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PubMed2004

[The methods for screening pulmonary hypertension related to systemic sclerosis in France. Descriptive survey of the French Research Group on Sclerosis].

CONTEXT AND OBJECTIVES: Pulmonary hypertension (PHT) represents one of the severest complications and is life-threatening for patients suffering from systemic sclerosis (SSc). In France, the modalities for screening and treating PHT related to SSc are not well codified and no consensus has been reached. We conducted a survey among physicians inscribed on the list of the French Research Group on Sclerosis (GRFS - Groupe de Recherche Francais sur la Sclerodermie) to gather information on the status of the management of PHT related to SSc. METHODS: In 2002, we sent a questionnaire to 160 physicians, members of the GRFS, to assess the epidemiology and clinical profile of SSc patients as well as the modalities of screening and management of PHT in these patients. RESULTS: Eighty-eight physicians in 71 centres replied to the questionnaire. Each centre followed-up a mean of 33 SSc patients, with a global distribution of 53% limited and 47% diffused SSc. These physicians saw a mean of 5 new cases of SSc per year. The patients had been referred by town practitioners (53%) or from the hospital (47%). The mean number of SSc patients with PHT was of 5.1 per physician (1.5 new SSc + PHT patients per year). Almost all the centres (65/67) who replied systematically screened for PHT in SSc patients using Doppler echocardiography a mean of every 1.3 years. For the management of the patients exhibiting PHT, the majority (41/63) of centres collaborated with a specialized unit. Around one third of the centres treated these patients with calcium channel inhibitors (82%) and/or prostacyclin (90%). All the patients were followed-up by Doppler echocardiography. The majority of the physicians (72%) were interested in a research protocol on the subject and each could have included 4 patients, i.e., a total of 160. CONCLUSION: Pulmonary hypertension, a severe complication of SSc is screened for by the physicians of the GRFS using echocardiography with a frequency similar to Who guidelines (1.3 versus once/year).

Diagnosis, Differential↗
PubMedDate not supplied

[Autologous stem cell transplantation in a patient with diffuse systemic sclerosis].

Systemic Sclerosis (SSc) is a systemic disease of unknown etiology presenting with disseminated skin thickening and fibrotic impairment of various organs including lung and kidney. According to the rate and degree of skin involvement, SSc can be classified in a limited and a diffuse form, the latter showing a severe and progressive lung involvement, which is responsible for its high related morbidity and mortality along with resistance to standard therapeutic protocols. High dose chemotherapy, followed by autologous stem cell transplantation, is a standard therapeutic regimen for haematological diseases: re-infusion of mobilised peripheral blood progenitor cells overcomes the myeloablative effect of super-maximal eradicative doses of chemotherapeutic agents. Recently, this therapeutic approach has been applied in some cases of resistant SSc and, albeit the low number of cases, it has been proven effective in early diagnosed and rapidly progressive forms of the disease showing a clinical improvement and an instrumentally detectable decrease of fibrosis extent. We report the case of a young woman affected by diffuse SSc with a rapid progression of clinical signs and instrumentally detectable lesions who underwent a conditioning regimen with fludarabine, cyclophosphamide and anti-thymoglobulines followed by re-infusion of autologous peripheral blood stem cells. Two years after transplantation a clinical and instrumental evidence of treatment was observed, with good control of disease evolution. The only sign of disease resumption was a slow worsening of skin involvement.

Adult↗
PubMed1998

Serum levels of interleukin 6 (IL-6), oncostatin M, soluble IL-6 receptor, and soluble gp130 in patients with systemic sclerosis.

OBJECTIVE: To determine whether serum interleukin 6 (IL-6), oncostatin M (OSM), soluble IL-6 receptor (sIL-6R), and soluble gp130 (sgp130) levels in patients with systemic sclerosis (SSc) are elevated and whether they are correlated with the clinical or serological features of the disease. METHODS: Serum samples from patients with SSc (n = 55) and control subjects (n = 20) were investigated by ELISA. Patients were divided into 4 groups: 12 with limited cutaneous SSc (lSSc) < or = 3 years' duration (early lSSc), 22 with lSSc > 3 years' duration (late lSSc), 9 with diffuse cutaneous SSc (dSSc) < or = 3 years' duration (early dSSc), and 12 with dSSc > 3 years' duration (late dSSc). RESULTS: Serum IL-6 levels were significantly elevated in patients with early dSSc compared with controls. In addition, serum IL-6 was detected more frequently in patients with pulmonary fibrosis, and these values were inversely correlated with the percentage of vital capacity of individual patients. Furthermore, serum IL-6 levels were correlated with erythrocyte sedimentation rates, C-reactive protein, and IgG and IgA levels in patients. Serum sIL-6R levels were significantly higher in patients with lSSc versus controls. Serum OSM and sgp130 levels were not significantly elevated in patients with SSc compared with controls. CONCLUSION: We suggest that IL-6 and sIL-6R may contribute to the disease process in SSc. In particular, IL-6 may be related to the early phase of the disease and the development of pulmonary fibrosis in SSc.

Adolescent↗
PubMed2000

Macrovascular disease and systemic sclerosis.

OBJECTIVE: To determine if macrovascular disease is more prevalent in systemic sclerosis (SSc) compared with unaffected subjects. METHODS: 54 patients with SSc (both limited and diffuse disease) and 43 unaffected control subjects of similar age and sex were recruited. All subjects underwent a basic screen for conventional atherosclerotic disease risk factors. All had non-invasive vascular assessments-that is, carotid duplex scanning and measurement of ankle brachial blood pressure index-to identify the presence of asymptomatic peripheral vascular disease. RESULTS: 33 of 52 (64%) patients had carotid artery disease compared with only 15 of 42 (35%) controls (p=0.007). Eleven (21%) of these patients had moderate disease compared with only two (5%) controls (NS). Nine of 53 (17%) SSc patients had evidence of peripheral arterial disease compared with no controls. This result was also statistically significant (p=0.003). There were no significant differences in the basic risk factor profile, which included cigarette smoking, systolic and diastolic blood pressure, cholesterol, trigyceride and glucose concentrations. CONCLUSION: Macrovascular disease is more common in SSc. Screening of these patients may allow identification of "at risk" patients at an early stage and allow the study of treatments to attenuate the high rate of cardiovascular mortality in these patients.

Adult↗
PubMed2003

Serum concentrations of the CXC chemokines interleukin 8 and growth-regulated oncogene-alpha are elevated in patients with systemic sclerosis.

OBJECTIVE: To determine whether serum concentrations of 2 CXC chemokines, interleukin 8 (IL-8) and growth-regulated oncogene-alpha (GRO-alpha), which are potent chemoattractants and activators for neutrophils, are elevated and whether they correlate with clinical features in patients with systemic sclerosis (SSc). METHODS: Serum samples from patients with diffuse cutaneous SSc (dSSc; n = 36), limited cutaneous SSc (lSSc; n = 42), systemic lupus erythematosus (SLE; n = 15), dermatomyositis (DM; n = 15), and healthy controls (n = 35) were examined by ELISA. RESULTS: Serum IL-8 was detected significantly more frequently in patients with dSSc (61%) and lSSc (55%) relative to healthy controls (6%), patients with SLE (7%), and those with DM (13%). Similarly, serum GRO-alpha concentrations in SSc patients were significantly increased compared with controls, patients with SLE, or those with DM. Elevated IL-8 concentrations significantly correlated with decreased % DLCO and rheumatoid factor positivity, while increased GRO-alpha levels were significantly associated with decreased % DLCO and % vital capacity, involvement of kidney and muscle, the presence of anti-topoisomerase I antibody, and increased serum IgG levels. CONCLUSION: Our results suggest that the elevation of serum levels of the CXC chemokines IL-8 and GRO-alpha is specific to SSc and that the elevation of CXC chemokines, particularly GRO-alpha, correlates with the involvement of internal organs, especially pulmonary damage.

Adolescent↗
PubMed2001

Clinical significance of soluble CD31 in patients with systemic sclerosis (SSc): association with limited cutaneous SSc.

OBJECTIVE: To determine serum levels of soluble CD31 (sCD31) and its clinical associations in patients with systemic sclerosis (SSc). METHODS: Serum sCD31 levels from 70 patients with SSc were examined by ELISA. For a longitudinal study, 64 sera from 17 SSc patients were analyzed (followup: 0.4-3.9 yrs). RESULTS: Serum sCD31 levels were elevated in patients with SSc (n = 70) compared with healthy controls (n = 20) and patients with systemic lupus erythematosus (n = 15). Serum sCD31 levels were higher in patients with limited cutaneous SSc (lSSc; n = 37) than those with diffuse cutaneous SSc (n = 33). Patients with elevated sCD31 levels had pulmonary fibrosis and decreased percentage vital capacity (%VC) less frequently than those with normal sCD31 levels. sCD31 levels correlated positively with %VC in patients with SSc. This association of elevated sCD31 levels with the lower frequency of pulmonary involvement and better %VC was still observed when analyzed among ISSc patients alone. The elevation of sCD31 was associated with shorter disease duration in patients with lSSc. In a longitudinal study, 75% of patients with SSc showed increased sCD31 levels only transiently in the early phase of the disease. Serum sCD31 levels remained normal during followup in all patients with normal sCD31 levels at the first visit. CONCLUSION: Elevated sCD31 levels were associated with ISSc with relatively early onset and lower frequency and severity of pulmonary fibrosis. These results suggest that sCD31 would be a protective factor for the development of skin sclerosis and pulmonary fibrosis in SSc, since sCD31 has an antiinflammatory effect by inhibiting CD31 mediated transendothelial migration of leukocytes.

Adolescent↗
PubMed2004

Elevated circulating CD40L concentrations in patients with systemic sclerosis.

OBJECTIVE: B cell activation, fibrosis, and expression of adhesion molecules on endothelial cells are regulated by soluble CD40L (sCD40L)/CD40 interactions. Since these effects are characteristic in patients with systemic sclerosis (SSc), serum concentrations of sCD40L were determined in patients with SSc. METHODS: Fifty-two Japanese patients with SSc were examined. They were grouped into 24 patients with limited cutaneous SSc (lSSc) and 28 with diffuse cutaneous SSc (dSSc). Serum sCD40L levels were examined by ELISA. As a disease control, serum samples from 20 patients with systemic lupus erythematosus (SLE) were also examined. In addition, a retrospective longitudinal study was performed in 71 serum samples from 18 patients with SSc. RESULTS: Serum sCD40L levels were elevated in SSc patients compared with healthy controls (p < 0.001). Levels of sCD40L in patients with SSc were higher than in patients with SLE (p < 0.001) that had elevated sCD40L levels compared with healthy controls. Among SSc subsets, there were no differences in sCD40L levels between lSSc and dSSc. sCD40L levels correlated positively with C-reactive protein levels in SSc patients (p < 0.0001, r = 0.449). In a cross-sectional study and a longitudinal study, serum sCD40L levels in dSSc patients were persistently elevated, although those in lSSc patients were temporarily elevated at the early phase of the disease process. CONCLUSION: Patients with SSc exhibited elevated sCD40L levels that may correlate with disease activity. These results suggest that CD40/CD40L interactions may be potential therapeutic targets in SSc.

Adolescent↗
PubMed2004

Different low doses of broad-band UVA in the treatment of morphea and systemic sclerosis.

BACKGROUND: Numerous treatment modalities, some with potentially hazardous side effects, are currently used for morphea (M) and systemic sclerosis (SS) with limited success. Low-dose ultraviolet A (UVA) phototherapy (20 J/cm(2)) was found to be highly effective for sclerotic patches, even in patients with advanced and rapidly evolving lesions. OBJECTIVE: To determine the effectiveness of different low doses of UVA in treating patients with M and SS. METHODS: Sixty-three patients complaining of M and 15 patients complaining of SS received 20 sessions of UVA (320-400 nm) each. Patients were divided randomly into three groups that received 5, 10 and 20 J/cm(2), with cumulative UVA doses of 100, 200, and 400 J/cm(2), respectively. The efficacy of therapy was judged clinically (by sequential inspection and palpation) and histopathologically by morphometry in M cases. RESULTS: Obvious clinical improvement, with no comparable differences between various low UVA doses, was noted in patients with M and SS, accompanied by histopathological changes towards normalization of collagen. CONCLUSIONS: After 20 sessions, it appears that lower doses of UVA (5, 10 J/cm(2)) are as beneficial as the relatively higher dose (20 J/cm(2)) in the treatment of M and SS.

Adolescent↗
PubMed2002

[The high frequency of spontaneous micronuclei observed in lymphocytes of systemic sclerosis patients: preliminary results].

OBJECTIVE: Aim of the study is to assess the presence of spontaneous chromosome damage in patients affected by limited (lSSc) or diffuse (dSSc) Systemic Slerosis, using the micronucleus (MN) assay. METHODS: We evaluated MN frequency in cultured peripheral lymphocytes of 18 SSc and in a group of 20 healthy controls. Patients were also classified as ACA+, Scl70+, FAN+ according to the presence of the specific anti-nuclear antibodies. We also explored the hypothesis that the extent of cytogenetic alteration might be related to the severity of the pathological condition and/or to the immunological profile. RESULTS: Compared to controls, the patient group as a whole showed significantly higher MN frequencies (10.8+/-4.5 vs. 27.8+/-13.7, p<0.001). No correlation was found between spontaneous chromosome damage and severity of the disease, being MN frequency 33.1+/-17.0 and 19.8+/-2.7 in lSSc and dSSc, respectively. Interestingly, ACA+ subjects displayed the highest MN frequency (36.9+/-15.0), as compared to patients with different antibody pattern (Scl70+, FAN+; 19.7+/-8.2). CONCLUSIONS: Our results confirm the presence of chromosomal damage in circulating lymphocytes of SSc patients and would suggest a key role of antibodies to the centromere in determining the observed cytogenetic anomalies.

Aged↗
PubMed2000

Cardiovascular autonomic function, autoantibodies, and esophageal motor activity in patients with systemic sclerosis and mixed connective tissue disease.

OBJECTIVE: To study cardiovascular autonomic nerve function and presence of autoantibodies in relation to esophageal motor activity in patients with systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). METHODS: Twenty-five patients with SSc (13 limited, 12 diffuse cutaneous disease; disease duration 1-19 yrs) and 6 patients with MCTD (disease duration 1-10 yrs) were studied. Cardiovascular autonomic function was assessed using 5 standard tests and autoantibody status determined. Esophageal motor activity and lower and upper esophageal sphincter pressures were recorded manometrically. RESULTS: Five patients with SSc had definite, 7 borderline, and 13 no autonomic dysfunction; 23 had antinuclear. 9 anti-Sc170, 4 anticentromere, and 1 U1snRNP antibodies. Contraction amplitudes in the smooth muscle as well as the striated muscle esophagus and lower esophageal sphincter pressures were significantly lower and autonomic dysfunction more frequent in patients with than in those without anti-Sc170 (6 of 9 vs 6 of 16 patients); upper esophageal sphincter pressures did not differ. All patients with MCTD had antinuclear antibodies, 5 had definite autonomic dysfunction; their lower esophageal sphincter pressures were significantly lower than in SSc patients without anti-Sc170 and anti-U1snRNP. CONCLUSION: Esophageal motor dysfunction may be associated with the presence of anti-Sc170 and anti-U1snRNP autoantibodies and prevail in patients with cardiovascular autonomic neuropathy.

Adult↗
PubMed2005

Chemokine receptor CCR2 expression by systemic sclerosis fibroblasts: evidence for autocrine regulation of myofibroblast differentiation.

OBJECTIVE: To investigate expression of the chemokine receptor CCR2 on key cell types involved in the pathogenesis of systemic sclerosis (SSc) and to assess the potential for autocrine activation of SSc dermal fibroblasts via CCL2/CCR2. METHODS: Chemokine receptor expression in skin biopsy tissues and explanted dermal fibroblasts from a well-characterized cohort of SSc patients was examined using immunohistochemistry and flow cytometry techniques. Autocrine regulation of the expression of fibrotic markers in CCR2+ SSc fibroblast cell lines was assessed using specific ligand or receptor antagonists. RESULTS: We identified strong CCR2 expression in skin biopsy samples of early-stage diffuse cutaneous SSc (dcSSc), but not late-stage dcSSc or limited cutaneous SSc. Double labeling confirmed up-regulation of CCL2/CCR2 on myofibroblasts, pericytes, lymphocytes, macrophages, and endothelial cells. Explanted dermal fibroblasts from early dcSSc tissues expressed CCR2 and CXCR2 in 55% and 66% of cell strains, respectively. There was no expression in control fibroblasts. CCR2+ fibroblasts demonstrated a profibrotic phenotype, with overexpression of alpha-smooth muscle actin (alpha-SMA), connective tissue growth factor (CTGF), and CCL2. Flow cytometric analysis identified a subset of CCR2+ SSc fibroblasts expressing the myofibroblast marker alpha-SMA. In these cultures, specific inhibition of CCL2 or CCR2 attenuated the overexpression of alpha-SMA, but not CTGF or plasminogen activator inhibitor 1. CONCLUSION: Our results show that CCR2 is up-regulated in early dcSSc on cell types known to be activated in the disease, which is consistent with a key role in SSc pathogenesis. CCR2 expression on SSc fibroblasts appears to regulate the expression of CCL2 and alpha-SMA. Our findings suggest potential autocrine regulation of key profibrotic properties via a CCL2/CCR2 loop in early-stage dcSSc.

Actins↗
PubMed2002

Abnormal expression of intracellular cytokines and chemokine receptors in peripheral blood T lymphocytes from patients with systemic sclerosis.

In patients with systemic sclerosis (SSc), there are conflicting findings regarding which is predominant between type 1 and type 2 immune responses. To determine the balance between type 1 and type 2 T lymphocytes in peripheral blood from SSc patients, we investigated the expression of intracellular cytokines, such as interferon-gamma (IFN-gamma), interleukin-2 (IL-2), IL-4, and IL-13, and chemokine receptors such as CXCR3 and CCR4 by flow cytometry. The frequency of IFN-gamma-producing cells among CD8+ cells was significantly increased in patients with diffuse cutaneous SSc (n = 11, P < 0.0001) and limited cutaneous SSc (lSSc; n= 16, P < 0.0001) compared with normal controls (n = 17) while there was no significant difference in the frequency of IL-4- or IL-13-producing cells. In contrast, the frequency of IFN-gamma- or IL-4-producing cells among CD4+ cells was similar between the three groups. Similar results were obtained when absolute numbers were assessed. The frequency of IFN-gamma-producing cells among CD8+ cells inversely correlated with percentage DLco in SSc patients (r = - 0.650, P < 0.005). CXCR3+ CD8+ cells selectively produced IFN-gamma, and the frequency of CXCR3+ CD45RO+ cells among CD8+ cells was higher in lSSc patients (n = 14, P < 0.01) than in normal controls (n = 22). In contrast, there was no significant difference in the frequencies of CXCR3- or CCR4-expressing CD45RO+ cells among CD4+ cells. These results demonstrate the predominance of type 1 cytokine-producing cells (Tc1 cells) in peripheral blood CD8+ T cells from SSc patients, but no definite Th1/Th2 imbalance in CD4+ T cells. Tc1 cells may be associated with pulmonary vascular damage in SSc.

Adult↗
PubMed2005

Anti-lipoprotein lipase antibody in systemic sclerosis: association with elevated serum triglyceride concentrations.

OBJECTIVE: The vascular damage systemic sclerosis (SSc) consists mainly of microvascular changes, but recently macrovascular changes with dyslipidemia were recognized. In systemic lupus erythematosus (SLE), autoantibody to lipoprotein lipase (LPL), a key enzyme that hydrolyzes triglycerides, suggested a role of autoimmunity for elevated serum triglyceride levels and atherosclerosis. We investigated the prevalence and levels of anti-LPL antibodies, their clinical correlation, and their functional significance in patients with SSc. METHODS: Serum samples from patients with diffuse cutaneous SSc (dSSc; n = 55), limited cutaneous SSc (lSSc; n = 75), SLE (n = 21), and dermatomyositis (DM; n = 21) and healthy controls (n = 41) were examined by ELISA. The presence of anti-LPL antibody was evaluated by immunoblotting analysis using purified LPL. To determine the functional relevance of anti-LPL antibody in vivo, we assessed whether anti-LPL autoantibody was able to inhibit LPL activity using the LPL activity kit. RESULTS: ELISA revealed that IgG or IgM anti-LPL antibodies were detected in 35% of SSc patients, while they were also positive in 67% of SLE patients and 43% of DM patients. The presence of IgG anti-LPL antibody was associated with elevated serum triglyceride levels, greater extent of skin fibrosis, and more frequent presence of lung fibrosis, heart involvement, and anti-topoisomerase I antibodies. The presence of anti-LPL autoantibody was confirmed by immunoblotting analysis. LPL activity was inhibited by IgG anti-LPL antibodies in sera from SSc patients with elevated serum triglyceride levels. CONCLUSION: Our results suggest that anti-LPL autoantibody contributes to elevated serum triglyceride levels by inhibiting LPL enzyme activity in patients with SSc.

Autoantibodies↗
PubMed2000

Extended extracorporeal photochemotherapy with extracorporeal administration of 8-methoxypsoralen in systemic sclerosis. An Austrian single-center study.

BACKGROUND: Extracorporeal photochemotherapy (EXP) is an immunomodulating therapy that has been used in a limited number of patients with systemic sclerosis (SSC) with controversial results. The present study was performed to evaluate the efficacy and safety of extended EXP with extracorporeal application of liquid 8-methoxypsoralen (8-MOP) in the treatment of SSC. METHODS: Eleven women with progressive SSC of recent onset were treated for a period of 16-57 months. Skin changes, physical performance, extracutaneous manifestations, and quality of life were evaluated before initiation of EXP and at regular intervals thereafter. RESULTS: From the start to the last set of EXP, we observed an overall improvement and/or stabilization of skin changes and physical performance in 5 of 11 patients (45%). Extracutaneous manifestations deteriorated in 10 of 11 patients (91%) (P<0.05), and quality of life deteriorated in 9 of 11 patients (82%) from a mean score of 10 before, to 17 at the last set of EXP (P<0.05). No major side effects were noted. CONCLUSION: Extended EXP with extracorporeal administration of 8-MOP is a safe and well tolerated treatment modality. However, it provides only (minor) improvement of skin changes of a subset of SSC patients and does not beneficially influence extracutaneous manifestations and quality of life.

Adult↗
PubMed1996

Von Willebrand factor, thrombomodulin, thromboxane, beta-thromboglobulin and markers of fibrinolysis in primary Raynaud's phenomenon and systemic sclerosis.

OBJECTIVE: To determine whether measurement of different markers of endothelial damage, activation of coagulation, and platelet activation might differentiate between patients with primary Raynaud's phenomenon (PRP), limited cutaneous and diffuse systemic sclerosis (lcSSc and dSSc), and healthy control subjects. METHODS: Under carefully controlled conditions, fasting blood was drawn from 19 healthy control subjects, 10 patients with PRP, 17 with lcSSc and nine with dSSc for measurement of the following: von Willebrand factor (VWF) and soluble thrombomodulin as markers of endothelial damage/activation, thromboxane (as thromboxane B2) and beta-thromboglobulin as markers of platelet activation, and tissue plasminogen activator antigen, tissue plasminogen activator activity and plasminogen activator inhibitor-1 (PAI-1) as markers of fibrinolysis. RESULTS: VWF was increased significantly in patients with SSc, and there was also a linear trend for thromboxane and tissue plasminogen activator antigen (in addition to VWF) to differentiate between different subgroups of patients with Raynaud's phenomenon. Patients with dSSc had the highest values. A combined index of VWF and thromboxane showed a highly significant trend across the four groups studied. CONCLUSION: VWF, and to a lesser extent thromboxane and tissue plasminogen activator antigen, are associated with disease severity in patients with Raynaud's phenomenon. Prospective studies are now required to establish if these parameters can be used as markers of disease progression.

Adult↗
PubMedDate not supplied

Capillary density in patients with systemic sclerosis, as determined by microscopy counts and compared with computer-based analysis.

OBJECTIVE: To develop a method enabling capillary density to be determined rapidly and accurately in patients with systemic sclerosis. METHOD: Capillary density was determined in 11 controls and 22 patients: 5 with diffuse cutaneous systemic sclerosis (dSSc), 12 with limited cutaneous systemic sclerosis (lSSc), two with suspected systemic sclerosis (suspSSc), 2 with sclerodermatomyositis, and one with undifferentiated connective tissue disease. Using a microscope equipped with a graticule, nailfold capillaries were counted within a 3 mm length of the nailfold; these counts were made by 4 different observers. The results were compared with the corresponding values obtained by the computerbased analysis of photographs. RESULTS: The median capillary density according to the direct counts was 8.0 loops/mm (6.7-10.0) in the controls, 6.0 loops/mm (range 4.8-8.8) in the dSSc subgroup, 5.6 loops/mm (4.2-6.5) in the lSSc subgroup, and 7.2 loops/mm (6.2-8.2) in the suspSSc subgroup. In the series as a whole, there was no significant difference between the median values for the left hands and those for the right hands, nor between the median value for all digit IVs and the median value for all four digits analysed (II, III, IV, and V). Interobserver variation was small between the 4 different observers. Direct microscopy counts were slightly higher than the corresponding values obtained by computer-based analysis. CONCLUSION: Direct microscopy counting is a rapid, simple, and reliable means of determining capillary density for screening purposes.

Adult↗
PubMed1997

Test performance in systemic sclerosis: anti-centromere and anti-Scl-70 antibodies.

PURPOSE: To determine the sensitivity and specificity of anti-centromere (ACA) and anti-Scl-70 antibodies in systemic sclerosis (SSc). METHODS: Four-hundred ninety-seven English language articles published from 1966 to 1994 were identified by structured MEDLINE search. Articles in which either ACA or anti-Scl-70 antibodies were measured in both SSc patients and a non-SSc control group were reviewed and rated using a previously published diagnostic testing scale. Reported sensitivity and specificity from each study was converted into a 2 x 2 table, and combined across studies to calculate summary rates for each antibody. Author's clinical classification criteria for SSc served as the gold standard for disease diagnosis. RESULTS: In 30 articles that fulfilled inclusion criteria, ACA were found in 441 of 1,379 SSc patients (sensitivity 32%, range 17% to 56%). This increased to 57% (332 of 585) in patients with the limited cutaneous, or CREST, subset of SSc (IcSSc). Anti-Scl-70 antibodies were found in 366 of 1,074 SSc patients (sensitivity 34%, range 3% to 75%), and this increased slightly to 40% in patients with the diffuse cutaneous form of SSc (dcSSc). Both antibodies were measured in 670 patients, and either test was positive in 58% (range 29% to 86%), but in only 3 patients were both antibodies present. The specificity of each antibody was high, but varied by control group. ACA were present in 5% and anti-Scl-70 antibodies were present in 2% of patients with other connective tissue diseases, but fewer than 1% of disease free controls had either antibody present. CONCLUSIONS: As individual diagnostic tests in SSc, both ACA and anti-Scl-70 antibodies are highly specific. Each performs somewhat better as discriminators of clinical subsets for patients in whom a diagnosis of SSc has already been established. Clinicians can rely on a positive test result as being specific in the detection of disease, but 40% of SSc patients are likely to have neither antibody present, and a negative result does not exclude the diagnosis. Measurement of these antibodies should be considered secondary to the clinical features when making a diagnosis of SSc.

Antibodies, Antinuclear↗
PubMed2004

Oesophageal manometry in early and definite systemic sclerosis.

The objective of this study was to evaluate the oesophageal dysfunction in patients with "early" systemic sclerosis (SSc), as defined by LeRoy and Medsger, to compare it with that of patients with definite SSc, and to correlate it with other features of the disease. Oesophageal manometry results were retrospectively evaluated in 181 patients classified by the 2001 LeRoy and Medsger criteria and the 1980 American College of Rheumatology (ACR) criteria: group 1: limited SSc: Raynaud's phenomenon plus specific nailfold capillaroscopy abnormalities and/or autoantibodies; group 2: limited cutaneous SSc not satisfying the ACR criteria (lcSSc ACR-); group 3: lcSSc ACR+; group 4: diffuse cutaneous SSc. Peristaltic abnormalities in the oesophageal body were present in 73 of 125 patients with SSc ACR+ (groups 3 and 4) compared with 13 of 56 with SSc ACR- (groups 1 and 2) (p < 0.0001). They were more severe in patients with more advanced disease (1 vs 2; 1 vs 3; 1 vs 4; 2 vs 4; p < 0.05) and in patients anti-Scl-70+ than in patients anticentromere positive (p = 0.02). Abnormalities of the lower oesophageal sphincter (LES) were present in 35 of 125 patients with SSc ACR+ and 11 of 56 with SSc ACR- (not statistically different). They were correlated with forced vital capacity (FVC) (LES pressure: p = 0.0005; LES length: p = 0.0004). Abnormalities of the oesophageal body and of the LES were found in 21 and 16% of 46 patients without oesophageal symptoms. Oesophageal manometry can detect abnormalities in a sizeable proportion of patients with "early SSc" not fulfilling the ACR criteria, including asymptomatic patients. The correlation between LES abnormalities and FVC suggests a possible causal relationship between these disease manifestations.

Adult↗