PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “Scleroderma, Systemic”

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 1,351 records · Page 75Linked to original sources
PubMed1985

Anticentromere antibody and immunoglobulin allotypes in scleroderma.

Fifty-five unrelated whites with disorders in the scleroderma spectrum who had both antinuclear antibodies and Raynaud's phenomenon (RP) were studied. Of the 22 patients with anticentromere antibody (ACA), three had diffuse scleroderma; 16 had the complete or incomplete syndrome of calcinosis, RP, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST syndrome); and three had RP only. Thirty-three patients with other nuclear patterns all had systemic scleroderma (28 diffuse scleroderma, five CREST syndrome). Patients with ACA had less organ system involvement, and lower frequencies of anemia and elevation of sedimentation rate than ACA-negative patients, but these differences were not statistically significant. They also had fewer manifestations of CREST syndrome. All 55 patients were studied for the Gm and Km allotypic markers. No association was found between Gm or Km allotypic markers and scleroderma or between the allotypic markers and the presence of ACA.

Adolescent↗
PubMed1993

Progressive systemic sclerosis sine scleroderma in a child presenting as nocturnal seizures and Raynaud's phenomenon.

Progressive systemic sclerosis sine scleroderma, as well as neurological manifestations of progressive systemic sclerosis are rare in adult-onset cases. Neither have been reported in children with progressive systemic sclerosis, either separately or together. We describe a six-year-old girl with nocturnal seizures and Raynaud's phenomenon of three years' duration. She died of cardiopulmonary sclerosis without ever fitting the required criteria of systemic sclerosis. Nailfold capillaroscopy revealed the specific "scleroderma-pattern" and provided the only clue for a diagnosis of progressive systemic sclerosis, confirmed eventually by skin biopsy.

Child↗
PubMed2006

Systemic and localized scleroderma in children: current and future treatment options.

Scleroderma is a group of rare and complex diseases with varied clinical manifestations. The most obvious manifestation of the diseases is skin hardening and sclerosis. Scleroderma can be divided into two main subgroups: systemic and localized. The systemic form, also known as systemic sclerosis, involves diffuse skin involvement and potentially severe visceral involvement. Localized scleroderma on the other hand is more common in children and usually confined to a specific region of the body with no internal organ involvement. The juvenile forms of systemic sclerosis and localized scleroderma are important conditions in children because of the clinical severity and substantial mortality of systemic scleroderma and the major growth defects associated with childhood-onset localized disease even if the active disease itself is self-limited. The pathogenic pathways of the various forms of scleroderma are only partially defined, but the main defect in scleroderma is abnormal collagen deposition leading to eventual fibrosis in the skin as well as multiple organ systems such as the heart and lungs in juvenile systemic sclerosis. Therapeutics are divided into three main subgroups for systemic sclerosis: antifibrotics, anti-inflammatories, and vasodilators. For localized disease, anti-inflammatories, vitamin D analogs, and UV irradiation have been investigated. However, the infrequency of scleroderma in the pediatric population plus the fact that this disease is very often self-limiting makes randomized controlled trials very difficult. It is for this reason that most data on treatment modalities for this disease have been extrapolated from studies in adult patients. There is no one therapy for systemic sclerosis or localized scleroderma that has proven to be very effective or significantly disease modifying. However, current therapeutic strategies must be initiated early in the disease course for maximum beneficial clinical effects. New interventions such as autologous stem cell transplant and cytokine-directed therapies are under investigation as potential treatments for this complex disease.

Child↗
PubMedDate not supplied

Circulatory system in children with localized scleroderma.

The circulatory system was studied in 43 children (ages 3-18 years, mean 10.6 years; 32 girls, 11 boys) suffering from localized cutaneous forms of scleroderma. The following studies were undertaken: general pediatric examination, cardiologic examination including routine electrocardiogram (ECG), 24-h Holter ECG monitoring, echocardiography plus Doppler study, and a treadmill exercise test. Three children found to have congenital heart malformations were excluded from the study. Children with localized scleroderma were often lighter and shorter than their appropriate controls. The most common ECG abnormality was incomplete right bundle branch block, but other ECG and Holter abnormalities were found as well. Abnormal echocardiographic results were obtained in 16 cases. The abnormalities concerned valvar function (in all heart valves but predominantly in the mitral valve). It was found that the children suffering from scleroderma had different indices than controls for left ventricular mass index, mitral valve function and left ventricular filling. During the exercise test (conducted according to Bruce's protocol) we found no difference between patients and controls.

Adolescent↗
PubMed2002

Systemic sclerosis sine scleroderma: is it always the same disease? Report of three patients and discussion.

The recent description of a large cohort of patients with the diagnosis of systemic sclerosis sine scleroderma (ssSSc) provided significant progress in our understanding of this entity. The prognosis of patients with ssSSc is, however, very variable, from benign in most cases to rapidly disabling in others. By reporting three new cases and analyzing previously published data, we discuss possible subsets and variants of the disease form.

Adolescent↗
PubMed1980

Diagnostic potential of in vivo capillary microscopy in scleroderma and related disorders.

The prevalence of scleroderma-type capillary abnormalities, as observed by in vivo microscopy, was determined in 173 patients from three rheumatic disease centers. The patients had a variety of connective tissue diseases: scleroderma (systemic sclerosis) 50; systemic lupus erythematosus 60; mixed connective disease 26; Raynaud's disease 11; other rheumatic disorders 26. Enlarged and deformed capillary loops surrounded by relatively avascular areas, most prominently in the nail-folds, were found in 82% of patients with scleroderma and in 54% with mixed connective tissue disease. The rarity of these abnormalities in systemic lupus erythematosus (2%) despite the presence of Raynaud's phenomenon suggests that they are not an expression of the Raynaud's phenomenon frequently associated with scleroderma and mixed connective tissue disease. The single patient with Raynaud's disease and sclerodermatype capillary changes subsequently developed scleroderma.

Adolescent↗
PubMed2002

Clinical trials for pediatric scleroderma.

Progressive systemic sclerosis (PSS), or scleroderma, is a rare disease in the pediatric population. Many children with PSS have significant involvement of their internal organs, which leads to decreased survival. Because of the infrequency of the condition and delayed diagnosis, there are no large studies to evaluate therapy for PSS in children. Treatment is controversial in the adult literature, and its applicability to children is unclear. Only through collaborative efforts will researchers be able to clearly delineate the etiopathogenesis of PSS, and gather information from multicenter studies to ultimately provide appropriate and effective care for children with PSS.

Antirheumatic Agents↗
PubMedDate not supplied

Peripheral neuropathy in scleroderma.

Nervous system involvement is rare in progressive systemic sclerosis (PSS). We present a clinical pathological and immunological study of two patients with peripheral sensory motor neuropathy and PSS. In both, the sural nerve biopsies showed axonal degeneration with increased endoneurial connective tissue. There were also clusters of myelinated fibres indicating axonal regeneration. Only mild microangiopathic changes were evident in the endo, peri and epineurial vessels. By Western immunoblots, patients' sera contained a band of reactivity to a protein from peripheral nerve identified as collagen type I. Primary involvement of the peripheral nerves during PSS is very unusual. Abnormal production of collagen tissue and presence of microvascular disease are considered to be two possible causes of neuropathy. We think that our results suggest the important role of the connective tissue proliferation in the pathogenesis of PSS neuropathy.

Adult↗
PubMed1996

The epidemiology of scleroderma among women: assessment of risk from exposure to silicone and silica.

OBJECTIVE: To investigate the relationship between exposure to silicone (including breast implants) and silica and the development of scleroderma (systemic sclerosis, SSc) among women. METHODS: A population based case-control study was conducted among women in Michigan. 274 confirmed cases of SSc diagnosed between 1985 and 1991 were identified by contacting rheumatologists, hospitals, and a scleroderma support group. These cases and 1184 controls were interviewed by telephone to ascertain past exposures to silicone or silica. RESULTS: Silicone in the form of breast implants was not associated with significantly increased risk of SSc (adjusted odds ratio, 1.30; 95% confidence interval, 0.27 to 6.23). Among 20 other potential silicone exposure surveyed, self-reported exposure to silicone based glues, sealants, and caulks, manufacture or repair of windows or windshields, repairing or frequently using photocopy machines, consumption of simethicone-containing antacids, and implanted medication delivery pumps were significantly associated with SSc. However, blinded assessment of all job and hobby descriptions in terms of their potential for silicone exposure failed to support the first 3 associations, antacid consumption may have been confounded by esophageal dysmotility before the diagnosis of SSc, and other silicone containing device categories (pacemakers, central nervous system shunts, other shunts and catheters) were not significantly associated with SSc. Surgically implanted metallic fixation devices were associated with significantly reduced risk for SSc. No association was detected between SSc and silica dust exposure. CONCLUSION: Consistent with other studies, we found no increased risk of SSc among women with silicone breast implants, equivocal evidence of risk from other silicone exposures, and no evidence of risk from silica exposure.

Adolescent↗
PubMed2003

The clinical relevance of autoantibodies in scleroderma.

Scleroderma (systemic sclerosis) is associated with several autoantibodies, each of which is useful in the diagnosis of affected patients and in determining their prognosis. Anti-centromere antibodies (ACA) and anti-Scl-70 antibodies are very useful in distinguishing patients with systemic sclerosis (SSc) from healthy controls, from patients with other connective tissue disease, and from unaffected family members. Whereas ACA often predict a limited skin involvement and the absence of pulmonary involvement, the presence of anti-Scl-70 antibodies increases the risk for diffuse skin involvement and scleroderma lung disease. Anti-fibrillarin autoantibodies (which share significant serologic overlap with anti-U3-ribonucleoprotein antibodies) and anti-RNA-polymerase autoantibodies occur less frequently and are also predictive of diffuse skin involvement and systemic disease. Anti-Th/To and PM-Scl, in contrast, are associated with limited skin disease, but anti-Th/To might be a marker for the development of pulmonary hypertension. Other autoantibodies against extractable nuclear antigens have less specificity for SSc, including anti-Ro, which is a risk factor for sicca symptoms in patients with SSc, and anti-U1-ribonucleoprotein, which in high titer is seen in patients with SSc/systemic lupus erythematosus/polymyositis overlap syndromes. Limited reports of other autoantibodies (anti-Ku, antiphospholipid) have not established them as being clinically useful in following patients with SSc.

Antibodies, Antinuclear↗
PubMed2005

[Acral scleroderma presenting simultaneously with small-cell bronchial carcinoma: a paraneoplastic disease?].

The possible causal connection between systemic scleroderma and malignant neoplasms is a controversial issue. In the case of dermatomyositis, a malignant tumor is viewed as a possible trigger of the collagen disease; in contrast, scleroderma is suspected of causing tumors because of the long-term tissue fibrosis. A 68-year-old woman presented with acral scleroderma but already had metastatic bronchial carcinoma without evidence for previous pulmonary fibrosis. The rapid spread of the tumor and the sudden appearance of scleroderma suggest that in this case scleroderma could represent a paraneoplastic syndrome. Attention is directed to several growth factors and cytokines. On the one hand they play a part in the neoplastic transformation of the host cells and on the other hand they can change fibroblasts into a profibrotic phenotype and induce fibrosis. Impressive in this case is the fact that acral scleroderma definitely developed after the malignant tumor but before treatment, so that both fibrosis of the lung and side effects of chemotherapy and radiation can be excluded as triggers.

Bronchial Neoplasms↗
PubMed2003

Silica exposure and systemic vasculitis.

Work in Department of Energy (DOE) facilities has exposed workers to multiple toxic agents leading to acute and chronic diseases. Many exposures were common to numerous work sites. Exposure to crystalline silica was primarily restricted to a few facilities. I present the case of a 63-year-old male who worked in DOE facilities for 30 years as a weapons testing technician. In addition to silica, other workplace exposures included beryllium, various solvents and heavy metals, depleted uranium, and ionizing radiation. In 1989 a painful macular skin lesion was biopsied and diagnosed as leukocytoclastic vasculitis. By 1992 he developed gross hematuria and dyspnea. Blood laboratory results revealed a serum creatinine concentration of 2.1 mg/dL, ethrythrocyte sedimentation rate of 61 mm/hr, negative cANCA (antineutrophil cytoplasmic antibody cytoplasmic pattern), positive pANCA (ANCA perinuclear pattern), and antiglomerular basement membrane negative. Renal biopsy showed proliferative (crescentric) and necrotizing glomerulonephritis. The patient's diagnoses included microscopic polyangiitis, systemic necrotizing vasculitis, leukocytoclastic vasculitis, and glomerulonephritis. Environmental triggers are thought to play a role in the development of an idiopathic expression of systemic autoimmune disease. Crystalline silica exposure has been linked to rheumatoid arthritis, scleroderma, systemic lupus erythematosus, rapidly progressive glomerulonephritis and some of the small vessel vasculitides. DOE workers are currently able to apply for compensation under the federal Energy Employees Occupational Illness Compensation Program (EEOICP). However, the only diseases covered by EEOICP are cancers related to radiation exposure, chronic beryllium disease, and chronic silicosis.

Arthralgia↗
PubMed2000

Recent advances in phototherapy.

This synopsis reviews recent developments in dermatological phototherapy. UVA1 phototherapy (340-400 nm) is effective in the treatment of inflammatory skin diseases such as acutely exacerbated atopic dermatitis, localized scleroderma, urticaria pigmentosa and disseminated granuloma annulare. Narrowband UVB radiation (311-313 nm) is used successfully as monotherapy or combined with dithranol, oral retinoids or 8-MOP in psoriasis, atopic dermatitis (AD) or photosensitivity disorders such as polymorphic light eruption. Bath water delivery of 8-methoxypsoralen and subsequent UVA-irradiation (PUVA bath therapy) for the treatment of psoriasis as well as for mycosis fungoides, localized scleroderma, urticaria pigmentosa or lichen planus is an effective alternative to its systemic application. The combination of salt water brine baths in different concentrations and subsequent UVA/B irradiation is used increasingly for the treatment of psoriasis or AD. Extracorporeal photopheresis (ECP) has proven to be a very effective treatment modality for cutaneous T cell lymphoma, chronic graft-versus-host disease and certain autoimmune diseases such as systemic scleroderma or pemphigus. However, despite the documented benefits of these new treatment modalities, little data exist as of yet on potential long-term side effects, thus the indications for these therapies should be considered carefully and patients should be followed up at regular intervals.

Female↗
PubMedDate not supplied

[Telangiectasias in progressive systemic sclerosis (generalized scleroderma). Observation on 120 cases].

120 patients with progressive systemic sclerosis (PSS) were studied and subdivided into five groups according to the PSS classification of Giordano et al. ("acute diffuse scleroderma", "intermediate syndrome", "acrosclerosis sensu stricto", "sclerodactylia" and "sclerosis sine scleroderma"). In all five subgroups telangiectasias incidence was high (from 75% to 100%). The "ramosus" and "telangiectatic mats" types occur more frequently than other forms of telangiectasias. The former particularly involves the face, neck and chest; whereas the latter more usually involves the upper extremities and is the only type which appears at the lips. Cuticular telangiectasia is a third type, as important as the others but less frequent. The incidence of telangiectasias is related to disease duration. Similar telangiectasias have been observed in rarer patients with other connective tissue diseases (SLE, RA, dermatomyositis and undifferentiated connective tissue diseases).

Arthritis, Rheumatoid↗
PubMed1985

Cardiac involvement in mixed connective tissue disease. A fatal case of scleroderma combined with systemic lupus erythematosus.

A 27-year-old black woman with cardiac failure, angina pectoris and Raynaud's syndrome is presented. Skin biopsy and barium studies established the diagnosis of scleroderma (progressive systemic sclerosis (PSS)). Systemic lupus erythematosus (SLE) was strongly suggested by the results of immunological studies and increasing severity of renal failure. Because of the possibility of a cardiomyopathy, cardiac catheterization, selective coronary angiography and right ventricular endomyocardial biopsy were carried out but failed to show any histological features of either SLE or PSS. The patient went into progressive renal failure despite immunosuppressive therapy and plasmapheresis and died; consent for autopsy was refused. A final diagnosis of mixed connective tissue disease (MCTD) was made. The salient features of cardiac involvement in SLE, PSS and MCTD are outlined.

Adult↗