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Results for “Scleroderma, Limited”

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PubMed2001

Increased urinary pyridinoline cross-link compounds of collagen in patients with systemic sclerosis and Raynaud's phenomenon.

OBJECTIVE: To study concentration changes in collagen degradation markers in patients with diffuse and limited cutaneous systemic sclerosis and patients with scleroderma-related diseases. METHODS: Pyridinoline cross-link compounds were analysed in urine samples using high-performance liquid chromatography. Samples were analysed for pyridinoline (Pyr), deoxypyridinoline (Dpyr) and soft-tissue pyridinoline (stPyr) in patients with diffuse cutaneous systemic sclerosis (dcSSc, n=23) and limited cutaneous systemic sclerosis (lcSSc, n=48) and in patients with scleroderma-related diseases such as primary Raynaud's phenomenon (pRP, n=16) and secondary Raynaud's phenomenon (sRP, n=14). Healthy controls (n=18) and patients with post-menopausal osteoporosis (OP, n=35) were also investigated. RESULTS: Urinary Pyr, Dpyr and stPyr concentrations were significantly higher in patients with Raynaud's phenomenon and systemic sclerosis than in healthy controls. The highest concentrations (two to three times greater than in healthy controls) were found in patients with dcSSc. The stPyr concentration was significantly higher in patients with dcSSc than in those with lcSSc, sRP and pRP. No significant difference in stPyr concentration was found between the healthy controls and the OP group, suggesting that stPyr is derived from soft tissues rather than bone. The extent and severity of skin involvement, measured as a skin score, significantly correlated with the concentrations of stPyr and Pyr, whereas no such correlation was found for Dpyr. CONCLUSIONS: Increased urinary concentrations of piridinoline cross-links reflect alterations in collagen turnover in both Raynaud's phenomenon and systemic sclerosis. The close correlation between stPyr concentration and the extent of skin involvement in systemic sclerosis suggests that this parameter may be useful in monitoring ongoing fibrosis in this disease.

Amino Acids↗

PubMed1988

Reduced natural killer cell activity in patients with systemic sclerosis. Correlation with clinical disease type.

Natural killer (NK) cell number and function were determined in 69 systemic sclerosis (SSc) patients (41 with diffuse cutaneous SSc, 24 with limited cutaneous SSc, and 4 with scleroderma in an overlap syndrome). The results were compared with those obtained from 5 patients with Raynaud's disease and from 27 normal controls. Natural and antibody-dependent killing was reduced in the total group of SSc patients compared with controls, but these differences were primarily attributable to patients with the diffuse form of the disease who were seen early in their illness (less than 5 years after onset). NK cell numbers were not significantly reduced in patients compared with controls, although lower numbers were observed in individuals with early diffuse disease. Other clinical parameters, such as treatment with D-penicillamine or the presence of scleroderma-specific autoantibodies, did not exert an independent effect on NK cell function. These findings suggest a possible central role for NK cells in the pathogenesis of SSc.

Adult↗

PubMed2004

[CREST syndrome and pulmonary hypertension: a dark prognosis].

The CREST syndrome initially described as a limited, more indolent form of diffuse scleroderma, is characterized by calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias. Subsequent studies showed that visceral abnormalities were not uncommon. Pulmonary abnormalities are frequent especially pulmonary hypertension, this one being a major cause of this syndrome's mortality. The authors present the case of white woman, 61 years old , with dyspnoea, cyanosis and peripheral edema with 12 months of evolution. She was admitted for pulmonary thromboembolism suspicion. After investigation the diagnosis of CREST syndrome was made, associated with severe pulmonary hypertension. The patient was treated with varfarina, nicorandil, nifedipine, furosemide and home oxygen. She died 3 months after. The authors discuss the diagnosis, treatment and follow-up.

CREST Syndrome↗

PubMed1990

L-tryptophan ingestion associated with eosinophilic fasciitis but not progressive systemic sclerosis.

OBJECTIVE: To assess the use of L-tryptophan by patients with eosinophilic fasciitis and compare this with its use by patients with progressive systemic sclerosis (scleroderma). DESIGN: Retrospective and prospective analysis. Six patients with eosinophilic fasciitis were identified retrospectively and two prospectively. Retrospective identification of patients was done by questioning hospital-affiliated rheumatologists and dermatologists and by searching the hospital dermatopathology database. The patients with scleroderma or morphea were prospectively identified by questioning consecutive office patients with these established diagnoses. SETTING: University of Pennsylvania rheumatology and dermatology practices. PATIENTS: Eight patients with eosinophilic fasciitis; 40 consecutive patients with scleroderma (27 with diffuse cutaneous and 13, limited cutaneous disease); 3 patients with morphea. RESULTS OF DATA ANALYSIS: All eight patients with eosinophilic fasciitis had taken L-tryptophan before the onset of their disease. All had myalgias and high peripheral eosinophil counts (most greater than 5000 cells/mm3). Only 1 of 40 patients with scleroderma (no patients with morphea) had used L-tryptophan preceding illness (P less than 0.001 compared with eosinophilic fasciitis). Six patients with eosinophilic fasciitis had taken L-tryptophan for less than 8 months. One patient had taken it for 9 years before developing skin induration. Two patients were newly identified as having hypothyroidism; two developed neuropathy; and two had severe flexion contractures (several occurring in areas without skin induration). Five patients had low-titer antinuclear antibodies, indicating a possible autoimmune process. Most patients had only a partial response to systemic corticosteroid therapy. One patient has had important disease regression in response to isotretenoin therapy that was evident even while she continued to take L-tryptophan. CONCLUSIONS: L-Tryptophan use can lead to eosinophilic fasciitis whereas it does not appear to cause classic scleroderma. The disease process does not automatically remit after discontinuation of L-tryptophan-containing products.

Adult↗

PubMed2006

Scleroderma in South Australia: further epidemiological observations supporting a stochastic explanation.

The aim of this study was to determine the incidence, prevalence, survival and selective demographic characteristics of scleroderma occurring in South Australia over the 10-year period 1993-2002. Analysis of the database of the South Australian Scleroderma Register: a population-based register established in 1993. Patients with scleroderma resident in South Australia (n = 353 at 2002) were ascertained from multiple sources and clinical and demographic data were obtained from mailed questionnaire and from review of computerized hospital databases, case notes or referring letters. Time-space cluster analysis was carried out according to the Knox method. Control data were obtained from the Australian Bureau of Statistics census. The mean prevalence was 21.4 per 10(5) (95% confidence interval 20.2-22.6) and the mean cumulative incidence of 1.5 per 10(5) (95% confidence interval 1.32-1.73) with no significant change in incidence over the study period (P = 0.13). Cumulative survival improved over the study period, with patients with diffuse disease having significantly reduced survival (as compared with limited disease, P < 0.001). The proportion with diffuse disease ( approximately 22%) remained steady. There was a small but significant predisposition in patients with a continental European birthplace (P < 0.001). A family history of scleroderma was noted in 1.6% with lambda1 (familial risk) of 14.3 (95% confidence interval 5.9-34.5). However, a family history of systemic autoimmunity (especially rheumatoid arthritis) was more common (6%). No socioeconomic stratification, temporal clustering nor spatio-temporal clustering was observed either at time of initial symptom or at 10 years before disease onset. Scleroderma occurs relatively infrequently in South Australia with no significant change in incidence observed over the 10-year study period. However, cumulative survival has improved. Identified risk factors include family history of scleroderma (risk approximately 14-fold), female sex (risk approximately 5-fold) and European birthplace (risk approximately 2.5-fold); however, the majority of the disease variance appears unexplained. A stochastic explanation based on genetic instability is favoured to explain this paradox.

Female↗

PubMed2001

Drug treatment of scleroderma.

Scleroderma or systemic sclerosis is a rare condition with many clinical manifestations including Raynaud's phenomenon. As with many other rarely encountered diseases, drug therapy for scleroderma is often empirical with little evidence in the form of randomised controlled trials to aid drug choice. Raynaud's phenomenon has been recognised for well over 100 years. A considerable number of clinical trials in this area have demonstrated unequivocally the use of nifedipine as a gold standard. Large studies have also demonstrated the efficacy of iloprost. However, this drug is not as yet licensed for scleroderma in the UK or elsewhere. This presents an additional problem as information regarding the use and administration of unlicensed drugs is often sparse and post-marketing surveillance to assess safety is not routinely performed. When looking at the other distinct conditions encountered by a patient with scleroderma it becomes evident that trials are often retrospective or limited in patient numbers. Studies investigating the use of methotrexate, antithymocyte globulin and cyclophosphamide in patients with scleroderma have been very small and in some cases not well designed. The major work on penicillamine was a retrospective trial. Again these drugs are not licensed for use in scleroderma. Drug therapy for pulmonary hypertension secondary to scleroderma closely follows that outlined for primary pulmonary hypertension. In the US there is a patient registry for primary pulmonary hypertension that has enabled well designed, large-scale studies to demonstrate the benefits of epoprostenol in severe primary pulmonary hypertension. Hence, research in this area has progressed considerably over the last decade. Clearly, a considerable amount of work is being carried out to elucidate new treatment regimens for scleroderma, however, evaluation of these studies is proving to be a difficult process. Designated hospital centres for scleroderma (there are currently 2 in the UK), better markers of disease activity and methods to measure improvement or deterioration in affected organs, should enable research into aetiology, disease progression and treatment to be carried out on a larger scale resulting, hopefully, in more conclusive answers.

Gastrointestinal Diseases↗

PubMed2004

Gemcitabine-associated scleroderma-like changes of the lower extremities.

Gemcitabine is a nucleosid analog approved for use in the treatment of metastatic urothelial carcinoma of the bladder. We describe an unusual case of scleroderma-like changes of the lower extremities after treatment by gemcitabine for metastatic carcinoma of the bladder. The patient developed initial inflammatory edema (3 kg) restricted to the lower extremities and subsequent scleroderma-like changes after 2 cycles of gemcitabine. Cutaneous biopsy specimen revealed diffuse sclerosis without involvment of the fascia or muscle. Discontinuation of gemcitabine resulted in dramatic removal of the edema, softening of the skin, and partial reversibility of the fibrotic process. This is the first case report of a scleroderma-like reaction associated with gemcitabine. This antineoplastic agent must be added to the very limited number of cytostatic agents capable of giving rise to scleroderma-like features.

Acetaminophen↗

PubMed1999

[Scleroderma and pregnancy: obstetrical complications and the impact of pregnancy on the course of the disease].

BACKGROUND: To describe the outcome of the pregnancy in patients with scleroderma. PATIENTS AND METHODS: Patients with scleroderma and control group were included in this retrospective study. Two groups were different in pregnant patients with scleroderma: pregnancy before scleroderma (A1) and pregnancy after scleroderma (A2). The presence of clinical problems during pregnancy and the outcome of scleroderma were collected in a questionnaire. Differences in the frequencies of complications were analyzed using the U Mann-Whitney, the chi-square or Fisher's exact test when necessary. RESULTS: The frequency of global fetal complications was increased in patients group, but there was no significantly increased frequency when variables were analyzed independently: number of births, miscarriages, fetal deaths, preterm births and low weight full term babies. There was no increased frequency of renal crisis, hypertension or eclampsia. Differences between diffuse and limited subsets were no observed. Improvement of scleroderma was seen in only 3 patients and worsening of skin thickening was experienced by 2 patients. CONCLUSIONS: The pregnant scleroderma patients are a group with high risk pregnancies and therefore well-supervised pregnancies are necessary.

Adult↗

PubMed2004

Localized scleroderma is an autoimmune disorder.

OBJECTIVES: There have been many studies suggesting that localized scleroderma has a strong autoimmune background, although the lesions are usually limited to the skin and subcutaneous tissue. Here we summarize previous data on the autoimmunity of localized scleroderma, mostly published in the last two decades, because there has not been a review paper summarizing autoimmunity in this disorder. METHODS: We classified the previous reports into three categories: antinuclear antibodies; cytokine and soluble receptors; and cell adhesion molecules and cell surface molecules. In each category, we introduce the important investigations. RESULTS: High frequencies of antinuclear antibodies, detected by the indirect immunofluorescence method using cultured cells, are confirmed by many groups. The major autoantigens have been revealed to be histones. Recently, anti-topoisomerase II alpha antibody has been found to be detected highly frequently in localized scleroderma, while anti-topoisomerase I antibody, which is highly specific for systemic sclerosis, has not been detected in any case of localized scleroderma. In other studies, elevated serum cytokines and cell adhesion molecules suggest the immunoactivation of localized scleroderma. CONCLUSIONS: Many previous studies conclude that localized scleroderma involves autoimmune abnormalities and is one of the organ-specific autoimmune disorders targeting mainly skin, although the types of autoimmune abnormality are different from systemic sclerosis.

Antibodies, Antinuclear↗

PubMed2006

Involvement of alphavbeta5 integrin in the establishment of autocrine TGF-beta signaling in dermal fibroblasts derived from localized scleroderma.

Localized scleroderma (LSc) is a connective tissue disorder limited to skin and subcutaneous tissue, which may share pathogenic processes with systemic sclerosis (SSc). We previously demonstrated that upregulated expression of integrin alphavbeta5 might contribute to autocrine TGF-beta signaling in SSc fibroblasts. Based on these data, we presently focused on alphavbeta5 and assessed its involvement in pathogenesis of LSc. We initially demonstrated that LSc fibroblasts might be activated by the stimulation of autocrine TGF-beta. Consistent with SSc fibroblasts, expression levels of alphavbeta5 were elevated in LSc fibroblasts in vitro and in vivo. Anti-alphavbeta5 antibody partially reversed expression levels of type I procollagen and MMP-1 and constitutive DNA-Smad3 binding in LSc fibroblasts. In LSc fibroblasts pretreated with antisense TGF-beta1, exogenous latent TGF-beta1 stimulation increased expression of type I procollagen in an alphavbeta5-dependent manner. The luciferase activities of TMLC cells, Mv1Lu cells stably expressing a portion of the plasminogen activator inhibitor 1 promoter, co-cultured with LSc fibroblasts were significantly elevated compared with those co-cultured with normal fibroblasts and were significantly reduced in the presence of anti-alphavbeta5 antibody. Anti-alphavbeta5 antibody reversed the myofibroblastic features of LSc fibroblasts. These results indicate that upregulated expression of alphavbeta5 contributes to autocrine TGF-beta signaling in LSc fibroblasts.

Antibodies↗

PubMed2001

The TGF beta receptor endoglin in systemic sclerosis.

Immunohistochemical, flow cytometric and ELISA studies were performed to examine the expression of endoglin (CD105, a TGF beta receptor) on dermal endothelial cells, peripheral blood monocytes and free and bound serum levels in patients with systemic sclerosis as compared with appropriate controls. Endoglin was found to be significantly upregulated on dermal blood vessels in patients with scleroderma (and in patients with inflammatory skin disorders) as compared to healthy skin (p < 0.05). In contrast, there was no significant difference in endoglin expression on circulating blood monocytes between scleroderma patients and patients with a rheumatic disoder or healthy control subjects; however, endoglin expression was upregulated on monocytes in inflammatory joint fluid from patients with rheumatoid arthritis. Endoglin expression on monocytes was also influenced by isolation techniques and during whole blood culture. No differences were found in circulating free or bound endoglin levels between scleroderma patients and healthy controls. In conclusion, endoglin expression on dermal endothelial cells was significantly enhanced in scleroderma but levels on circulating monocytes and in the serum were within normal limits. The functional significance of this upregulation is uncertain but may reflect endothelial activation in scleroderma.

Aged↗

PubMed2003

A cross-sectional comparison of three self-reported functional indices in scleroderma.

OBJECTIVES: In scleroderma, outcome measures such as skin score provide only limited information about the functional impact of the disease. The requirement for validated and convenient instruments that reliably reflect disease morbidity is now recognized. This study compares the Disability Index of the Health Assessment Questionnaire (HAQ-DI) with two more recently developed scleroderma-specific tools: scleroderma-visual analogue scales (scleroderma-VAS) and the UK scleroderma Functional Score (UKFS). In addition, the use of clinical and laboratory measures as predictors of disease severity have been examined. METHODS: One hundred and fifteen consecutive patients were studied. Subjects completed the 20-item HAQ-DI, the scleroderma-VAS and a questionnaire related to hand and muscle function (UKFS). Clinical details, measurement of maximal hand-spread, fist-closure and investigations for internal organ involvement were recorded. RESULTS: Over 68% of patients with diffuse disease had moderate to severe disease on the UKFS, compared with 44% with limited disease. The mean UKFS in diffuse disease was 14.7 (s.d. 9.1) and 10.6 (s.d. 8.5) in the limited subset (P=0.02). The mean HAQ-DI in diffuse disease was 1.23 (s.d. 0.77) and 0.79 (s.d. 0.75) in the limited subset (P=0.005). The HAQ-DI showed significant correlation with UKFS (r=0.9; P < 0.001). Several clinical and laboratory measures were associated with higher HAQ-DI and UKFS. CONCLUSIONS: This is the first comparative study of the UKFS and the HAQ-DI. These data show a strong correlation between assessment methods. Higher scores correlated with clinical and laboratory indicators of severe disease. Used together, these inexpensive tools assess general and organ-specific symptoms, as well as functional limitation.

Adult↗

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↗

PubMed2002

Recurrent bronchiolitis obliterans organizing pneumonia in a patient with limited cutaneous systemic sclerosis.

Systemic sclerosis (SSc) is a generalized disorder characterized by fibrosis and vascular obliteration in the skin, lung, gastrointestinal tract, and kidney. One of its two subsets is a stable, limited cutaneous group (lSSc). Pulmonary involvement in scleroderma is common, and several types of pulmonary disorders are associated with SSc. Bronchiolitis obliterans organizing pneumonia (BOOP) is a rare finding in lung disorders associated with SSc. We describe a case of lSSc with BOOP that was responsive to steroid therapy. Of interest is that the lung disorders appeared in different periods and areas. It might be important to diagnose abnormal shadows in lung fields before treatment of patients with SSc.

Aged↗

PubMed1999

Thrombotic thrombocytopenic purpura as an initial presentation of limited systemic sclerosis.

Thrombotic thrombocytopenic purpura (TTP) is a rare complication of scleroderma (systemic sclerosis, SSc). In the 5 reports documenting the association of TTP and SSc, the TTP syndrome developed on a background of well established SSc. We describe a 51-year-old woman with a 5 month history of an evolving connective tissue disease syndrome who presented initially with TTP, followed 4 months later by limited cutaneous SSc and Raynaud's phenomenon.

Brain↗

PubMed2003

Carotid and femoral arterial wall mechanics in scleroderma.

OBJECTIVE: Large-vessel arterial disease is increasingly recognized as a major cause of morbidity in autoimmune rheumatic disorders. Recent evidence suggests that scleroderma (systemic sclerosis, SSc) may be linked to altered fibrillin-1 metabolism associated with a defect in chromosome 15q. If this is the case, we may expect to see changes in the arterial wall mechanics of large vessels not clinically involved in the disease process. We undertook a study to determine whether the biomechanical properties and intima-media thickness (IMT) of the elastic carotid artery and the muscular femoral artery are altered in subjects with limited (lcSSc) and diffuse (dcSSc) cutaneous SSc. METHODS: Measurements of carotid and femoral wall mechanics were made in 33 patients with lcSSc, 19 patients with dcSSc and 21 control subjects, using a duplex scanner coupled to a Wall Track system. Their age, gender, body mass index, heart rate, systolic and diastolic blood pressures, presumed cardiovascular load, and plasma creatinine, fasting cholesterol, triglyceride and glucose concentrations were also measured. RESULTS: There was a progressive and significant reduction (P < 0.001) in the elastic properties of the carotid artery from the control group (compliance, 16.24 +/- 4.39 %mmHg(-1) x 10(-2)) to the lcSSc group (10.89 +/- 2.43 %mmHg(-1) x 10(-2)) to the dcSSc group (7.65 +/- 2.08 %mmHg(-1) x 10(-2)), even after adjustment for the systemic physiological and biochemical variables studied, which are known to influence the mechanics of arterial walls. There was no apparent difference between the groups in the mean elastic indices of the femoral artery and the IMT of the carotid and femoral arteries. CONCLUSION: The elastic properties of the carotid artery are significantly altered in SSc, and the two major subsets of SSc may be distinguished by their carotid artery biomechanics. This suggests that connective tissue abnormality occurs at sites not previously assessed.

Carotid Arteries↗

PubMed2004

Parvoviral infection of endothelial cells and stromal fibroblasts: a possible pathogenetic role in scleroderma.

BACKGROUND: Systemic sclerosis (SSc) is a connective tissue disease (CTD) which differs from other CTDs by progressive irreversible fibrosis in lung, kidney, skin, and heart. It has a worse prognosis compared to several other CTDs. The pathogenesis may reflect a humorally mediated microangiopathy in concert with the overproduction of collagen triggered by immune-mediated cytokine production. Having previously demonstrated parvovirus B19 (B19) DNA in bone marrow and skin biopsies of SSc patients in the absence of B19 viremia, we sought to further elucidate a role for B19 in the pathogenesis of SSc. DESIGN: Twelve patients who fulfilled American College of Rheumatology criteria for a diagnosis of SSc were encountered. Ten were serologically screened for B19 infection. Solution phase polymerase chain reaction (PCR) for B19 DNA was performed on skin tissue from six patients, and in all biopsies, reverse transcriptase in situ PCR (RT in situ PCR) for B19 and tumor necrosis factor (TNF)-alpha mRNA was performed. B19 viral protein (VP2) expression was sought by immunohistochemistry and correlated to PCR findings and to light microscopy of hematoxylin and eosin-stained sections. Frozen tissue was also available on five of the patients. Two control groups were assessed for B19 and TNF expression comprising one with irrelevant primers and the other representing 18 cases of inflammatory skin lesions where the etiology was known and unrelated to B19 infection. In addition, frozen and paraffin-embedded tissues procured from skin lesions unrelated to B19 infection were assessed for B19 genome. In all cases, pretreatment with RNase was also performed to verify that any positive signal was indeed RNA based. RESULTS: Diffuse SSc was seen in seven patients, and limited disease in five. All patients had an antinuclear antibody--specifically, an antinucleolar, anticentromere, and/or anti-Scl 70 antibody. Eleven of the 12 had lung involvement, whereas eight patients had myocardial disease. Of 12 patients tested serologically, nine had B19-specific antibodies, which included immunoglobulin M (IgM)-specific antibodies in two cases. Solution phase PCR showed B19 DNA in the skin in three cases and in the bone marrow in three cases, including two in whom skin-based B19 DNA was observed. In all cases, RT in situ PCR demonstrated B19 and TNF-alpha mRNA in endothelia, fibroblasts, mast cells, and perivascular inflammatory cells. Immunohistochemistry to assess VP2 was either negative or equivocal. Immunofluorescent studies revealed prominent deposition of C5b-9 within the cutaneous vasculature from biopsies of all patients tested. The control samples were negative for B19 and TNF RNA and DNA. CONCLUSIONS: Parasitism of endothelia and fibroblasts by B19 with resultant enhanced TNF-alpha expression may be of pathogenetic importance in SSc even in the absence of demonstrable viremia. The vascular deposition of C5b-9 suggests a role for humoral immunity possibly induced by a state of endothelial neoantigenicity evoked by virally mediated cell injury. Treatment strategies include anti-viral therapy, including in the context of intravenous gamma-globulin and anti-TNF therapy.

Adult↗

PubMed2004

[Quality of life assessment with the MOS-SF36 in patients with systemic sclerosis].

OBJECTIVE: To investigate the metric properties and the validity of the Medical Outcome Study Short Form 36 (SF-36), a questionnaire to assess the quality of life, in patients with either diffuse or limited systemic sclerosis (SS), and to examine the effect of the disease on quality of life. METHODS: Cross sectional study of 86 patients with a SS (64 diffuse SS, 22 limited SS). Disease severity was assessed by clinical examination, pulmonary functional tests and Health Assessment Questionnaire (HAQ) modified for scleroderma. RESULTS: The SF-36 scores values were lower in diffuse than in limited sclérodermie systémique. The Physical Component Score was worse in patients with than without any clinical involvement. This score increased in relation with the number of clinical involvements. The quality of life of patients with SS was correlated to its functional repercussion. CONCLUSION: The quality of life in SS patients is correlated with the clinical severity of the disease. The use of SF-36 to measure the quality of life is useful for the clinical evaluation of patients with SS.

Adolescent↗