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PubMed1999

Systemic sclerosis prevalence and mortality in Sydney 1974-88.

BACKGROUND: Systemic sclerosis prevalence and mortality estimates have demonstrated wide variability. The sole Australian study published to date demonstrated high prevalence rates when compared to overseas estimates. The prevalence and mortality findings reported in this paper derive from a larger study which addressed the distribution and determinants of systemic sclerosis within Sydney. AIMS: To determine systemic sclerosis prevalence and mortality rates within Sydney over 15 years, 1974-88. METHODS: Cases were ascertained from multiple sources including death certificates, hospitals, physicians, vascular surgeons' and dermatologists' private practices, a systemic sclerosis self-help group and private medical laboratories. RESULTS: Overall, 715 cases were identified. Females comprised 77% (95% CI: 74-80) of cases. Disease of the limited subtype accounted for 79% (95% CI: 76-82) of all systemic sclerosis, being relatively more frequent in living than deceased cases, and in females than males. Crude prevalence estimates appeared to rise between 1975 (4.52/100,000 95% CI:3.75-5.29/100,000) and 1988 (8.62/100,000 95% CI:7.64-9.60/100,000) as did estimates of diffuse disease. However, diffuse disease prevalence, when expressed as a proportion of total disease prevalence, showed no significant temporal change. Although crude mortality rates also showed apparent temporal increases (0.24/100,000 in 1975 to 0.80/100,000 in 1988) standardised mortality rates showed less convincing trends (0.41/100,000 in 1976 and 0.40/100,000 in 1988). Death certificate-derived mortality rates provided relatively large underestimates of total mortality. However, these underestimates were relatively constant over time. CONCLUSIONS: This study has demonstrated systemic sclerosis prevalence and mortality rates comparable to overseas estimates, consistently higher prevalence and mortality rates in females than males, proportionally higher rates of diffuse disease in males than females and in deceased cases than living cases, a diffuse: limited disease ratio apparently stable over time, apparently increasing temporal prevalence and mortality rates and, by implication, rising incidence rates. The observed temporal rise in diffuse disease prevalence and the absence of a convincing fall in diffuse disease mortality suggests a rising temporal incidence rate of diffuse disease. Standardised mortality rates demonstrated less consistent trends than did crude mortality rates and failed to demonstrate convincing declines in mortality subsequent to the introduction of ACE inhibitors for management of systemic sclerosis renal disease. Death certificate-derived systemic sclerosis mortality rates considerably and consistently underestimated systemic sclerosis-all cause mortality.

Data Collection↗

PubMed2000

Ultraviolet A1 (340-400 nm) phototherapy for scleroderma in systemic sclerosis.

BACKGROUND: The presence of an inflammatory infiltrate consisting of helper T cells and a dysregulated matrix metabolism leading to excessive deposition of collagen are two pathogenetic factors responsible for the developments of fibrosis and sclerosis in patients with systemic sclerosis. In previous studies, ultraviolet A1 (UVA1) radiation phototherapy was shown to deplete skin-infiltrating T cells through the induction of T-cell apoptosis and to up-regulate the expression of matrixmetalloproteinase-1 (collagenase-1) in dermal fibroblasts. OBJECTIVE: Our purpose was to determine whether UVA1 phototherapy is effective for systemic sclerosis. METHODS: Lesional skin on the forearms of patients with systemic sclerosis (diffuse type, n =3; limited type, n =1) was exposed to medium-dose UVA1 radiation (60 J/cm(2)) daily. RESULTS: In all patients studied, UVA1 phototherapy-treated skin lesions were markedly softened after 9 to 29 exposures. Clinical improvement was associated with an increase in (1) joint passive range of motion values (P <.05), (2) skin temperature (thermography, P <.05), and (3) cutaneous elasticity (cutaneous elastometry, P <.05). Histologic evaluation of skin specimens obtained before and after UVA1 phototherapy revealed loosening of collagen bundles and the appearance of small collagen fibers. CONCLUSION: These studies indicate that UVA1 phototherapy is effective for patients with systemic sclerosis.

Aged↗

PubMed1999

Systemic sclerosis. A clinical overview.

Systemic Sclerosis (SSc) is a multisystem disease that affects the skin and internal organs (i.e., gastrointestinal tract, lung, heart, kidney and peripheral nervous system). In the early phase, lung involvement is characterized by interstitial inflammatory alterations that are detected by bronchoalveolar lavage analysis and high resolution computed tomography (ground glass). As the disease progresses, fibrotic changes become evident and the diffusing capacity for carbon monoxide (DLCO) is impaired. Cardiac involvement in SSc can be manifested as myocardial disease, pericardial disease, conduction system disease, or arrhythmias. Cardiac involvement is a poor prognostic factor, but the diagnosis may be late or missing because of the frequent discrepancy between clinical manifestations and the real cardiac involvement. For this reason, resort to all the available diagnostic procedures is recommended to achieve an early diagnosis. The motility disorders are a major feature of gastrointestinal involvement in SSc, striking any part of this system (especially esophagus and anorectal region). Kidney involvement and scleroderma renal crisis are now considered rare because of the introduction of ACE inhibitors. Some patients may develop myositis or erosive arthropathy that complicate enormously the joint retraction induced by skin fibrosis. The peripheral nervous system (PNS) is also targeted by SSc: a distal mononeuropathy of the median nerve is a frequent and early feature; autonomic nerve dysfunction (parasympathetic impairment and marked sympathetic overactivity) seems to be a fundamental etiologic factor linked to the development of microvascular, cardiac and gastrointestinal alterations. The whole approach to the SSc patient is very complex and must consider, at the same time, many organs and systems. Thus, a global vision of SSc patient is needed in order to assure an early diagnosis of specific organ involvement as well as early treatment. Systemic Sclerosis (SSc) is a multisystem disease, that affects the skin, the gastrointestinal tract, the lung, the heart and the kidney. The extent and severity of internal organ involvement are the more important factors influencing the disease outcome and prognosis in SSc. In recent years, it has become evident that early diagnosis and accurate staging of visceral involvement are fundamental for appropriate management and therapeutic approach to the disease. Diagnostic procedures for specific organ and system involvement are now more sensitive because of the continuous technological improvement and, mostly, because they take advantage of the studies carried out in other diseases by other medical branches. This review will consider briefly the most frequent and important organ involvement in SSc.

Gastrointestinal Diseases↗

PubMed1991

Diffuse systemic sclerosis and related diseases in Thailand.

Diffuse systemic sclerosis and the related syndromes were diagnosed in 115 patients during 1977-1987. Sixty-nine patients were available for clinical evaluation and analysis. These included 46 patients with diffuse systemic sclerosis, 2 with limited cutaneous systemic sclerosis, 2 with linear scleroderma, 18 with overlap syndrome (OS), and one with primary Raynaud's disease. The major clinical manifestations among the diffuse scleroderma patients were the involvement of the following systems; cutaneous (93.3%), musculoskeletal (69.6%), gastrointestinal (54.3%), and pulmonary (43.3%) while renal involvement (4.3%) was less common. Antinuclear antibodies were found in 89.7% with predominating speckle pattern (86.2%) and anti Scl 70 antibody (40.6%). In contrast to the Western series, HLA-DR2 was significantly increased in our diffuse systemic sclerosis patients (p less than 0.01). Among the 18 OS patients, the involvement of pulmonary system (44.4%) was the major cause of morbidity and renal involvement (55.6%) was found frequently although it was clinically mild. In conclusion, our diffuse systemic sclerosis and related diseases patients seem to have milder clinical manifestations than those of the Western series.

Adult↗

PubMedDate not supplied

Comparison of high frequency (20 MHz) ultrasound and palpation for the assessment of skin involvement in systemic sclerosis (scleroderma).

BACKGROUND AND OBJECTIVES: Systemic sclerosis (SSc) is a connective tissue disease characterized by microvascular changes and fibrosis of the skin and internal organs. Increased skin thickness proximal to the metacarpophalangeal joints is the single major diagnostic criterion. The aim of this study was to evaluate high frequency (20 MHz) ultrasound for the assessment of skin thickness in patients with SSc of different disease durations. METHODS: Skin thickness was measured with high frequency (20 MHz) ultrasound equipment (Dermascan) in 41 patients with SSc (23 women and 18 men) and in 41 controls. Twenty-five patients had limited cutaneous systemic sclerosis (ISSc), 12 had diffuse cutaneous systemic sclerosis (dSSc) and 4 had suspected SSc. RESULTS: Skin thickness of the forearm was inversely correlated to disease duration. Compared to controls, skin thickness was increased over the proximal phalanx of the right second finger and over the forearm in patients with a disease duration of 2 years or less. Assessments of skin thickness in 10 controls by 2 independent investigators showed an inter-observer variability of 1.0% for the proximal phalanx and 0.0016% for the forearm. Patients whose ultrasound showed increased skin thickness on the hand and forearm also had thickened skin by palpation. CONCLUSION: High frequency (20MHz) ultrasound is a feasible method for measuring skin thickness in SSc, and may be useful for diagnosis, long-term follow-up, and assessment in therapeutic studies.

Adult↗

PubMed1997

[Cognitive function in patients with systemic sclerosis].

Central nervous system involvement is seldom reported in patients with systemic sclerosis (SSc). Cognitive functions were determined in 21 patients with definite SSc and 42 healthy controls. Thyroid function was also measured in order to eliminate the effect of hypothyroidism on cognitive functioning. It was found that the SSc patients with normal thyroid function showed defective long-term and recent memory, learning ability, criticism, perception and visuo-perceptual skills, their simple reaction time was prolonged. Similar but less advanced cognitive defects were shown in the SSc patients with overt or latent hypothyroidism. The obtained results indicate that the central nervous system involvement is more common in patients with SSc than it has been reported earlier.

Adult↗

PubMed1988

Ketanserin in the treatment of progressive systemic sclerosis.

Now progressive systemic sclerosis (PSS) is considered a disease of small vessels with which many immunologic alterations are associated. The presence in the blood of large amounts of serotonin can be considered a very important aggravating factor able to cause the sclerodermic alterations. The authors have treated 10 PSS patients with ketanserin, a selective antagonist of the S2 serotonin receptors, which are found in small vessels and platelets. Their results show that ketanserin represents an efficacious and very well tolerated therapy for treatment of the initial vascular symptoms of PSS.

Adult↗

PubMed1982

The immunopathology of progressive systemic sclerosis (PSS).

Progressive systemic (sclerosis) is one of the most enigmatic of the rheumatic diseases. It is a connective tissue disorder of unknown etiology characterized by fibrosis in skin and internal organs. Although similar lesions are found with increased prevalence in workers exposed to coal, gold, silica, and polyvinyl chloride, most patients have had no known predisposing factors. Select reports of a familial occurrence of PSS have been observed but a definitive genetic basis is lacking and no clear associations with the major histocompatability complex have been demonstrated. Moreover, although a variety of immunologic abnormalities in patients with PSS have been reported, they are generally diffuse and non-diagnostic. Such abnormalities include defects in cell mediated immunity, increases in sera immunoglobulins, antinuclear antibodies, and cryoglobulins. In contrast to these non-specific findings, there appears to be significant evidence of a relationship between cell mediated immunity to collagen and appearance of scleroderma. For example, peripheral blood lymphocytes in patients with scleroderma undergo lymphocyte transformation when cultured with specific collagen preparations. The pathology of skin and internal organs in PSS generally reflects both collagen deposition and small vessel occlusion. All organ systems may be involved but mortality significantly increases with involvement of heart, kidney, or lung. Unfortunately, at present a reliable experimental model of PSS has not been found although similar immunopathology can be induced in homologous disease of rats and in chronic graft vs host disease of humans.

Animals↗

PubMedDate not supplied

[Systemic sclerosis in childhood].

Systemic sclerosis is a multisystem disease of unknown cause, characterized by inflammation, vascular and fibrotic changes with excessive accumulation of connective tissue. The lesions may involve the skin and various internal organs (kidney, lung, gastrointestinal tract and heart). The first symptom is usually Raynaud's phenomenon, followed by skin changes; at the beginning the skin is swollen and oedematous, and then becomes thick, taut, shiny and atrophic. The prognosis of SS depends mainly on the severity of visceral involvement. The treatment of SS consists of drugs that improve the microcirculation and reduce collagen proliferation, such as calcium-antagonists (nicardipine) and D-Penicillamine.

Adolescent↗

PubMed2004

Interleukin 1alpha single-nucleotide polymorphism associated with systemic sclerosis.

OBJECTIVE: In systemic sclerosis (SSc), constitutive expression of the proinflammatory and fibrogenic cytokine interleukin 1alpha (IL-1alpha) by dermal fibroblasts from the affected skin has been observed. We investigated the association of a single-nucleotide polymorphism at position -889 in the IL-1alpha gene in patients with SSc. METHODS: Genotyping of IL-1alpha-889 polymorphism was performed in 46 patients with SSc and in 150 healthy controls by polymerase chain reaction with sequence-specific primers. All subjects were unrelated Slovak Caucasians. RESULTS: In SSc patients, carriers of the IL-1alpha-889 T allele were significantly overrepresented in comparison with controls (63.0% vs 42.0%; p = 0.01, OR 2.3, 95% CI 1.2-4.6). The frequency of the IL-1alpha-889 T allele was increased in SSc patients (38.0%) in comparison with controls (25.7%; p = 0.02). CONCLUSION: The IL-1alpha-889 polymorphism, previously shown to predispose to increased IL-1 protein expression, may be involved in susceptibility to SSc.

Case-Control Studies↗

PubMed2001

Treating systemic sclerosis in 2001.

Systemic sclerosis (SS) is characterized by sclerosis of the dermis and internal organs and by vascular abnormalities. Although the pathophysiology of the disease has been partly elucidated, the efficacy of long-term treatments remains limited, with no significant increase in survival in prospective studies. Conventional drug treatments are disappointing in clinical practice, and in a recent prospective randomized study standard-dose D-penicillamine was not more effective than mini-dose D-penicillamine. New long-term treatments are emerging for diffuse SS, including cyclophosphamide for patients with progressive interstitial lung disease or stem cell transplantation for those with early organ involvement. The most effective treatments remain symptomatic, such as angiotensin-converting enzyme inhibitors for acute renal crisis, calcium channel antagonists for Raynaud's phenomenon, and proton pump inhibitors for the complications of gastroesophageal reflux. This review article focuses on long-term treatments that are most likely to be effective and suggests symptomatic treatment strategies tailored to specific organ involvements.

Antineoplastic Agents↗

PubMed1999

Cytogenetic study and FISH analysis in lymphocytes of systemic lupus erythematosus (SLE) and systemic sclerosis (SS) patients.

Systemic lupus erythematosus (SLE) and systemic sclerosis (SS) are autoimmune diseases characterized by the presence of antibodies against ubiquitous self antigens. The presence of clastogenic factors (CF) capable of inducing chromosome breakage has also been reported in the plasma of some patients. We aimed to assess basal frequency of cytogenetic damage in lymphocytes and presence of CF in the plasma of two groups of SLE and SS patients displaying a different antibody status (ACA-/Scl70+ or ACA+/Scl70-), using the micronucleus (MN) assay and FISH analysis with a pancentromeric DNA probe. As compared with controls, we found significantly higher MN frequencies in SS patients, but not in SLE patients. In addition, our data showed a significant prevalence of C-MN in SLE and ACA-/Scl70+ patients and of C + MN in ACA+/Scl70- patients. We observed a positive response in three out of the five CF experiments performed on plasma of SS patients. The three patients whose plasma caused MN induction were subtyped as ACA-/Scl70+, whereas the other subjects had ACAs. The same tests on six SLE patients gave negative results.

Adult↗

PubMed2005

Predictive markers for development of severe organ involvement in patients with systemic sclerosis.

Prognosis of systemic sclerosis (SSc) is associated with the extent of skin involvement and the presence of lung, heart, kidney, and/or digestive tract damage. Most patients do not require disease-modifying therapy. However, to avoid irreversible tissue injury, early detection of visceral involvement is crucial for prompt initiation of therapy. The aim of this study is to identify, among initial investigations performed at time of diagnosis, predictive markers for the development of severe organ involvement (SOI). We retrospectively reviewed the medical records of 41 patients with SSc who were followed for 5 years after diagnosis, including those who died because of SSc within this 5-year period. We considered 68 clinical, biological, and technical parameters obtained at time of diagnosis and studied their association with development of SOI during this 5-year period, using a multivariate logistic regression. Eighteen patients (44%) developed SOI, with a median delay of 1.5 years following diagnosis. Three independent markers were identified: reduced vital capacity, fulfilment of American College of Rheumatology criteria at diagnosis, and presence of rheumatoid factor. To identify patients at risk for developing severe disease, given the short delay between diagnosis of SSc and development of SOI, we recommend monitoring these markers at diagnosis.

Adult↗

PubMed1991

Altered T cell subpopulations and lymphocytes expressing natural killer cell phenotypes in patients with progressive systemic sclerosis.

Scleroderma (progressive systemic sclerosis [PSS]) is known to be associated with abnormal T cell immunoregulation. In the present study, we evaluated lymphocyte phenotypes in patients with PSS and normal control subjects by flow cytometry and monoclonal antibodies for total T (CD3), T suppressor (CD8), T helper (CD4), T helper-inducer (CDw29), T suppressor-inducer (CD45R), human leukocyte antigen, DR+B (CD19), DR+T, and natural killer subsets, HNK-1 (CD57) and NKH-1 (CD56) cells. Patients with PSS compared to normal subjects had significantly lower percentages of CD3+ (p less than 0.005) and CD8+ (p less than 0.05) (similar to several patients with rheumatoid arthritis also evaluated), as well as CD45R (p less than 0.05), T+DR+ (p less than 0.05), and NKH-1 (CD56) (p less than 0.0005) cells. Patients with PSS with late-limited or generalized disease had lower percentages of CD8+, CD19, NKH-1+, and CDw29, but higher percentages of CD4+, HNK-1, and CD45R cells compared to patients with early stage disease, but these results were not statistically significant. These unique alterations in patients with PSS may prove to be useful in monitoring the stage of disease activity for therapy and further define immunologic defects.

Adult↗

PubMed1986

Co-ordinate increase in the expression of type I and type III collagen genes in progressive systemic sclerosis fibroblasts.

Progressive systemic sclerosis (PSS), is a connective tissue disease characterized by excessive accumulation of collagen in the skin and various internal organs which is due, at least in part, to increased collagen production by PSS fibroblasts. In order to examine the molecular mechanisms responsible for this abnormality, we compared the kinetics of collagen biosynthesis, the intracellular degradation of collagen and the expression of Types I and III procollagen genes between normal and PSS dermal fibroblasts in culture. Two age- and sex-matched normal and PSS dermal fibroblast cell lines were studied. The results showed that the PSS cultures produced higher amounts of collagen than did normal fibroblasts and displayed an abnormal kinetic pattern. Furthermore, the PSS cells showed a slight but statistically significant increase in the fraction of collagen degraded intracellularly when compared with normal cells (23% against 18% respectively). The levels of mRNA for procollagen Types I and III were determined by Northern and dot-blot hybridization with specific cloned cDNA probes for alpha 1(I), alpha 2(I) and alpha 1(III) and it was found that they were 2-3-fold higher for each of the three chains in the PSS cell lines compared with the controls. These findings indicate, therefore, that the overproduction of collagen characteristic of PSS fibroblasts can be largely accounted for by the increased levels of collagen mRNA.

Cell Line↗

PubMed1981

Pharyngo-oesophageal dysphagia in systemic sclerosis.

A patient with systemic sclerosis developed recurrent dysphagia consistently localised to the pharyngo-oesophageal region. This was due to narrowing of the lumen as a result of gross thickening of the pharyngo-oesophageal muscles. Histologically the changes were typical of systemic sclerosis. The lower oesophagus was less involved. Eventually she died of aspiration pneumonia and respiratory arrest.

Deglutition Disorders↗

PubMed2003

[The role of extracellular matrix components in the T-cell activation in systemic sclerosis].

Patients with systemic sclerosis (SSc) show, in addition to tissue fibrosis, a variety of distinct vascular and immunological abnormalities. Extracellular matrix proteins (ECM) play an important role in the regulation of cell migration and expression of various genes involved in activation of immune reactions. The aim of our study was to examine the co-stimulatory effects of ECM components (type I and IV collagen, laminin, fibronectin) in patients with SSc and to examine the expression of beta 1-integrins (VLA-2/CD49b, VLA-3/CD49c and VLA-5/CD49e) on PBMC in these patients. We examined 66 patients with various types of SSc and 62 healthy volunteers. The results showed that fibronectin and type I collagen had the highest co-stimulatory activities in both SSc patients and controls, whereas laminin and type IV collagen were less potent co-stimulatory agents. Flow cytometry data showed an increased expression of integrins (VLA-2, VLA-3, VLA-5) in SSc T-cells in comparison to healthy volunteers. The results of the proliferation assays in patients with SSc and normal volunteers showed that only fibronectin stimulated CD3--dependent lymphocyte response. Fibronectin co-stimulated T-cell proliferation in patients with SSc which may be dependent on increased expression of integrins CD49c or CD49e or/and level of circulating T-cells bearing these markers. Increasing of the expression of integrins on PBMC can help their migration to the perivascular spaces and can stimulate them. Fibronectin could enhance activation of PBMC followed by production of some cytokines involved in fibrosis in SSc.

Adult↗

PubMed2002

High-dose immunosuppressive therapy for severe systemic sclerosis: initial outcomes.

Systemic sclerosis (SSc) is a multisystem disease of presumed autoimmune pathogenesis for which no proven effective treatment exists. High-dose immunosuppressive therapy (HDIT) has been proposed as an investigational treatment for severe autoimmune diseases. Nineteen patients with poor-prognosis SSc underwent HDIT. The median age was 40 years (range, 23-61 years), the median modified Rodnan skin score (a measure of dermal sclerosis) was 31, and the median DLCO was 57%. Conditioning therapy involved 800 cGy total body irradiation (TBI) (+/- lung shielding to approximately 200 cGy), 120 mg/kg cyclophosphamide, and 90 mg/kg equine antithymocyte globulin. CD34-selected granulocyte-colony-stimulating factor-mobilized autologous blood stem cells provided hematopoietic rescue. With median follow-up at 14.7 months, the Kaplan-Meier estimated 2-year survival rate was 79%. Three patients died of treatment complications and one of disease progression. Two of the first 8 patients had fatal regimen-related pulmonary injury, a complication not found among 11 subsequent patients who received lung shielding for TBI. Overall, internal organ functions were stable to slightly worse after HDIT, and 4 patients had progressive or nonresponsive disease. As measured by modified Rodnan skin scores and modified health assessment questionnaire disability index (mHAQ-DI) scores, significant disease responses occurred in 12 of 12 patients evaluated at 1 year after HDIT. In conclusion, though important treatment-related toxicities occurred after HDIT for SSc, modifications of initial approaches appear to reduce treatment risks. Responses in skin and mHAQ-DI scores exceed those reported with other therapies, suggesting that HDIT is a promising new therapy for SSc that should be evaluated in prospective randomized studies.

Adult↗