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PubMed1991

Overlap syndrome of progressive systemic sclerosis and polymyositis: report of 40 cases.

Forty cases of overlap syndrome of progressive systemic sclerosis and polymyositis (OS PSS-PM) are reported in this paper. All of these cases had manifestations of both PSS and PM as well as Raynaud's phenomenon. The sclerodermatous skin changes were diffused over the whole body in most cases. All cases had muscular weakness, elevated skeletal muscle enzyme levels and muscle damage as seen on the electromyogram. Histopathologic changes showed characteristics of myositis. There was noticeable systemic involvement, especially with the digestive and circulatory systems. Serologic examination frequently revealed autoantibodies. The patients responded well to traditional Chinese medicines and corticosteroids.

Adolescent↗
PubMed1983

[Anti-collagen antibodies. Their detection in rheumatoid polyarthritis, chronic atrophic polychondritis and various chronic inflammatory rheumatism].

The authors administered by passive haemagglutination, natural or denatured type I and II anticollagen antibodies in the serum of 83 rheumatoid polyarthritis, 20 ankylosing spondylarthritis, 30 relapsing polychondritis, 13 diffuse sclerodermics, 12 Gougerot-Sjögren syndrome patients. Type II natural anti-collagen antibodies are common in relapsing polychondritis (37 percent). They are rarer in rheumatoid polyarthritis (15 percent) and the frequency is identical to that found in patients suffering from gonarthrosis and coxarthrosis. Natural type II anti-collagen antibodies may be occur in etiopathogenesis of relapsing polychondritis. Denatured II ant-collagen antibodies are common during ankylosing spondylarthritis (35 percent) and diffuse sclerodermia, and are found in rheumatoid polyarthritis (23 percent) and in Gougerot Sjögren syndrome (25 percent). They possibly play a pathological role in these diseases although this has not been proved. They are detected almost as often in the synovial fluid of inflammatory rheumatisms (53 percent) and in gonarthrosis (41 percent).

Adult↗
PubMed2004

Associations of right ventricular myocardial function with skin and pulmonary involvement in asymptomatic patients with systemic sclerosis.

BACKGROUND: Systemic sclerosis (SSc) is a multisystem disorder characterized by widespread vascular lesions and fibrosis of the skin and specific internal organs. Cardiac involvement is a common finding in SSc, but often clinically occult. The aim of the present study was to analyze both left and right ventricular (RV) myocardial function in patients with SSc, and their relation to other instrumental features of the disease. METHODS: Twenty-five healthy subjects and 23 age- and sex-comparable asymptomatic patients classified as having either diffuse (11 patients) or limited cutaneous (12 patients) SSc underwent clinical examination, serological analysis, high-resolution chest computed tomography, standard Doppler echocardiography and pulsed Doppler myocardial imaging (DMI) of both the mitral and tricuspid annuli. SSc was classified using the modified Rodnan skin score (mRSS) into high mRSS (score > or = 10) and low mRSS (score < 10). RESULTS: Serological antibody analysis revealed the presence of antinuclear antibody in all patients, an anticentromere pattern in 8 patients, and anti-Scl-70 antibodies in 15 patients. Eleven patients were diagnosed with interstitial pulmonary fibrosis at chest computed tomography. Standard Doppler echocardiography revealed that the left ventricular mass index and ejection fraction were comparable between the two groups, while the RV end-diastolic diameter was increased in SSc (p < 0.01). The tricuspid inflow peak E and E/A ratio were slightly decreased in SSc (p < 0.01), while the systolic pulmonary pressure was increased (p < 0.0001). DMI analysis revealed, in SSc, an impaired RV myocardial early-diastolic (Em) peak velocity (p < 0.001) as well as a prolonged myocardial relaxation time (RTm) (p < 0.001) only at the tricuspid annulus level, even after correction for age, sex, heart rate and left ventricular mass index. Independent inverse associations of the RV Em peak velocity with both the Rodnan skin score (beta coefficient = -0.62, p < 0.0005) and the pulmonary systolic pressure (beta coefficient = 0.71, p < 0.0001), as well as the independent inverse correlation of the same RV Em peak velocity with interstitial pulmonary fibrosis (odds ratio 0.68, 95% confidence interval 0.45-0.83, p < 0.0005) in SSc patients were assessed at multivariate analysis. In addition, the RV Em velocity was an independent predictor of the anti-Scl-70 antibody pattern (odds ratio 0.68, 95% confidence interval 0.45-0.83, p < 0.01). Of note, a RV Em peak velocity < 0.11 m/s well selected SSc patients with pulmonary artery pressure > 35 mmHg, pulmonary fibrosis, a high mRSS, and an anti-Scl-70 antibody pattern. CONCLUSIONS: The relationships of RV myocardial diastolic dysfunction with both skin and pulmonary involvement as well as with the serological antibody pattern emphasizes the ability of DMI to identify patients with a more diffuse and severe form of SSc. This issue may be critical for the early identification of those SSc patients who are at higher risk of cardiac impairment, ideally when they are still asymptomatic before developing severe vasculopathy.

Case-Control Studies↗
PubMed2001

Pulmonary function tests, high-resolution computerized tomography, alpha1-antitrypsin measurement, and early detection of pulmonary involvement in patients with systemic sclerosis.

OBJECTIVE: Pulmonary disease represents a major complication of systemic sclerosis (SSc). However, pulmonary involvement is commonly silent. In this study, we investigated the relationship between serum alpha1-antitrypsin and other means of assessing pulmonary involvement. METHODS: Twenty-two patients affected by SSc were studied (mean age 37.6+/-14.3 years, mean duration of disease 9.9+/-11.9 years). Fourteen had the diffuse form of disease (dSSc) and eight had the limited form (lSSc). All patients underwent pulmonary function tests, high-resolution computed tomography (HRCT) of the lungs, echocardiography, and serum assessment of alpha1-antitrypsin. RESULTS: Mean percentage of predicted values of forced vital capacity was lower in patients with dSSc than with lSSc (72.3+/-17.8 vs 74.5+/-8, P=NS). Mean percentage of predicted values of forced expiratory volume in 1-s forced vital capacity (FEV1/FVC) was lower in patients with lSSc (79.8+/-7.5 for lSSc vs 84.4+/-7.8 for dSSc, P= NS). The overall HRCT score was 5.6+/-5.9 with no significant difference between disease subgroups. Pulmonary hypertension was detected in two cases, both with dSSc. Alpha1-antitrypsin was significantly higher in patients than in controls (P < 0.01), with no significant difference between disease subgroups, and correlated significantly with ground glass opacities in H RCT (P < 0.05) and the detection of diffusion defects (r= -0.61, P<0.01). No significant correlation was observed between skin score or degree of dyspnea with HRCT score, lung volume, or carbon monoxide diffusing capacity. CONCLUSION: Restrictive lung disease was more pronounced in patients with dSSc. Alpha1-antitrypsin levels correlated significantly with ground glass opacities, an early finding of pulmonary involvement in SSc. Extent and severity of skin involvement and degree of dyspnea were not related to pulmonary involvement.

Adolescent↗
PubMed1993

Autoantigenic epitopes on DNA topoisomerase I. Clinical and immunogenetic associations in systemic sclerosis.

OBJECTIVE: To elucidate the clinical and immunogenetic associations with reactivity to autoantigenic epitopes on DNA topoisomerase I (topo I) recognized by sera from patients with systemic sclerosis (SSc). METHODS: Autoantigenic epitopes on topo I were identified by screening an epitope library constructed from topo I complementary DNA restriction fragments using autoimmune anti-topo I-positive sera as a probe. Epitope reactivities of sera from 43 anti-topo I-positive SSc patients were surveyed by immunoblotting, and associations with clinical symptoms and HLA-DR types were examined. RESULTS: Four different epitope regions were identified on the topo I molecule. Immunoreactivity to the region encompassing amino acid residues 658-700, termed ER4, was found to be associated with diffuse cutaneous SSc, progressive pulmonary interstitial fibrosis, and poor prognosis for 15-year survival. SSc patients with ER4 reactivity frequently displayed the DR2/DRw52 phenotype. CONCLUSION: Molecular analysis of precise antigenic epitopes on topo I is helpful in classifying clinical subsets of SSc.

Adult↗
PubMed2005

A randomized unblinded trial of cyclophosphamide versus azathioprine in the treatment of systemic sclerosis.

Current therapeutic modalities for the treatment of systemic sclerosis (SSc) have significant limitations. The window of opportunity to prevent tissue fibrosis and irreversible damage occurs during the early inflammatory phase of this condition. A drug that we believe has promise to exert a desired effect in early SSc is cyclophosphamide (CYC). However, there are only a few published reports regarding the use of cytotoxic immunosuppressive medications at the onset of the illness. The goal of this study was to test the efficacy and toxicity of CYC in patients with early diffuse SSc. The study design was a randomized, unblinded, 18 months per patient trial with a comparison group that received azathioprine (AZ). Thirty patients were assigned to receive oral CYC (2 mg/kg daily for 12 months and then maintained on 1 mg/kg daily) and 30 patients were assigned to receive oral AZ (2.5 mg/kg daily for 12 months and then maintained on 2 mg/kg daily). During first 6 months of the trial, the patients also received prednisolone, which was started at a dosage of 15 mg daily and tapered to zero by the end of the sixth month. After treatment there was a statistically significant improvement in the modified Rodnan skin score (MRSS), attack frequency of Raynaud's phenomenon (RP), and erythrocyte sedimentation rate (ESR) in the CYC-group, but not in the AZ-group. The forced vital capacity (FVC) and carbon monoxide diffusing capacity (DLCO) did not change after treatment in the CYC-group, but statistically significantly worsened in the AZ-group. No life-threatening or irreversible adverse reactions were observed in either group. This study showed that CYC is a promising disease modifying medication for SSc as it exhibited a positive influence on the evolution of disease.

Administration, Oral↗
PubMed2001

Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival.

OBJECTIVE: To assess the rate of progression of pulmonary hypertension (PHT) in systemic sclerosis (SSc) and its bearing on mortality. METHODS: A retrospective record review of 930 patients with SSc attending a specialist centre was carried out. Those at risk for both idiopathic and secondary PHT were assessed by serial Doppler echocardiography. Mortality data were reviewed. RESULTS: The cumulative prevalence of PHT was 13%. Pressures remained static in most cases. The mortality among those with a single pressure reading of 30 mmHg or higher was 20% at 20 months. An increased mortality risk was associated with high initial pressures and rising pressures. Rapid pressure rises occurred more frequently in limited than in diffuse SSC. CONCLUSIONS: The prevalence of PHT in SSc is high and the detection of PHT at any time in the disease course is associated with substantial mortality. These results demonstrate the value of echocardiographic screening for PHT in all patients with SSC.

Adult↗
PubMedDate not supplied

Familial occurrence of axillary papular mucinosis.

Primary cutaneous mucinosis encompasses a wide range of different skin conditions with circumscribed, follicular or diffuse manifestation. For the first time, to our knowledge, we report on the occurrence of so-called "atypical" localized papular mucinosis in the axillary pits of a mother and her (identical) twin daughters presenting as asymptomatic soft minuscule papules. This unique observation allows us to define an autosomal dominant inheritance pattern with a high level of penetrance for this rare skin disease.

Adolescent↗
PubMed1993

[Pulmonary hypertension and systemic sclerosis].

BACKGROUND: Pulmonary involvement in systemic sclerosis (SS) is a frequent complication and is associated with a poor prognosis. Pulmonary hypertension may or may not develop, however, its recognition is usually possible only in advanced stages. METHODS: In this study we noninvasively evaluated the pulmonary artery systolic pressure in 31 patients with SS using Doppler echocardiography. Pulmonary hypertension was detected in 48.4% of the patients. RESULTS: The prevalence of pulmonary hypertension was similar in patients with limited SS and diffuse SS (42.9% and 52.9%, respectively; p = NS). No differences were observed in pulmonary artery systolic pressure between patients with limited or diffuse SS and pulmonary hypertension. Pulmonary hypertension was usually mild, and only in two cases was pulmonary systolic pressure higher than 50 mmHg (63 and 107 mmHg, respectively). CONCLUSIONS: Pulmonary hypertension is frequently observed in patients with SS. The patients with diffuse or limited SS are equally affected by this complication. Doppler echocardiography has proved to be the technique of choice for the evaluation of pulmonary involvement in patients with SS because it is noninvasive, inexpensive and allows serial examinations. Early recognition of pulmonary hypertension may favor the use of therapeutical strategies to prevent the progression to advanced forms.

Adult↗
PubMed2004

Oxygen free radical scavenger enzyme polymorphisms in systemic sclerosis.

We performed a case-control study of polymorphisms of glutathione S-transferase (GST) isoenzymes and manganese superoxide dismutase (MnSOD) in black South Africans with systemic sclerosis (SSc). The frequency of the GSTM1*B phenotype was significantly decreased in the overall SSc group compared with controls (OR=0.19, p(corr)<.05), implying a possible protective effect against development of the disease. There was also a trend toward increased MnSODAla allele and phenotype frequencies in the diffuse cutaneous SSc subset compared with controls (OR=2.11 and 3.15, respectively, p(corr)<.1). Our findings provide new data on the distribution of GST and MnSOD polymorphisms in healthy Africans and further evidence that genetic factors may have a contributory role to play in predisposing to oxidative stress in SSc.

Alleles↗
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↗
PubMed2006

Radiological hand involvement in systemic sclerosis.

BACKGROUND: The osteoarticular and soft tissue structures of the hand may be involved in systemic sclerosis (SSc), causing functional disability. OBJECTIVE: To assess radiological hand features in a cross sectional study of SSc patients and in controls. METHODS: Hand radiology was done systematically in patients with SSc seen over a two year period and in unselected controls with rheumatoid arthritis or digital trauma. Two independent investigators blind to the diagnosis carried out the radiological assessment. RESULTS: 120 consecutive SSc patients (median (range) age, 56.5 (20 to 90) years; disease duration, 6 (0 to 42) years) and 42 controls (22 with rheumatoid arthritis and 20 with digital trauma) were studied. Radiological abnormalities in SSc patients included erosion (21%), joint space narrowing (28%), arthritis (defined by concomitant erosion and joint space narrowing) (18%), radiological demineralisation (23%), acro-osteolysis (22%), flexion contracture (27%), and calcinosis (23%). In univariate and multivariate analysis, the resorption of distal phalanges was significantly associated with digital ulcers, extra-articular calcification, and pulmonary arterial hypertension; flexion contracture was associated with the diffuse cutaneous form and high HAQ (Health Assessment Questionnaire) disability score. Calcinosis was most often seen in patients with digital ulcers, but was similarly observed in patients with the diffuse or limited cutaneous subtypes. CONCLUSIONS: Flexion contracture was associated with disability and occurred in patients with the diffuse cutaneous subtype of SSc, consistent with the tendency towards fibrosis and functional impairment of this subtype. Calcinosis and acro-osteolysis were both associated with vascular complications, highlighting a potential role of vascular injury in such lesions.

Adult↗
PubMed2002

Correlation between the numbers of gammadelta T cells and CD4+ HLA-DR+ T cells in broncho-alveolar lavage fluid from patients with diffuse lung disease.

CD4+ HLA-DR+ T cells are known to be increasing in broncho-alveolar lavage fluid (BALF) from patients with sarcoidosis, and related to disease activity. Although there are several reports that the number of gammadelta T cells in peripheral blood from patients with sarcoidosis are increasing, contradictory assertions can be seen about the number of gammadelta T cells in BALF, and the clinical significance on the presence of gammadelta T cells in disease site of patients with diffuse lung disease including sarcoidosis. The absolute number of gammadelta T cells and CD4+ HLA-DR+ T cells in BALF were determined by flow cytometry in 107 patients with diffuse lung diseases; 56 with sarcoidosis, 36 with collagen vascular diseases with lung involvement and 15 with idiopathic pulmonary fibrosis. We also measured the number of the transferrin receptor-positive macrophages in BALF. The correlation between gammadelta T cells and activated (maybe antigen-specific) T cells and macrophages were evaluated. Sarcoidosis patients were also evaluated from the data of the number of gammadelta T cells in peripheral blood by flow cytometry and clinical backgrounds. A significant correlation between the numbers of these two cell types was detected in each of the three patient groups. The percentage of peripheral gammadelta T cells was markedly increased in 7 sarcoidosis patients, each of whom also showed affected organs other than lung, however, 5 individuals did not show an increased number of gammadelta T cells in BALF. The number of gammadelta T cells in BALF did not correlate with the number of transferrin receptor-positive macrophages in all three patient groups. These results suggest that the increased number of gammadelta T cells in diffuse lung diseases likely plays a role in immunosurveillance and contributes to the activation of antigen-specific alphabeta T cell.

Adult↗
PubMed1976

[Screening of collagen diseases in the years 1961-1972 from a selected population sample].

In our article is referred to the recognition of collagenosis in the demarcated whole district with 100,000 inhabitants. The occurrence of these diseases is lower than the occurrence of other rheumatic diseases. Our results as well as the observations of other authors confirm that the lupus erythematodes and the diffuse sclerodermia are not rare diseases even in our circumstances and with their evolution tendency they shorten the lives of the affected persons. The high recognition coefficient with large probability corresponds to the real incidence of these two diseases, which can be achieved only by an observation of many years and repeated controls of the correctness of the diagnosis.

Adolescent↗
PubMedDate not supplied

Clinical determinants of bone mass and bone ultrasonometry in patients with systemic sclerosis.

OBJECTIVE: The aim of this study was to evaluate bone mass and bone ultrasonometry in patients affected with systemic sclerosis (SSc). METHODS: Fifty-five patients (mean age 54.1 +/- 14.1 years; 25 premenopausal, and 30 postmenopausal women) affected with SSc (in a limited, intermediate or diffused form) and 60 age-matched healthy controls (30 premenopausal, and 30 postmenopausal women) were studied for Bone Mineral Density (BMD) measured by fan-beam x-ray densitometry, Stiffness Index (SI) measured by ultrasonometry of the heel, inflammation indices (erithrocyte sedimentation rate, C-reactive protein), and autoantibodies (ANA, ENA). Examinations were also carried out in order to determine any internal organ involvement. None of the patients had previously received steroid treatment. RESULTS: BMD was significantly lower in the SSc group than in the control group, whether it was expressed in g/cm2 (lumbar spine: 0.980 vs 1.241, p < 0.01; femoral neck: 0.832 vs 0.955, p < 0.05; total body 1.050 vs 1.168, p < 0.01) or by T- and Z-score (lumbar spine: T = -2.48; Z = -1.10; femoral neck: T = -1.69; Z = -0.55; total body: T = -1.11; Z = -0.48). SI was also altered (75.8 vs 96.2, p < 0.01; T = -2.10, Z = -1.12). BMD and SI were lower in women with the diffuse form of skin involvement. BMD and SI were lower in women in whom one or more internal organs were involved. CONCLUSION: SSc patients had reduced BMD and SI that was more marked in the diffuse form and in those with internal organ involvement and that became more marked with age and estrogen deficiency. This demineralisation was not related to the inflammation indices, disease duration, or to the immunological pattern.

Absorptiometry, Photon↗
PubMed2000

Induction of immune tolerance to human type I collagen in patients with systemic sclerosis by oral administration of bovine type I collagen.

OBJECTIVE: To determine whether oral tolerance to type I collagen (CI) could be induced in patients with systemic sclerosis (SSc). METHODS: Twenty adult patients with limited or diffuse SSc were enrolled in a study to receive 0.1 mg of solubilized native bovine CI daily for 1 month, followed by 0.5 mg daily for 11 months. Peripheral blood mononuclear cells (PBMC) were obtained from the patients and cultured with human alpha1(I) and alpha2(I) chains, before and after CI treatment. Culture supernatants were analyzed for levels of interferon-gamma (IFNgamma) and interleukin-10 (IL-10). Sera obtained before and after treatment were analyzed for levels of soluble IL-2 receptor (sIL-2R). Although this study was not intended to assess the clinical efficacy of oral CI administration in SSc, selected measures of disease severity and organ involvement were evaluated. RESULTS: Oral administration of CI to SSc patients induced significant reductions in levels of IFNgamma and IL-10 in alpha1(I)- and alpha2(I)-stimulated PBMC culture supernatants, indicating that T cell immunity to CI was decreased by this treatment. Serum levels of sIL-2R also decreased significantly after oral CI treatment, suggesting a reduction in T cell activation. Significant improvements occurred in the modified Rodnan skin thickness score and the modified Health Assessment Questionnaire after 12 months of oral CI in this open trial. The lung carbon monoxide diffusing capacity improved statistically and showed a trend toward clinically significant improvement. CONCLUSION: Oral administration of bovine CI to patients with diffuse or limited SSc induces a reduction in T cell reactivity to human CI, appears to be well tolerated, and does not worsen the disease. Further evaluation of oral tolerance to CI in patients with SSc is justified to determine whether it has therapeutic efficacy.

Administration, Oral↗
PubMed1997

Survival prognostic factors and markers of morbidity in Spanish patients with systemic sclerosis.

OBJECTIVE: To identify survival prognostic factors and markers of morbidity among patients with systemic sclerosis (SSc). PATIENTS AND METHODS: The study included 72 patients diagnosed with SSc. According to the extent of skin involvement, three groups of patients were established: group 1, without sclerosis and with sclerosis of fingers and neck; group 2, with sclerosis of face and distal to elbows and knees; group 3, with generalised sclerosis including the trunk. All patients were included in a study protocol to determine visceral involvement. Cumulative survival after first symptom has been estimated according to the Kaplan-Meier method. The association between a hypothetical prognostic factor and cumulative survival after first symptom was assessed by log rank test. The association between a hypothetical risk factor and the prevalence of severe morbidity was assessed by the odds ratio. Multiple logistic regression models were used to identify the main predictors of severe morbidity. RESULTS: Survival was estimated to be 85% 10 years after first SSc symptom. Survival was higher among SSc patients with skin involvement distal to elbows and knees than among the rest of patients; a forced vital capacity (FVC) on spirometry lower than 70% of expected value was associated with a shorter survival, even after adjustment for diffuse SSc. Skin involvement proximal to elbows or knees was associated with a higher prevalence of severe morbidity (OR = 46.57; p < 0.001). According to a multiple logistic regression, severe morbidity was higher among patients with skin involvement proximal to knees or elbows (OR = 40.92; p < 0.001) or among patients with pulmonary hypertension detected by Doppler echocardiography (OR = 23.66 p < 0.001). CONCLUSIONS: In patients with SSc the extent of skin sclerosis was found to be a determining factor on the prognosis. According to skin sclerosis extent two main subsets of SSc patients with different survival incidence and degree of morbidity could be clearly established: limited SSc, formed by patients with no skin sclerosis or with sclerosis distal to elbows and knees and diffuse SSc, formed by patients with skin sclerosis distal and proximal to elbows and knees. Moreover, lung involvement (FVC < 70% on survival study and pulmonary hypertension on morbidity study) was an important and independent prognostic factor.

Adolescent↗
PubMed2005

Clinical correlations of potential activity markers in systemic sclerosis.

The objectives of the project were the following: (1) to establish a group of patients with a confirmed diagnosis of systemic sclerosis (Ssc), (2) to perform a detailed entrance examination of each patient, (3) to determine concentrations of potential activity markers, and (4) to make a comprehensive examination of each patient 1 year after inclusion into the study. A total of 49 patients were examined, 36 with a limited form of SSc, 9 with diffuse SSc, and 4 with other forms of SSc. We determined plasma or serum levels of the N-terminal propeptide of procollagen type III (NPIIIP), interleukin-6 (IL-6), soluble receptor for interleukin-2 (sIL-2r), soluble intercellular adhesion molecule-1 (sICAM-1), soluble vascular adhesion molecule-1 (sVCAM-1), von Willebrand factor antigen (vWFAg), and big endothelin-1 (BET-1) using commercial kits, and urinary excretion of pyridinoline (PYR) and deoxypyridinoline (D-PYR) using high-performance liquid chromatography. Correlations of these markers with selected clinical data were calculated. The mean levels of all potential activity markers were increased compared with normal values, but differences were not significant. The levels of NPIIIP, D-PYR, and IL-6 were normal. The measured values after 1 year did not differ from the entry values. At entry, NPIIIP concentrations correlated with the finger-to-palm distance, and D-PYR corresponded with findings on a simplified health assessment questionnaire (FQ). IL-6 levels correlated with the leukocyte count, sIL-2r with the FQ, and ET-1 with the diffuse lung capacity for carbon monoxide. In general, we found only a few clinical correlates of potential activity markers. Our data confirmed the correlations of collagen metabolism markers with skin involvement and FQ, as was reported previously. Larger studies in this field are needed.

Adult↗