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PubMed1992

Systemic sclerosis in the elderly.

In our study, the characteristics of 114 patients with systemic sclerosis (SSc) are discussed with emphasis on the subgroup of cases whose onset of disease occurred above the age of 60 years. Seven out of the 9 cases showed symptoms of diffuse cutaneous systemic sclerosis with an extensive skin involvement, and 5 of these cases died within 2 years following the onset of SSc. Seven of the 9 cases showed a rapid disease course with symptoms of cardiac, pulmonary and/or renal involvement, while no secondary Sjögren's syndrome, subcutaneous calcinosis and myositis were demonstrated among these patients.

Aged↗
PubMed1990

Clinical and laboratory profile of systemic sclerosis in northern India.

The clinical and laboratory features of 87 patients with systemic sclerosis (SSc) are described. A large majority of patients presented with diffuse disease with extensive ulcerations and digital resorption. No case of CREST syndrome was seen. The pattern of systemic involvement was generally similar to that reported in the Western literature. Renal involvement appeared to be less aggressive and chronic in its course. A possible aetiology was identified in three patients.

Adolescent↗
PubMed1982

Eosinophilic fasciitis.

Eosinophilic fasciitis (EF, a diffuse fasciitis with eosinophilia) is a recently recognized connective tissue disorder. It consists of deeply indurated, bound-down plaques of skin and subcutaneous tissue, most commonly present on the extremities. It is associated with peripheral eosinophilia, hypergammaglobulinaemia and an elevated sedimentation rate. There is usually no evidence of Raynaud's phenomenon, acral sclerosis or visceral involvement. Antinuclear antibodies are usually absent. The abnormal histopathology primarily involves the lower subcutis and the fascia. The clinical and laboratory features usually improve following the administration of systemic corticosteroids. Serious haematological abnormalities have been associated with eosinophilic fasciitis (EF) and have been discussed in detail. The similarities and distinctions between eosinophilic fasciitis and scleroderma have been discussed.

Adrenal Cortex Hormones↗
PubMed2006

Significant elevation of IgG anti-WRN (RecQ3 RNA/DNA helicase) antibody in systemic sclerosis.

Werner syndrome, caused by the homologous mutation of RecQ3 RNA/DNA helicase (WRN), is often misdiagnosed as systemic sclerosis (SSc) because of apparent similar skin changes and its relatively high frequency in Japan. The present study was undertaken to determine whether anti-WRN antibodies assayed by specific enzyme-linked immunosorbent assay occur in 41 SSc patients (30 diffuse and 11 limited types) and, if so, to determine any clinical association, such as skin sclerosis. Serum level of IgG anti-WRN antibody in SSc was significantly higher than that from 30 age- and sex-matched normal volunteers (P < 0.001). The serum level of IgG anti-WRN antibody in diffuse type SSc was significantly higher than the limited type (P < 0.05). A significant correlation was observed between serum levels of IgG anti-topoisomerase I antibody and IgG anti-WRN antibody in the same samples from SSc (P < 0.05). Moreover, in 119 normal healthy individuals aged from 0 to 99 years, a statistically significant correlation (P < 0.001) existed between serum level of IgG anti-WRN antibody and advancing age. A significantly higher level of IgG autoantibody specific for WRN detected in diffuse than in limited type SSc and normal may contribute to the pathogenesis of skin sclerosis in SSc.

Adolescent↗
PubMed1986

The cutaneous mucinoses.

The cutaneous mucinoses are a group of connective tissue disorders characterized by the deposition of mucin, either focally or diffusely, in the interstices of the dermis. The diseases may be a primary (metabolic) or secondary (catabolic) process. Systemic abnormalities are seen with most of these disorders. This review discusses the primary mucinoses in which the predominant dermal mucin is hyaluronic acid. Current therapy and proposed mechanisms for the mucinoses are considered.

Age Factors↗
PubMed2004

Nailfold capillary microscopy can suggest pulmonary disease activity in systemic sclerosis.

OBJECTIVE: To evaluate the association of capillaroscopic alterations with pulmonary disease activity in systemic sclerosis (SSc). METHODS: Ninety-one patients with SSc were studied by means of interview, physical examination, nailfold capillary microscopy (NCM), serology, pulmonary function tests, esophageal transit scintigraphy, Doppler echocardiography, and pulmonary high resolution computed tomography (HRCT). Pulmonary disease activity was diagnosed by the observation of ground-glass opacities on pulmonary HRCT. Capillary loss on NCM was evaluated using the avascular score: patients with mean score > or = 1 or mean number of megacapillaries per finger > or = 1 were considered to have severe capillaroscopic alterations. RESULTS: Patients with higher skin scores, longer disease duration, signs of peripheral ischemia, esophageal dysfunction, antitopoisomerase I antibodies, and ground-glass opacities had higher mean avascular scores (p < or = 0.05 in all tests). The association between ground-glass opacities and higher avascular scores was particularly strong in patients with disease duration < or = 5 years. Among these patients, ground-glass opacities were present in 14 of 19 patients with severe NCM alterations, but were absent in all patients (n = 8) with mild or no NCM alterations (p < 0.001). ROC curves confirmed the ability of NCM to discriminate between patients with and without ground-glass opacities among those with disease duration < or = 5 years. However, NCM could not predict the presence of reduced pulmonary diffusing capacity. CONCLUSION: The severity of NCM abnormalities is associated with lung disease activity in SSc, particularly when the disease duration is relatively short.

Adult↗
PubMedDate not supplied

Dehydroepiandrosterone sulphate serum levels in systemic sclerosis.

OBJECTIVE: To evaluate in a cohort of women with systemic sclerosis (SSc) the dehydroepiandrosterone sulphate (DHEAS) serum levels and their relationship with disease severity. METHODS: DHEAS serum concentrations were measured by radioimmunoassay in 40 SSc patients and compared with those in 40 controls matched for sex and reproductive status. IL-2 sR alpha was evaluated as a disease activity index. A preliminary organ/system severity scale proposed by Medsger et al. in 1999 was used to evaluate disease severity. RESULTS: Mean serum levels of DHEAS in SSc women of childbearing age were significantly lower than in controls (0.87 +/- 0.85 microgram/ml versus 2.75 +/- 0.42 micrograms/ml; p < 0.001). On the contrary, no difference was found between postmenopausal women and controls. A reduction below the 95% confidence limits was found in 10 out of 11 patients of childbearing age and in 8 out of 29 postmenopausal women, respectively. In 5 out of 11 patients of childbearing age taking steroids for their SSc (< 10 mg/daily) DHEAS levels were significantly lower than in patients not taking steroids (p = 0.01). On the contrary, 16 out of 29 postmenopausal women using steroids had lower DHEAS concentrations than in patients not taking steroids, although the difference was not statistically significant. There was no statistically significant difference in DHEAS levels between patients with diffuse or limited SSc, or between those with or without organ system involvement. No correlations were found either in pre- and post-menopausal steroid nonusers, or in limited and diffuse subsets, between DHEAS levels and age, postmenopausal years, disease duration, IL-2 sR alpha, disease organ/system severity scale. CONCLUSION: Our data show that, as in other autoimmune diseases, low serum DHEAS is a feature of premenopausal SSc patients. More extensive prospective studies are needed to define the exact role of DHEAS dysregulation in SSc.

Adolescent↗
PubMed2002

Causes of death and poor survival prognostic factors in thai patients with systemic sclerosis.

Causes of death and poor prognostic factors for patients with systemic sclerosis (SSc) were studied in 222 cases. Their mean age at the onset and duration of disease was 48.9 +/- 12.0 years and 23.3 +/- 29.3 months, respectively. Fifty-three per cent were diffuse subtype. Patients with diffuse SSc had more digital pitting scars and more muscle, heart, lung, and esophageal involvement than those with limited subtypes (p < or = 0.02). One hundred and six patients were lost to follow-up. With a median follow-up duration of 25 months, 31 of the remaining 116 patients (26.7%) died. SSc related death occurred in 18 cases, in which the lung, heart and kidney (renal crisis) were the major causes. Infection contributed to the remaining 13 deaths. When compared with living patients, using a univariate analysis, factors associated with a reduced survival rate were age of > 45 years at the onset, diffuse skin thickness, and lung, gastrointestinal tract, heart, kidney and muscle involvement (p < or = 0.001). In the multivariate analysis, only age of > 45 years at onset and cardiac involvement remained poor prognostic factors (p = 0.04 and 0.001, respectively).

Adult↗
PubMed1998

Serum concentrations of vascular endothelial growth factor in collagen diseases.

Vascular endothelial growth factor (VEGF) is an angiogenic cytokine which has been reported to be important in the pathogenesis of rheumatoid arthritis (RA). In this study, the serum level of VEGF was measured using enzyme-linked immunosorbent assay in 17 patients with systemic lupus erythematosus, 49 patients with polymyositis/dermatomyositis (PM/DM), 40 patients with systemic lupus erythematosus, 49 patients with polymyositis/dermatomyositis (PM/DM), 40 patients with systemic sclerosis (SSc), 11 patients with RA and 20 control subjects. The VEGF level was 184 +/- 62 pg/mL (mean +/- SD) in the serum of normal individuals. The mean VEGF levels in the patients with PM/DM or RA were significantly higher than in the normal controls. In 21 of the 49 patients with PM/DM and nine of the 11 patients with RA, the serum VEGF level was considered to be elevated. In patients with SSc, those with diffuse cutaneous SSc showed elevated VEGF levels in comparison with normal controls. An elevated serum VEGF level was correlated with the frequency of lung fibrosis and reduced vital capacity in the patients with SSc.

Adolescent↗
PubMed1994

Acute effects of single dose nifedipine on cold-induced changes of microvascular dynamics in systemic sclerosis.

Calcium-channel blockers are widely used in the treatment of systemic sclerosis (SSc), but their in vivo influence on microcirculation is not fully elucidated. We evaluated the acute effect of nifedipine on the cold-induced changes of microvascular dynamics in SSc. Eleven SSc patients and seven healthy volunteers were studied. Dynamic aspects of the nailfold microcirculation (appearance time at the nailfold, transcapillary diffusion, interstitial distribution and interstitial clearance of sodium fluorescein given i.v.) were quantitatively assessed by a computer-aided fluorescence videomicroscope. Fluorescent light intensities (FLIs) at predefinite pericapillary and interstitial sites were measured under three experimental conditions: (1) baseline; (2) after cold test; (3) after single oral administration of 10 mg of nifedipine 5 min before cold exposure. The interval between the intravenous injection of sodium fluorescein and the first appearance of the dye at the nailfold significantly increased after cold exposure in the SSc patients (224.1 +/- 182.3 s vs 27.5 +/- 25.1 s at baseline) (P = 0.0026), but not in the controls (28.0 +/- 13.3 s vs 29.6 +/- 12.4 s at baseline). The effect of cold exposure on the appearance of the dye was not significantly antagonized by nifedipine (112.7 +/- 91.8 s) in the SSc patients (P = 0.07). Cold exposure significantly decreased transcapillary diffusion and interstitial distribution of sodium fluorescein in the SSc patients (P < 0.016), but not in the controls. The cold-induced changes of FLI values were antagonized by nifedipine in the SSc patients (P < 0.016), but not in the controls.(ABSTRACT TRUNCATED AT 250 WORDS)

Administration, Oral↗
PubMed2006

Arteriographic evaluation of vascular changes of the extremities in patients with systemic sclerosis.

BACKGROUND: Although digital ulcerations frequently occur in patients with systemic sclerosis (SSc), there have been few reports on the macrovascular involvement. OBJECTIVES: To evaluate the macrovascular involvement in patients with SSc exhibiting digital ulceration or gangrene. METHODS: Transfemoral catheter arteriography of the upper and/or lower extremities was performed in eight patients (one man and seven women, age range 42-71 years) with SSc exhibiting digital ulceration or gangrene. The background of the patients, such as autoantibody profiles and vascular risk factors including smoking habits, was also investigated. RESULTS: Macrovascular involvement was detected in seven of eight patients. In three of seven patients who underwent arteriography of the upper extremity, occlusion was limited to the digital arteries. Obliteration of the ulnar artery and superficial palmar arch was detected in three of seven patients, and the radial artery in one patient. Only one of five patients who underwent arteriography of the lower extremity showed the occlusion limited to digital arteries of the foot. Occlusion of the posterior tibial artery, dorsalis pedis artery and arcuate artery was detected, each in one patient. Two patients showed occlusion of the plantar arch. Overall, the occlusion of arteries proximal to the digits was demonstrated in four of eight patients. Three of the four patients were positive for antitopoisomerase-1 antibody and had diffuse cutaneous SSc (dcSSc) with multiple skin ulcers or gangrene. CONCLUSIONS: Macrovascular involvement as detected with arteriography is not rare in SSc patients with digital ulceration or gangrene. Moreover, the vascular occlusion proximal to the digits seemed to be frequent in antitopoisomerase-1 antibody-positive dcSSc patients with multiple skin ulcers or gangrene.

Adult↗
PubMed1995

Lack of natural killer cell augmentation in vitro by human interferon gamma in a subset of patients with systemic sclerosis.

Systemic sclerosis (SSc) is a generalized connective tissue disorder characterized by fibrosis of skin and various viscera. Natural killer (NK) cells are a subset of lymphocytes that can lyse targets without prior sensitization. Few studies have tried to assess NK cell function in patients with SSc. To evaluate NK cell cytotoxicity in patients with SSc and to see the extent of its augmentation in vitro by human interferon (hIFN) gamma in the clinical subset of limited and diffuse cutaneous diseases, we evaluated 27 patients with SSc and 22 age- and sex-matched controls by 51Cr release assay. Fifteen patients had limited cutaneous disease (mean disease duration 6.2 +/- 2.7 years) and 12 diffuse cutaneous disease (mean disease duration 5.7 +/- 2.4 years). Patients with limited SSc had significantly higher baseline NK cell function than controls (p < 0.05) and the augmentation following in vitro stimulation with hIFN gamma was negligible. Patients with diffuse SSc had lower baseline NK cell cytotoxicity than controls but this was not statistically significant. Augmentation with hIFN gamma in this group was comparable to controls. This study suggests that NK cells may have a role in the pathophysiology of this disease.

Adult↗
PubMedDate not supplied

Clinical value of a peculiar nuclear fluorescence staining in systemic sclerosis.

Using unfixed rat liver sections as substrate for the detection of antinuclear antibodies, distinct nuclear staining was observed in the sera of patients with systemic sclerosis. Its reaction differed from previously described fluorescence patterns and was characterized by numerous fine speckles throughout the nucleus associated with one or more coarse lines. These lines were more evident in diluted sera, when fine speckled fluorescence faded. This picture "fine speckles with lines" (FLS) was defined. The FLS pattern was present in 35% of 91 patients with systemic sclerosis, while it was absent in 517 patients with rheumatic and non-rheumatic disorders and in 100 matched healthy subjects. Moreover, the FSL pattern was significantly associated with the diffuse subset of systemic sclerosis (50% of cases) and was related to the presence of anti-Scl-70 antibody. These results indicate that this fluorescent pattern of antinuclear antibodies may be considered a simple and very useful aid in the diagnosis and prognosis of systemic sclerosis.

Adult↗
PubMed1985

Serum beta 2-microglobulin in systemic sclerosis.

The role of beta 2 microglobulin (beta 2-m) in Systemic Sclerosis (SS) has been evaluated. Twenty-four female patients have been examined: 15 of them were affected by acrosclerosis (Group 1) and 9 of them by diffuse sclerosis (Group 2). 46.6% of Group 1 and 44.4% of Group 2 had values significantly higher than normal controls. (P less than 0.01 and P less than 0.005 respectively). The authors deal with the validity of the use of B2-m as index of inflammatory activity of the disease.

Adult↗
PubMed1998

Management of systemic sclerosis: the art and science.

There have been substantial strides in the therapy of systemic sclerosis (SSc) in recent years, particularly in the management of individual organ manifestations. Effective treatments are available for SSc renal crisis and many of the gastrointestinal manifestations of the disease. Raynaud's phenomenon, a nearly universal problem in SSc, also may be effectively managed. Treatment of the pulmonary complications, pulmonary hypertension and interstitial lung disease, remains difficult. Patients with early, diffuse SSc are the best candidates for experimental therapies intended to modify the overall disease process. Most disease-modifying agents have been directed at the fibrotic and inflammatory processes characteristic of SSc and have achieved little success. Future therapies may target mediators of vascular dysfunction in SSc. The success of future therapeutic trials will depend on collaborative efforts between treatment centers.

Clinical Trials as Topic↗
PubMedDate not supplied

Systemic sclerosis complicated by diffuse alveolar hemorrhage.

A 38-year-old woman with limited cutaneous systemic sclerosis and pulmonary fibrosis developed diffuse alveolar hemorrhage during the course of her disease that responded well to steroids. We present the clinical history of the patient and discuss the different theories behind the association. The importance of steroid therapy for treatment of alveolar hemorrhage in this particular condition is emphasized.

Adult↗
PubMed2004

IgG reactivity with a 100-kDa tissue and endothelial cell antigen identified as topoisomerase 1 distinguishes between limited and diffuse systemic sclerosis patients.

We have analyzed antibody (Ab) reactivities of patients with limited systemic sclerosis (SSc) and anti-centromere Ab, patients with diffuse SSc and anti-topoisomerase 1 (anti-topo 1) Ab, patients with diffuse SSc without anti-topo 1 or anti-centromere Ab and age- and gender-matched healthy controls with normal human tissue and endothelial cell (EC) antigens. IgG reactivities with tissue antigens differed significantly between patients with anti-topo 1 Ab and patients with anti-centromere Ab. One 100-kDa band identified as topoisomerase 1 in macrovascular and microvascular EC extracts was recognized by IgG from patients with anti-topo 1 Ab and 50% of patients without specific Ab. IgG from patients with limited SSc and anti-centromere Ab, but not those of other patients or controls specifically recognized a 80-kDa band only in microvascular EC. Our results indicate that Ab from patients with limited or diffuse SSc with or without anti-topo 1 Ab exhibit specific and mutually exclusive reactivity patterns.

Autoantibodies↗
PubMed2002

Autoantibody to DNA binding protein B as a novel serologic marker in systemic sclerosis.

Systemic sclerosis is a systemic disease that is characterized by tissue fibrosis, small-vessel vasculopathy, and an autoimmune response associated with autoantibodies. We performed serological analysis of cDNA expression library (SEREX) to identify autoantibodies associated with systemic sclerosis. We identified 4 clones that react with sera of patients with SSc but not with those of healthy donors. These clones are phosphoglycerate mutase, centromere autoantigen C, U1 small nuclear ribonucleoprotein, and DNA binding protein B (dbpB). We chose to study autoantibody to DNA binding protein B. Immunoreactivity against recombinant dbpB was detected in 40.5% (15/37) of patients with SSc, 14.6% (6/41) of patents with systemic lupus erythematosus, 6.7% (1/15) of patients with rheumatoid arthritis, 0% (0/12) of patients with Sjogren syndrome, and 5.9% (1/17) of patients with polymyositis/dermatomyositis. The frequency of anti-dbpB was significantly higher in the SSc patients (15/37, 40.5%) compared to the healthy controls (3/41, 7.3%, p=0.0005 by chi(2) test). Eleven patients (11/20, 55%) with the diffuse cutaneous type of SSc had anti-dbpB and 4 patients (4/17, 23.5%) with the limited cutaneous type had anti-dbpB. The presence of anti-dbpB was significantly associated with the diffuse cutaneous type (p=0.00003 by chi(2) test). This is the first report to suggest that autoantibody to dbpB can be used as a serologic marker of systemic sclerosis.

Adult↗