SEARCH · PubMed Health

Results for “Scleroderma, Limited”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 217 records · Page 12Linked to original sources

PubMed2000

Pathogenesis of telangiectasia in scleroderma.

Scleroderma (progressive systemic sclerosis) is a systemic autoimmune disorder characterised by skin sclerosis, calcinosis and changes in microvasculature. The etiology of the disease is unknown but both genetic and environmental factors have been implicated. Telangiectasia (macroscopically visible dilated skin vessels) occurring primarily on the hands and face, are a prominent feature in scleroderma and are present in the majority of patients. Similarly, telangiectasia are found in patients with hereditary hemorrhagic telangiectasia (HHT), a mutational disorder of the germline genes endoglin and ALK-1, members of the TGFbeta receptor family, expressed on endothelial cells. Our study investigated the number, distribution and microscopic characteristics of telangiectasia in both limited (n = 29) and diffuse scleroderma (n = 9) and compared findings with 3 patients with HHT. In limited scleroderma, the mean number of telangiectasia (hand and face) was 36 (0-150) compared with 23 (0-135) in diffuse scieroderma. A significant correlation was observed between the number of telangiectasia on the face and on the hands (p = 0.014). The total number of telangiectasia correlated significantly with the disease duration (p = 0.009). The spatial distribution of the telangiectasia appeared to be random on both hands and foreface in contrast with the distribution of subcutaneous calcification of the hands which occurred predominantly on the distal and flexor surfaces of the first, second and fifth digits. Nailfold microscopic capillaroscopy was performed on 12 patients. No significant correlation was observed between capillary diameter or density and with total number of telangiectasia observed macroscopically. The distribution and microscopic appearance of telangiectasia in scleroderma appeared very similar to those observed in HHT. In view of these similarities we therefore conclude that telangiectactic development in scleroderma may be associated with disorders of the TGFb receptor family proteins found on the microvasculature.

Adult↗

PubMed2000

[Severe isolated pulmonary hypertension as main manifestation of Crest syndrome].

Interstitial pulmonary fibrosis is the leading cause of secondary pulmonary hypertension in systemic sclerosis, and it occurs in either limited or diffuse cutaneous scleroderma subset. Isolated pulmonary hypertension, without pulmonary disease, occurs primarily in patients with limited cutaneous scleroderma (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia [CREST] variant) although it is an unusual feature in this subset, with a worse prognosis in the short term. We present a previously undiagnosed patient with the CREST syndrome, with severe isolated pulmonary hypertension and secondary respiratory failure as major feature of its connective tissue disease. Clinical, prognostic and therapeutical aspects are commented.

Aged↗

PubMed1977

[Idiopathic and sclerodermic facial hemiatrophy with generalized myopathy. Clinical, electromyographic and histologic examinations of six patients].

Six patients with facial hemiatrophy (H.f) were thoroughly examined by clinical and laboratory investigations. Two were found to have idiopathic and four facial hemiatrophy due to different types of localized scleroderma. In all cases a generalized myopathy was present, demonstrated by electromyographical, histological, and biochemical means. In none of these cases a hereditary or neurological cause for the facial hemiatrophy was found. However, in two cases autoantibodies against nuclei and muscle were repeatedly obtained. No prolongation of sensory or pain chronaxy occurred in either the patients with sclerodermal or idiopathic facial hemiatrophy. These observations suggest that facial hemiatrophy can originate (a) in a localized scleroderma in the involved part of the face, (b) in an ipsilateral "sclérodermie en coup de sabre", or (c) in the coexistence of both. There is a close pathological and physiological correlation between idiopathic and sclerodermal facial hemiatrophy. In both forms of facial hemiatrophy the disease involves skeletal muscle tissue systematically. This myopathy is similar to that of progressive scleroderma, far exceeding the limited muscular involvement of localized scleroderma. Sclerodermal facial hemiatrophy can be associated with autoimmune phenomena.

Adipose Tissue↗

PubMedDate not supplied

Scleroderma in South Australia: epidemiological observations of possible pathogenic significance.

BACKGROUND: Scleroderma is an autoimmune disorder of unknown cause. Previous epidemiological studies have suggested some regional clustering and associations with occupations involving exposure to silica dusts and hydrocarbons. AIMS: To determine: (i) prevalence and incidence of scleroderma in South Australia (SA), a state with a stable population living in well-defined urban, rural and industrial regions, (ii) hospital separation rates, (iii) cumulative survival rates, (iv) gender and disease subclassification, (v) geographical residency and occupations, (vi) familial associations and (vii) age at onset versus age-specific incidence rate. METHODS: Creation of the South Australian Scleroderma Register (SASR) to identify demographics and clinical and serological features of all scleroderma patients resident in SA. RESULTS: Scleroderma occurs in South Australia with a point prevalence of 23 per 10(5) and an incidence of approximately 1/15th of this. However, this prevalence is high compared with other regional world studies. Scleroderma is predominantly a female disease, with most patients having the limited form of scleroderma characterized by the presence of the anticentromere antibody and a mean survival of 27.6 years. In contrast, diffuse scleroderma is a less benign disease occurring at an early age of onset and has a mean survival of 9.6 years. Scleroderma occurs throughout SA without regional localization. Weak associations are seen in males, but not females, with occupations involving possible environmental exposure to silica or hydrocarbons. Scleroderma rarely occurs in families. CONCLUSION: No strong genetic or environmental influences were found to account for the relatively common occurrence of scleroderma in SA. The age at onset versus age-specific incidence curve suggests that scleroderma can be considered as a stochastic illness involving a number of random events occurring in a predisposed population. These random events may involve mutations of pivotal somatic genes.

Age of Onset↗

PubMed2003

Homocysteine plasma concentration is related to severity of lung impairment in scleroderma.

OBJECTIVE: To investigate the correlation between plasma concentration of total homocysteine and pulmonary involvement in patients with limited or diffuse scleroderma (systemic sclerosis, SSc). METHODS: Seventy-one patients with scleroderma were divided into 3 groups based on pulmonary involvement: Group A comprised patients without lung involvement (9 cases); Group B patients with lung involvement of mild and moderate stages (44 cases); and Group C patients with lung involvement of severe stage and endstage (18 cases). At the time of evaluation of lung involvement all patients underwent determination of plasma homocysteine concentration. Homocysteine concentration was also measured in 30 healthy controls homogeneous for sex and age. RESULTS: In patients with scleroderma the homocysteine concentration was significantly higher than in controls (11.1 and 6.9 micromol/l, respectively; p < 0.001). We found a significant association between plasma homocysteine concentration and severity of lung involvement that was not modified by correction for age, time from the diagnosis, type of scleroderma pattern, and serum creatinine and folate levels. Homocysteine concentration progressively increases in scleroderma patients with more severe pulmonary involvement. Subjects with high homocysteine concentration (i.e., > or = 75th percentile of homocysteine concentration in patients with scleroderma without lung involvement) were mostly present in the group with the greatest lung involvement. CONCLUSION: High level of homocysteinemia is associated with an increased risk of pulmonary disease in patients with scleroderma. We hypothesize that hyperhomocysteinemia may worsen injury of the endothelium, a key lesion in scleroderma disease, favoring the development of lung involvement. Our data support the hypothesis that homocysteine could be involved in the pathogenetic process of scleroderma pulmonary involvement.

Adult↗

PubMed2006

Scleroderma renal crisis in a patient with anticentromere antibody-positive limited cutaneous systemic sclerosis.

We have encountered a 68-year-old Japanese woman with limited cutaneous systemic sclerosis who developed de novo onset of accelerated hypertension and renal dysfunction; thus we diagnosed scleroderma renal crisis. Anticentromere antibody alone was identified, and not anti-DNA topoisomerase I antibody, anti-RNA polymerase antibodies, anti-Th/To antibodies, or antiribonucleoprotein antibodies, even with use of immunoprecipitation assay. She was successfully treated with angiotensin-converting enzyme inhibitor. This case, scleroderma renal crisis with detection of anticentromere antibody, is thought to be extremely uncommon.

Aged↗

PubMed2001

Localized and systemic scleroderma.

Scleroderma is a broad term encompassing both localized and systemic sclerosis. Localized scleroderma is a cutaneous limited fibrosis that manifests as plaque morphea, generalized morphea, linear scleroderma, and deep morphea. Systemic scleroderma (sclerosis) can manifest as either limited or diffuse disease. Limited systemic sclerosis is typically preceded by Raynaud's phenomenon, involves cutaneous sclerosis distal to the elbows, with gastrointestinal and pulmonary fibrosis, and anticentromere antibody positivity. Diffuse systemic scleroderma is characterized by simultaneous Raynaud's phenomenon, cutaneous skin involvement proximal to the elbow with gastrointestinal, pulmonary, renal and cardiac fibrosis, and positive serology for antitopoisomerase and anti-RNAP III antibodies. This article discusses the classification, epidemiology, pathogenesis, clinical manifestations, treatment, and prognosis of the scleroderma.

Adult↗

PubMed1997

Autoantibodies: diagnostic fingerprints and etiologic perplexities.

Autoantibodies are a hallmark of systemic rheumatic diseases, organ-specific autoimmune diseases and paraneoplastic syndromes. Cell biologists have used autoantibodies as probes to define the structure and function of novel macromolecules and to determine the chromosomal location of their respective genes. The observation that many autoantibodies appear before the clinical expression of disease suggests that they are not epiphenomena. Some autoantibodies are disease-specific markers and are in aid to establishing a diagnosis. Although it has been difficult to link autoantibodies to pathogenesis, they can be used to predict disease progression and outcome. For example, autoantibodies directed against topoisomerase are associated with progression of scleroderma to diffuse skin involvement and severe systemic disease, whereas antibodies to centromere proteins predict a more slowly progressive disease and development of a limited variant of scleroderma. Certain models of autoantibody production hold promise of a clearer understanding of the mechanisms that underlie autoimmunity. Drugs such as procainamide and hydralazine induce the production of chromatin autoantibodies. Exposure to heavy metals (e.g., mercury) is also linked to the development of autoantibodies. The data provide evidence that the autoimmune response is driven by autoantigens, which are multimolecular complexes involved in essential cellular functions.

Animals↗

PubMed1997

Reconstruction of scleroderma of the breast.

Although limited to a single case, our study suggests that patients with localized scleroderma of the breast can incur developmental impairment leading to severe breast deformity. The results of any of several methods of breast reconstruction were not available to us for review because of the rarity of this clinical problem. Tissue expansion allowed our patient to avoid the additional scarring and possible functional loss associated with the transfer of autologous tissues. Its successful completion in this one patient indicates that it can be considered for other patients encountered with similar unusual findings.

Adolescent↗

PubMed2002

Clinical utility of telangiectasia of hands in scleroderma and other rheumatic disorders.

Telangiectasia of the hands were observed in 76% of patients with scleroderma (n = 53) as compared with 12% of patients with other rheumatic disorders (n = 100) and in 13% of healthy subjects (n = 30). In scleroderma, telangiectasia of the hands were commonly multiple (mean number +/- SD = 22.9+/-30.1) with 7.3% being >1 mm in size. They were found in greater numbers in those patients with the limited subtype. The distribution of telangiectasia was observed on all but 4 of 158 sectors of the hand with significant higher numbers on the ventral surface of the digits. Significant associations of telangiectasia of the hands were also observed with numbers of telangiectasia on face and lips (p = 0.001), disease duration (p = 0.002), surface area of digital calcinosis (p = 0.03) and with the presence of the centromere antibody (p = 0.005). Possible associations were observed with prior gastrointestinal bleeds (particularly with telangiectasia >1 mm) and with isolated pulmonary hypertension. No significant correlation was found between number of telangiectasia and with nailfold capillary size or density. In conclusion, multiple telangiectasia of the hands were most commonly observed in patients with the centromere positive, limited subtype of scleroderma of long duration. Their pathogenesis is unknown.

Abnormalities, Multiple↗

PubMed1999

Serum levels of soluble TNF alpha receptor type I and the severity of systemic sclerosis.

Several abnormalities of cytokines have been shown to occur in systemic scleroderma; however their correlation with clinical parameters is controversial. Since serum concentrations of cytokine receptors have been shown to correlate with inflammatory processes, including systemic sclerosis, the aim of our study was to compare serum concentrations of TNF alpha receptor type 1 with the concentrations of soluble intercellular adhesion molecule-1 (sICAM-1), soluble interleukin-2 receptor (sIL-2R) and aminoterminal propeptide of procollagen type III (PIII NP). The findings were correlated with the clinical parameters and antibody patterns, and with the disease severity. Serum samples were studied with the use of enzyme-linked immunosorbent assay (ELISA) or radioimmunoassay (RIA) for sTNF alpha R1, sICAM-1, sIL-2R and PIII NP. The series comprised 36 patients with systemic scleroderma: 13 with diffuse variety and 23 with limited variety, and 7 with Raynaud's disease. Healthy volunteers (n = 25) were chosen from doctors and/or other laboratory staff. Increased levels of sTNF alpha-receptor type 1 were found in 77% of patients with diffuse variety and in only 30% of patients with limited form. Increased serum concentrations in patients with diffuse scleroderma and limited variety were found for sICAM-1 54% and 65%, for sIL-2R 46% and 15%, and for PIIINP 77% and 50%, respectively. There were significant correlations between serum levels of sTNF alpha-receptor type 1 and PIIINP (r = 0.653, p < 0.0001), and sTNF alpha-receptor type 1 and sIL-2R (r = 0.625, p < 0.0001), but not between sTNF alpha-receptor type 1 and sICAM-1 (r = 0.127, p < 0.526). Clinical analysis revealed that serum concentrations of sTNF alpha-RI seem to correlate best with the severity of the disease and, as the only parameter, correlated with lung involvement. The study showed that, in addition to recognized parameters of scleroderma severity (IL-2R, PIIINP), a new important marker appears to be sTNF alpha-receptor type 1.

Adult↗

PubMed2006

Antibodies to fibroblasts in idiopathic and scleroderma-associated pulmonary hypertension.

The aim of the present study was to investigate the presence of anti-fibroblast antibodies in patients with idiopathic or scleroderma-associated pulmonary arterial hypertension (PAH) and healthy controls. PAH was documented by right-heart catheterisation (mean pulmonary artery pressure at rest >25 mmHg). Serum immunoglobulin (Ig)G and IgM reactivities of patients with idiopathic PAH (n = 35), scleroderma-associated PAH (n = 10), diffuse (n = 10) or limited cutaneous (n = 10) scleroderma without PAH and age- and sex-matched healthy individuals (n = 65) were analysed by cell-based ELISA and immunoblotting on normal human fibroblasts. As assessed by ELISA, 14 out of 35 (40%) patients with idiopathic PAH and three out of 10 (30%) patients with scleroderma-associated PAH expressed anti-fibroblast IgG antibodies. IgG from all individuals bound to one major 40-kDa protein band. IgG from patients with idiopathic PAH bound to two 25- and 60-kDa bands with a higher intensity than IgG from other individuals. In conclusion, immunoglobulin G anti-fibroblast antibodies are present in the serum of patients with pulmonary arterial hypertension. Immunoglobulin G from patients with idiopathic pulmonary arterial hypertension or scleroderma-associated pulmonary arterial hypertension express distinct reactivity profiles with fibroblasts antigens, suggesting distinct target antigens.

Adult↗

PubMed1989

The scleroderma neck sign.

The scleroderma neck sign, as described by Barnett, is a visible and palpable tight band over platysma in the hyperextended neck. A recent survey of 76 patients with scleroderma revealed that more than 90% had the scleroderma neck sign. Our study was performed using 15 patients with scleroderma and 30 controls including 3 with primary Raynaud's disease to examine the specificity of the scleroderma neck sign, and to look for a correlation between the presence of the scleroderma neck sign and histological changes of scleroderma in the skin overlying platysma. The scleroderma neck sign was present in 12 of the 15 patients with scleroderma but in none of the 30 controls. It was found both in patients with diffuse (5 out of 5) and limited (7 out of 10) scleroderma. In 10 of the 12 cases where the scleroderma neck sign was positive, there were characteristic histological changes of scleroderma on biopsy of the skin overlying platysma, in 1 there were nondiagnostic abnormalities, and in 1 the biopsy was unsatisfactory. The 3 patients with scleroderma in whom the scleroderma neck sign was absent had either nondiagnostic changes (1) or normal biopsies (2). The 3 patients with Raynaud's disease had normal skin biopsies. The scleroderma neck sign appears to be produced by scleroderma changes in the skin of the neck. In limited or early scleroderma where these changes are otherwise clinically inapparent, the scleroderma neck sign may be diagnostically useful.

Adult↗

PubMed2003

Antiidiotypic antibodies neutralize autoantibodies that inhibit cholinergic neurotransmission.

OBJECTIVE: Functional autoantibodies that inhibit M(3) muscarinic receptor (M3R)-mediated neurotransmission have been reported in patients with Sjögren's syndrome (SS) and in patients with scleroderma. Because of limited reports that intravenous immunoglobulin (IVIG) improves dysautonomia in primary SS, we investigated whether IVIG neutralizes the effect of anti-M3R antibodies on colon smooth muscle contractions, in an in vitro functional assay. METHODS: IgG obtained from patients with primary SS, patients with rheumatoid arthritis and secondary SS, and patients with scleroderma was tested, before and after coincubation with equimolar amounts of IVIG or its F(ab')(2) and Fc fractions, for the ability to inhibit carbachol-evoked colon smooth muscle contractions. In addition, patient IgG was passed through an IVIG F(ab')(2) column, and unretained IgG was tested for functional activity on colon smooth muscle strips. Purified IgG obtained from healthy adults was also examined for a neutralizing effect on anti-M3R antibody activity. RESULTS: Inhibition of colon contractions was mediated by the Fab fraction of patient IgG. Coincubation of IgG from the 3 patient groups with IVIG or its F(ab')(2) fragment neutralized anti-M3R antibody-mediated inhibition of cholinergic smooth muscle contractions. Preabsorption of patient IgG with Sepharose-bound IVIG F(ab')(2) removed the anti-M3R inhibitory activity. In addition, purified IgG from each of 4 healthy adults neutralized the functional autoantibodies. CONCLUSION: Anti-M3R antibody activity does not require receptor crosslinking. Antiidiotypic antibodies present in pooled IgG neutralize patient IgG-mediated inhibition of M3R cholinergic neurotransmission, providing a rationale for IVIG as a treatment of autonomic dysfunction in patients with SS and patients with scleroderma. Furthermore, antiidiotypic antibodies in healthy individuals may prevent the emergence of pathogenic anti-M3R autoantibodies.

Acetylcholine↗

PubMed1992

[Scleroderma and esophageal reflux, surgical monitoring].

Ten patients with documented scleroderma were assessed before and after antireflux operations over a twelve year period. The approach was through the left chest in 9 of 10 patients (7 short Nissen, 1 Collis Nissen, 1 Collis Belsey) and through the abdomen for 1 (vagotomy, antrectomy and Roux en Y). Clinically, 5 of 10 patients still mention episodes of heartburn. Dysphagia, which was present in 8 patients before the operation, has been replaced by a slow emptying impression in five. Endoscopically five patients had a columnar lined esophageal mucosa. Four more were considered to have ulcerative esophagitis and stricture which proved to be also Barrett's esophagus in the postoperative assessment. Three patients had preoperative 24 h pH assessment when the technique became available. Their postoperative control studies revealed persistent acid exposure but to a lesser degree. All ten patients had 24 h pH studies in their postoperative assessment and 5 out of 10 still show abnormal exposure to acid. Esophageal motility studies did not reveal significant changes to the hypomotility of the distal esophageal body and to the decreased tone of the Lower Esophageal Sphincter area. Both radiologically and using esophageal transit scintigrams the esophagus shows atony and poor emptying before the operation. Retention is increased following the creation of an antireflux technique at the esophagogastric junction. The success rate of antireflux operations in scleroderma patients is limited. The ideal procedure to use in this condition remains unclear.

Adult↗

PubMed2002

Scleroderma renal crisis and concurrent isolated pulmonary hypertension in mixed connective tissue disease and overlap syndrome: report of two cases.

We here report on scleroderma renal crisis (SRC) appearing concurrently with isolated pulmonary hypertension (IPHT), that is, pulmonary hypertension without interstitial lung disease with fibrosis, in a patient with mixed connective tissue disease (MCTD) and in one with overlap syndrome or undifferentiated connective tissue disease (UCTD). To the best of our knowledge there are only five previous reports on SRC in MCTD and UCTD. The unexpected appearance of SRC in the setting of concomitant IPHT and limited or absent scleroderma skin changes is discussed.

Acute Kidney Injury↗

PubMed2005

Classification of systemic sclerosis. Visions and reality.

Systemic sclerosis, scleroderma (SSc) is a disabling condition that shortens life expectancy. Disease heterogeneity and difficulties separating SSc from SSc-like conditions make classification an important issue. Limited cutaneous and diffuse cutaneous SSc, with different severity and survival, have been recognized for several years as distinct subsets. Some authors have suggested an intermediate cutaneous form with intermediate survival. This issue remains unsettled, however. The technique of capillaroscopy is helpful as an adjunct diagnostic tool to separate idiopathic Raynaud's phenomenon from SSc. Digitized video-capillaroscopy is developing as a powerful new method to assess individual capillaries over time. Using the simpler techniques of video-capillaroscopy, different patterns have been described and named 'early', 'active', 'late' and 'slow'. The value of nailfold video-capillaroscopy to distinguish different subsets or provide prognostic information for use in daily practice remains to be assessed. The features of CREST (calcinosis, Raynaud's, oesophagus dysmotility, sclerodactyly, telangiectasias) are not confined to single subsets of SSc. There is no convincing evidence of any advantage for distinguishing the limited, intermediate and diffuse forms of SSc rather than only the limited and diffuse forms.

CREST Syndrome↗

PubMed1995

[Antiendothelial antibodies in systemic scleroderma and Raynaud's disease].

Using ELISA on fixed endothelial hybridoma (EAhy.926) cells we investigated the occurrence of antiendothelial antibodies (AEA) in the sera from patients with scleroderma systematica (SS) (n = 70) and Raynaud's disease (RD) (n = 19). The mean IgG and IgA-AEA levels were significantly higher in the SS patients than RD patients (p < 0.001) and controls (p < 0.001). We have detected circulating IgG-AEA in 64.2% of patients with SS, 35.2% of SS sera were positive for IgA-AEA. In patients with RD, the frequency of AEA corresponded to that of the random population sample. We have found a high incidence of Raynaud's phenomenon, myositis, telangiectasia and marked digital ischaemia (digital ischemic pulp ulcers, digital scars, osteolysis and autoamputation) among AEA positive patients with SS. AEA-positive patients were characterized by high extent and severity of Raynaud's phenomenon and higher average nailfold capillary microscopy scores. No correlations were found between AEA and different clinical or laboratory parameters, including the type of scleroderma (diffuse and limited), the presence of anti-Scl-7O and anticentromere antibodies and the clinical features of SS (lungs, kidneys and heart involvement, esophageal dysfunction, calcinosis, Sjogren's syndrome). There was no significant correlation between the AEA level and patient age, extent of skin involvement (skin score). Thus, in SS, AEA is associated with a peripheral vasculopathy and represent a useful marker for the diagnosis of endothelial dysfunction.

Adolescent↗