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 523 records · Page 29Linked to original sources

PubMed1996

Pregnancy outcome in systemic sclerosis.

The outcome of pregnancy in systemic scleroderma (SSc) has been a subject of much interest. In a retrospective analysis of 59 pregnancies of 17 mothers with SSc, the effect of disease on pregnancy outcome was variable. There was no adverse effect on fertility. While there was an apparent decrease in fertility in the limited cutaneous disease, this was not seen in the diffuse variety. There was an decreased incidence of live birth after the onset of disease as compared prior to the onset of disease. The foetal wastage after the onset of disease was contributed equally by spontaneous abortions as well as induced terminations of pregnancies. The presence of anticardiolipin antibodies was not related with adverse outcome. The effect of pregnancy on disease was variable with the disease being stable in five patients, regressing in five and progressing in three. The increased terminations of pregnancy reflects the uncertainities of positive outcome both by parent and attending physicians.

Adolescent↗

PubMed2004

Surgery of the hand in severe systemic sclerosis.

Ten patients with scleroderma and severe hand problems required surgery, and seven were available for follow-up (two died from scleroderma-related complications and one was lost to follow-up). The mean duration of follow-up was 4 (range 1.5-9) years. Thirty-three procedures were carried out, including five metacarpophalangeal joint excisional arthroplasties, 13 proximal interphalangeal joint fusions, ten distal interphalangeal joint fusions, and one thumb interphalangeal joint fusion. The metacarpophalangeal joint excision arthroplasties and proximal interphalangeal joint fusions were performed for the correction of severe fixed "finger-in-palm" deformities. Lesions of cutaneous calcinosis were removed in four patients. Fixation was satisfactory in all cases of interphalangeal joint fusion, with no cases of nonunion. Wound healing was satisfactory in six of seven patients. A second surgical procedure was required in three patients for the removal of tension band wires following interphalangeal fusion. Calcinosis was effectively removed using a high-speed dental burr. The results of hand surgery for systemic sclerosis are reliable, but goals must be limited and patient expectations should be modest.

Adult↗

PubMed1993

The course of skin involvement in systemic sclerosis over three years in a trial of chlorambucil versus placebo.

OBJECTIVE: To describe the course of cutaneous involvement in systemic sclerosis (SSc; scleroderma) over 3 years, in the context of a placebo-controlled drug trial. METHODS: The course of skin tethering (assessed by a semiquantitative skin scoring technique) was documented annually for 3 years in 64 SSc patients with early (< 3 years duration at entry), intermediate (3-8 years duration), or late (> 8 years duration) diffuse or limited SSc. RESULTS: Mean +/- SD entry skin scores were significantly greater in the 33 diffuse SSc patients (13.1 +/- 5.0) than in the 31 limited SSc patients (4.3 +/- 2.1) (P < 0.001). In patients with diffuse SSc, the skin score remained stable for the first 12 months, but had decreased significantly by 24 months (P < 0.022) and 36 months (P < 0.004). In those with limited SSc, the skin score did not change significantly over 3 years. CONCLUSION: Trials of treatments designed to affect skin thickening/tethering should be conducted in patients who have diffuse SSc (of short, intermediate, or long duration) at entry. The best time to study therapies designed to affect skin thickening may be in the first year after entry.

Adult↗

PubMed1996

Silicone granuloma in acral skin in a patient with silicone-gel breast implants and systemic sclerosis.

A 68-year-old white woman was referred to us by her rheumatologist for possible participation in a clinical study of photopheresis for scleroderma. In February 1993, she noticed edema of her distal phalanges, Raynaud's phenomenon in both hands, flu-like symptoms, fatigue, intermittent diarrhea, abdominal pain, tearing in both eyes, dyspnea on exertion, dysphagia, and odynophagia. Bilateral silicone-gel breast implants had been placed 12 years before; 2 months before her present evaluation, they were removed and found to be ruptured. Physical examination revealed edema, limited to the fingers and hands bilaterally, and slight induration of the skin on the dorsum of both hands and distal forearms. The remainder of the physical examination was normal. According to our study protocol, a skin biopsy specimen from the dorsum of the right hand was taken, but all other laboratory investigations were refused. Histopathologic examination revealed multiple clear spaces of varying sizes in the dermis and multinucleated macrophages containing small refractile particles, characteristic of silicone granuloma (Figs. 1 and 2); however, the specimen showed no evidence of scleroderma. X-ray energy dispersive analysis by scanning electron-microscopy confirmed the presence of elemental silicon in the small refractile particles. The patient did not receive any treatment after her diagnosis and shortly thereafter, she was lost to follow-up.

Aged↗

PubMed2006

Esophageal involvement in scleroderma: gastroesophageal reflux, the common problem.

OBJECTIVES: Systemic sclerosis (SSc) is a multisystem disease characterized by abnormalities of small blood vessels, and fibrosis of the skin and internal organs including gastrointestinal tract. This article reviews the esophageal involvement in SSc, emphasizing the gastroesophageal reflux, which is a common problem in SSc patients. METHODS: We conducted a Medline search from 1966 to 2005. The keywords "systemic sclerosis," "esophageal involvement," "gastroesophageal reflux," "esophagitis," and "treatment" were used. RESULTS: The gastrointestinal tract is frequently affected in diffuse and limited disease. Although any part of the gastrointestinal tract can be involved, esophageal disease occurs in nearly all patients with SSc. Common esophageal manifestations in SSc include motility abnormalities and gastroesophageal reflux (GER), Barrett's esophagus, adenocarcinoma, infectious esophagitis, and drug-induced esophagitis. Common complications of GER include esophagitis with erosions and bleeding and stricture formation. Extraesophageal manifestations of GER include mouth ulcers, chronic cough, hoarse voice, sore throat, pharyngitis, laryngospasm, asthma, and recurrent pneumonia. Diagnostic procedures used in the investigation of esophageal involvement include barium esophagram, esophageal manometry, 24-hour ambulatory pH, and endoscopy. Treatment of GER in SSc includes behavioral modification and medical therapy, mainly with proton pump inhibitors. Surgical intervention has a limited role in the management of GER in selected SSc patients. CONCLUSIONS: Esophageal involvement is frequent in SSc patients. Gastroesophageal reflux may cause high morbidity. Careful examination of the patients reveals gastrointestinal abnormalities even in patients without symptoms. Appropriate treatment of esophageal involvement ameliorates symptoms and prevents complications.

Esophagitis↗

PubMed1999

[Scleroderma--systemic sclerosis. Serology, lung function and survival].

Patients with systemic sclerosis (SSc) were studied with regard to the presence of antinuclear antibodies (ANA) and their clinical correlates (n = 230), pulmonary function (n = 176), and mortality and causes of death (n = 344). ANA were found in 85%. Anti-centromere antibodies were found in 34%, anti-Scl-70 antibodies in 13% and anti-U1-RNP antibodies in 6.5%. These serological groups were associated with limited SSc, diffuse SSc, and myositis/arthritis, respectively. The most prevalent finding at first lung function test was isolated reduction of diffusion capacity (47%). Further deterioration of diffusion capacity was related to the presence of anti-centromere antibodies and increased sedimentation rate. The standardized mortality rate (SMR) was 2.9, higher in young patients (SMR = 13) and patients with diffuse SSc (SMR = 4.5).

Adolescent↗

PubMed2006

[Systemic sclerosis].

Systemic sclerosis (SSc; syn. systemic scleroderma) is a rare autoimmune disorder with characteristic cutaneous manifestations. Prevalence in women is fivefold higher than in men. The course of the disease is slowly progressive with a variable degree of internal organ involvement due to fibrosis and obliteration of small vessels. The diffuse form shows more frequent and severe organ manifestations compared to the limited form. Increased mortality is particularly related to a cardiopulmonary involvement leading to a 5-year survival of around 75%. Treatment indications are dependent on the severity of the disease. In the acute state, immunosuppressive agents are needed in case of significant organ involvement. Vasodilative drugs are often used for the symptomatic treatment of Raynaud's phenomenon.

Autoantibodies↗

PubMed2002

Reproductive factors and the risk of scleroderma: an Italian case-control study.

OBJECTIVE: To investigate the association between scleroderma (systemic sclerosis; SSc) and reproductive factors in a hospital-based case-control study conducted at the University Hospital of Verona in Northeastern Italy. METHODS: Forty-six confirmed cases of SSc in women (42 with diffuse SSc and 4 with limited SSc) and 153 female control subjects with orthopedic disorders were recruited at the University Hospital. For each subject, information on age at first pregnancy, number of children and abortions, and use of oral contraceptives was obtained by means of a structured questionnaire, and information on the subject's occupational history and lifestyle factors was also sought. RESULTS: Parous women had a reduced risk of SSc (age-adjusted odds ratio [OR] 0.3, 95% confidence interval [95% CI] 0.1-0.8) compared with nulliparous women. The risk decreased with an increasing number of children: the age-adjusted OR was 0.6 (95% CI 0.2-1.7) for those women who had had 1 child, 0.3 (95% CI 0.1-0.7) for those having had 2 children, and 0.3 (95% CI 0.1-0.8) for those having had 3 or more children. Abortive pregnancies were inversely related to SSc risk: for women who had an abortion, the OR was 0.5 (95% CI 0.2-1.5) compared with women with no history of abortion. The overall history of any pregnancy (abortive or age-adjusted complete) was associated with a reduced risk of SSc (age-adjusted OR 0.3, 95% CI 0.1-0.7). CONCLUSION: Since the incidence of SSc is higher in women and has its peak after childbearing age, it has been hypothesized that immunobiologic modifications during pregnancy may be associated with SSc development. Our findings showing a reduced risk of SSc among parous women seem to exclude the possibility of pregnancy itself as a risk factor for SSc. The role of HLA compatibility between the mother and the fetus, however, should be evaluated in further research.

Abortion, Spontaneous↗

PubMed1996

Anti-topoisomerase-I and clinical findings in systemic sclerosis (scleroderma).

The relationship between anti-topoisomerase-I antibodies and clinical findings was studied in 191 patients with definite systemic sclerosis. This was done by performing ELISA to detect antibodies to recombinant topoisomerase-I. Antibodies to topoisomerase-I were found in 72 patients (37%) with systemic sclerosis, which is a higher percentage than reported in most previous reports on a large unselected population. In 43 patients the presence of antibodies to recombinant topoisomerase-I was confirmed using both the immunodiffusion method and ELISA, with similar results. When classified into diffuse versus limited disease, a significant difference in antibody prevalence was demonstrated (P < 0.005), thus indicating that anti-topoisomerase-I antibody detection with ELISA may assist in early identification of systemic sclerosis subtypes.

Autoantibodies↗

PubMed2005

T cell repertoire in patients with stable scleroderma.

At onset of systemic sclerosis (SSc), T cells have been found to oligoclonally expand in the skin, presumably in response to auto-antigens, but the T cell repertoire has not been evaluated at a later stage. To determine whether a perpetuating immune response contributes to the pathogenesis of stable SSc, the T cell repertoire was analysed in patients with diffuse (d) or limited (l) SSc, and compared to patients with primary Raynaud's phenomenon (RP) or healthy volunteers (Ctrl). The T cell repertoire (total, CD4 or CD8 sorted blood T cells) was analysed by qualitative and quantitative immunoscope (14 BV families analysed) in 11 untreated dSSc and 11 untreated lSSc, 10 RP and 11 Ctrl. To better detect in vivo activated cells, repertoire analysis was also performed on sorted CD4 T cells after in vitro culture with IL-2. In parallel, 6 skin biopsies from SSc patients were analysed. After 7-8 years of disease evolution, SSc patients did not show detectable clonal T cell expansions in the skin, even after tentative expansion from the biopsy with IL-2. Total T cell, sorted CD4 and CD8 T cell repertoires from the blood of patients with SSc did not show significant perturbation as compared to patients with RP and Ctrl. After IL-2 culture for 7 days, blood CD4 T cells from the patients did not preferentially expand as compared to RP and Ctrl. These findings suggest that antigen-driven immune responses may play a lesser role in established SSc than at disease onset.

CD4-Positive T-Lymphocytes↗

PubMed1995

Cellular and molecular mechanisms in the pathogenesis of severe pulmonary hypertension.

Chronic pulmonary hypertension leads to structural alterations of the lung vessels. The pathophysiology of this remodelling process is still poorly understood. Furthermore, the structural damage of the lung vessels limits the clinical success of vasodilator treatment. Given a genetic susceptibility, shear stress and inflammation are the principal pathogenetic factors involved in lung vessel remodelling. In this overview, we compared the lung vascular histology of patients with unexplained pulmonary hypertension, scleroderma, or acquired immune deficiency syndrome (AIDS)-associated pulmonary hypertension. We demonstrate the presence of inflammatory cells (T- and B-lymphocytes and macrophages) in plexiform lesions and discuss the potential role of growth factors (platelet-derived growth factor (PDGF), transforming growth factor-beta (TGF-beta) and vascular endothelial growth factor (VEGF) in pulmonary hypertensive remodelling. Ultimately, we need to develop an in-depth understanding of the key mechanisms of gene expression and signalling pathways, which transduce signals generated from increased chronic shear stress (or from chronic hypoxia) and lead to vascular injury and repair.

Acquired Immunodeficiency Syndrome↗

PubMed1987

CT of interstitial lung disease: a diagnostic approach.

Weibel divided the pulmonary interstitium into three compartments: axial, parenchymal, and peripheral. Heitzman and others have shown that certain interstitial diseases selectively involve these compartments. A model is proposed of the CT appearances in interstitial lung disease based on Weibel's divisions. To assess the validity of this model, the distribution of disease was studied in 44 patients with proven interstitial lung disease. Lymphangitic carcinomatosis (n = 5), lymphoma (n = 2), and sarcoid in two of three patients typically involved the axial compartment. The middle (or parenchymal) compartment was abnormal in advanced stages of many interstitial diseases, particularly granulomatous diseases and drug toxicity (n = 2) in this series, but diffuse changes were seen early in extrinsic allergic alveolitis (n = 4). The peripheral compartment was predominantly affected in idiopathic pulmonary fibrosis (n = 9), rheumatoid lung (n = 3), and scleroderma (n = 1). Prominent nodularity was a feature of lymphangitic carcinomatosis and of the granulomatous diseases, silicosis and sarcoid. The presence or absence of nodules coupled with CT demonstration of differential involvement of the three interstitial compartments is useful in limiting diagnostic possibilities in interstitial disease. By more accurately showing disease distribution, a more reasonable approach to lung biopsy options may be developed.

Humans↗

PubMed2006

Natural history of mild-moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma.

OBJECTIVE: To determine risk factors for developing pulmonary hypertension (PH) in patients with scleroderma (SSc, systemic sclerosis). METHODS: We used a cohort of 1136 SSc patients using severe PH as the primary outcome in a natural history study. RESULTS: Among 361 individuals with no initial echocardiographic PH, 92 (26.0%) developed mild-moderate PH and 48 (13.6%) severe PH. Patients developing severe PH had lower initial DLCO (48.8% of predicted) than those who did not develop PH (56.8% of predicted). Patients with mild-moderate PH had a 17% probability of progressing to severe PH, and 15.6% probability of regressing to no PH. Individuals with limited disease, mild-moderate PH, and age > or= 47 years at diagnosis had a 27.3% probability of developing severe PH, compared to 8.5% in individuals with diffuse disease, no evidence of PH, and age < 47 years at diagnosis. Longitudinal regression models estimated that individuals with limited disease, mild-moderate PH, and DLCO < 50% predicted had an age-adjusted odds ratio of 8.6 of developing severe PH within 2 years compared to individuals without these risk factors. CONCLUSION: Development of severe PH is uncommon in certain subgroups of SSc patients. Risk factors for progression of PH include older age, limited skin disease, and elevated pulmonary artery pressures at the time of initial evaluation.

Adult↗

PubMed1991

Immunological markers of the subsets of systemic scleroderma and its overlap.

The studies performed repeatedly in 298 cases of systemic scleroderma (SSc) disclosed high prevalence of antinuclear antibody (in about 95% of the cases) with the use of HEp-2 cells as the substrate for indirect immunofluorescence method. Anticentromere antibody (ACA) was found in 25% of acrosclerosis cases, but in 50% of patients with cutaneous involvement limited to the digits, i.e. almost without cutaneous indurations. The visceral involvement was comparable in cases positive and negative for ACA, and a follow-up of these patients showed that the course, although protracted, was not always mild as previously believed. Scl 70 antibody proved to be a most characteristic marker of SSc, appearing in about 87% of diffuse variety and in above 50% of acrosclerosis cases. The most important finding was the detection of the coexistence of ACA and Scl 70 antibody in 10 of 180 cases studied by three methods: indirect immunofluorescence, double immunodiffusion, (for Scl 70) and immunoblotting with recombinant kinetochore antigen for ACA and recombinant Topoisomerase I antigen for Scl 70 antibody. These antibodies, believed to be mutually exclusive, are most often missed due to masking effect on HEp-2 cells by the stronger antibody, usually Scl 70. Its detection proved to be possible by evaluation of different serum dilutions and several cell mitoses, and confirmed by immunoblotting with recombinant antigens. Of practical significance are the clinical correlations between Pm-Scl antibodies, showing homogeneous nucleolar staining on HEp-2 cells, and overlap or atypical SSc cases with concomitant muscle involvement. This antibody was found to have a surprisingly high association with atypical SSc or scleromyositis of children.

Antibodies, Antinuclear↗

PubMed1985

Association of anti-centromere and anti-Scl 70 antibodies in scleroderma. Report of two cases.

In testing for antinuclear antibodies the sera of 121 patients affected with scleroderma, we found in 2 of them the concurrent presence of anti-centromere and anti-Scl 70 antibodies. Since the association of these antibodies has never been reported, to the best of our knowledge, we report the clinical and serological features of the 2 patients. Both subjects were women with an incomplete CREST syndrome (RST), a relatively limited involvement of the skin, a mild pulmonary fibrosis without apparent signs of other visceral involvement. On the other hand, the clinical course of the disease was different in the 2 cases. One patient had a longer disease duration and up to date the clinical features appear to be unchanged; the other had a shorter disease duration, when suddenly she died of cardiac arrest.

Antibodies, Antinuclear↗

PubMed1980

Chronic graft-versus-host syndrome in man. A long-term clinicopathologic study of 20 Seattle patients.

This study of chronic graft-versus-host disease (GVHD) describes the clinical, pathologic and laboratory features, and the causes of morbidity and mortality in 20 patients who received allogeneic marrow transplants from HLA identical sibling donors. Chronic GVHD is a pleiotrophic syndrome with variability in the time of onset, organ systems involved and rate of progression. The clinical-pathologic features resemble an overlap of several collagen vascular diseases with frequent involvement of the skin, liver, eyes, mouth, upper respiratory tract, esophagus and less frequent involvement of the serosal surfaces, lower gastrointestinal tract and skeletal muscles. Major causes of morbidity are scleroderma with contractures and ulceration, dry eyes and mouth, pulmonary insufficiency and wasting. Chronic GVHD has features of immune dysregulation with elevated levels of eosinophils, circulating autoantibodies, hypergammaglobulinemia and plasmacytosis of viscera and lymph nodes. In this study, three patients had limited chronic GVHD with relatively favorable prognosis characterized by localized skin involvement and/or hepatic disease without chronic aggressive histology. Most patients, however, had extensive disease with a progressive course. Survival was largely determined by the presence or absence of serious recurrent bacterial infections. The over-all severity of disease was best assessed by using the Karnofsky performance rating.

Adolescent↗

PubMed2004

Efficacy and safety of intravenous immunoglobulin for immune-mediated skin disease: current view.

Intravenous immunoglobulins (IVIgs) exert a variety of immunomodulating activities and are, therefore, increasingly being used for the treatment of immune-mediated as well as autoimmune diseases. There is also accumulating evidence that high-dose IVIg (hdIVIg) is highly efficacious in the treatment of skin diseases, despite the lack of evidence from randomized, double-blind, placebo-controlled trials. A major advantage of hdIVIg in comparison with other commonly used immunomodulating therapeutic strategies is the excellent safety profile. Accordingly, IVIgs have been used successfully for the treatment of bullous autoimmune diseases such as pemphigus and bullous pemphigoid, dermatomyositis, scleroderma, cutaneous lupus erythematosus, toxic epidermal necrolysis, and erythema exudativum multiforme. In most cases, hdIVIg is effective only in combination with other immunomodulating strategies and allows for the reduction of adjuvants. Adverse effects of hdIVIg are generally mild and self-limiting. These include headache, myalgia, flush, fever, nausea or vomiting, chills, lower backache, changes in blood pressure, and tachycardia. To avoid infusion-related rigors, headaches, and other adverse events, pre-treatment with analgesics, NSAIDs, antihistamines, or low-dose intravenous corticosteroids may be beneficial. Controlled, double-blind, long-term clinical trials and a better understanding of the complex immunomodulating mechanism of IVIg are required to ultimately optimize dose, frequency, duration, and mode of IVIg administration.

Autoimmune Diseases↗

PubMed1995

Elevated procollagen type I carboxyterminal propeptide production in cultured scleroderma fibroblasts.

BACKGROUND: We recently reported that the serum concentration of procollagen type I carboxyterminal propeptide (P1CP) in patients with systemic sclereosis (SSc) was elevated. In the present study, we investigated collagen metabolism in in vitro cultured scleroderma fibroblasts by measuring P1CP levels in the culture medium. METHODS AND RESULTS: Spontaneous P1CP production was 4.2 times higher in fibroblast cultures from patients with SSc (n = 11) than in those from healthy controls (n = 10). P1CP production in fibroblasts derived from diffuse cutaneous SSc patients was significantly greater than that from limited cutaneous SSc patients. The serum P1CP level in SSc patients was correlated with the P1CP production of cultured fibroblasts (r = 0.815, p < 0.005). Transforming growth factor beta increased P1CP production, and gamma-interferon decreased P1CP production similarly in both SSc and normal fibroblasts. In contrast, histamine dihydrochloride increased P1CP production only in SSc fibroblasts but not in controls. CONCLUSION: These findings suggest that P1CP production in SSc fibroblasts is relevant to in vivo collagen synthesis in SSc patients.

Adolescent↗