerosis patients at least one method showed altered results, whereas half the CREST patients showed no pathological findings with both techniques. These results confirm a lower myocardial involvement in the CREST syndrome than in diffuse progressive systemic sclerosis and consequently this is probably related to a better prognosis.

Adult↗
PubMed2001

Antibodies against oxidized low-density lipoproteins in systemic sclerosis.

OBJECTIVE: To investigate whether circulating concentrations of antibodies against oxidized low-density lipoproteins (LDL) are increased in patients with systemic sclerosis (SSc). METHODS: Oxidation of LDL and anti-oxidized LDL antibodies were measured in 26 patients with limited cutaneous SSc (LCSSc), in eight patients with diffuse cutaneous SSc (DCSSc) and in 24 healthy control subjects. Results were adjusted for age, sex and smoking. RESULTS: Binding to oxidized LDL was increased in patients with both limited and diffuse cutaneous disease (geometric mean 0.35 and 0.39 optical density units respectively) compared with controls (0.28) (P=0.03 and P=0.01 respectively). Circulating concentrations of anti-oxidized LDL were increased only in patients with diffuse SSc (geometric mean 0.22 optical density units) compared with controls (geometric mean 0.16, P=0.02). CONCLUSION: These preliminary findings lend further weight to the concept that oxidation of LDL contributes to the vascular pathology of SSc, particularly in patients with diffuse cutaneous disease. Prospective longitudinal studies are required to investigate whether anti-oxidized LDL antibodies may be a marker of vascular damage in SSC.

Adult↗
PubMed2003

Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population.

OBJECTIVE: To estimate the prevalence, incidence, survival, and disease characteristics of systemic sclerosis (SSc) in the Detroit tricounty area. METHODS: A census of SSc cases for the period 1989-1991 was conducted in the Detroit area, using multiple sources for case identification. Diagnoses were verified by medical record review. Capture-recapture analysis was used to estimate the total SSc population. Cases of localized scleroderma (morphea and linear disease) were excluded. RESULTS: Based on 706 verified cases of SSc, prevalence was initially estimated to be 242.0 cases per million adults (95% confidence interval [95% CI] 213-274), with an annual incidence of 19.3 new cases per million adults per year (95% CI 12.4-30.2). Capture-recapture analysis, based on the degree of overlap of verified cases among multiple sources, resulted in a revised prevalence estimate of 276 cases per million adults (95% CI 245-310). Sex- and race-specific prevalence estimates were significantly higher for women than for men, and for blacks than for whites. The average age at diagnosis was significantly younger for blacks than for whites. Compared with white patients, black patients were almost twice as likely to have diffuse disease (prevalence proportion ratio 1.86, 95% CI 1.48-2.35). Median survival was approximately 11 years. Factors negatively affecting survival included male sex (hazard ratio 1.81, 95% CI 1.29-2.55) and older age at diagnosis (hazard ratio 1.04, 95% CI 1.03-1.05). CONCLUSION: This study establishes baseline estimates of SSc occurrence and characteristics in a large US cohort consisting primarily of black adults and white adults. These data should facilitate research regarding the role of geographic, ethnic, racial, and environmental factors for this disease in comparison populations.

Adult↗
PubMed1995

A novel cell-cycle-dependent 350-kDa nuclear protein: C-terminal domain sufficient for nuclear localization.

We have screened human scleroderma patients for immunoreactivity with the components of the nucleus and the mitotic apparatus. We announce the identification of a novel cell-cycle-dependent nuclear protein using serum from a CREST patient AH. AH protein first appears at the nucleus of G2-phase and associates with the centrosome throughout the cell cycle. As chromosomes condense during the prophase, AH protein becomes enriched at the kinetochores. During mitosis, AH protein progressively disperses from the kinetochore and becomes diffusely localized in the cytoplasm and in telophase; it appears to be enriched within the intracellular bridge. Molecular cloning and transfection studies reveal that the 350-kDa AH protein contains a coiled-coil and a globular domain at the C-terminus that is sufficient for nuclear localization.

Amino Acid Sequence↗
PubMed2005

Healthy children have a significantly increased skin score assessed with the modified Rodnan skin score.

OBJECTIVES: The modified Rodnan skin score (MRSS) is used as a primary outcome measure in most therapeutic trials in systemic sclerosis (SSc) in adults. Before we can apply this outcome measure in trials in juvenile patients with SSc, we need to evaluate this assessment method in children without sclerodermatous skin changes, to establish values for the normal paediatric population. METHODS: To determine the MRSS in healthy paediatric population, patients of the paediatric rheumatology out-patient clinic with mechanical pain or with juvenile idiopathic arthritis at the age of 16 yr or under were assessed between 1 January and 31 March 2004. Patients with any sign of connective tissue disease or skin disorders, such as psoriasis or ectopic dermatitis, were excluded. The MRSS was determined at a standardized location and with a standardized pinching method. RESULTS: Two hundred and seventeen patients, including 100 females, were assessed. The mean age of the patients was 10.5 yr (2.9-16), the mean body mass index (BMI) was 18.3 (9.3-35.7), and the mean MRSS was 13.92 (range 4-25). The MRSS score showed a difference between males and females at every Tanner stage. There was a linear correlation between MRSS and body mass index independently of age and Tanner stage. CONCLUSION: The mean MRSS in healthy children is 13.92 units and this range would be expected in a patient with a diffuse form of SSc. The MRSS score in children correlates with the body mass index and the Tanner stage, so it should be corrected to these parameters, according to this pilot study.

Adolescent↗
PubMed1994

Plasma TGF beta in systemic sclerosis: a cross-sectional study.

OBJECTIVES: To determine whether the active 25 kDa form of the fibrogenic cytokine transforming growth factor beta (TGF beta) can be detected in plasma from patients with systemic sclerosis and to examine the relationship between plasma TGF beta and clinical markers of disease severity and serum concentrations of the aminoterminal peptide of type III procollagen (PIIINP) (a laboratory marker of the fibrotic process). METHODS: A cross sectional study was made of 39 patients with systemic sclerosis (11 diffuse and 28 limited), nine patients with primary Raynaud's disease and 60 healthy controls. TGF beta 1 and TGF beta 2 were measured by enzyme linked immunosorbent assay (ELISA) (sensitivity 100 pg/ml) and PIIINP by radioimmunoassay. RESULTS: TGF beta 1 was detected in plasma from six of 39 patients with systemic sclerosis but not in any patient with primary Raynaud's disease or healthy controls. TGF beta 2 was not detected in plasma from patients or controls. No clear relationship was demonstrated between TGF beta 1, clinical features or PIIINP concentrations. CONCLUSIONS: The 25 kDa form of TGF beta 1 can be detected in the plasma of some patients with systemic sclerosis. This provides limited support for the hypothesis that this cytokine plays a role in the pathogenesis of this disease. However, longitudinal studies, particularly in early diffuse disease, are required to clarify the relationship between circulating TGF beta 1 and disease activity.

Adult↗