PubMed Health⌕ Search

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 379 records · Page 21Linked to original sources
PubMed1989

[Scleroderma and thyroid diseases].

The authors report a prospective study of the thyroid function of 18 consecutive patients with systemic scleroderma and 7 patients with morphea. A history was taken and patients were examined for thyroid disease. The patients were tested for free thyroxin, TSH, cholesterolemia, circulating TeBG, anti-microsomial and anti-thyroglobulin antibodies and had a TRH test. The incidence of thyroid diseases was correlated with an age and sex-matched control population consisting of 2,534 subjects of the Loire department. None of the patients with morphea had a history of thyroid pathology and the thyroid screenings were within normal limits. A familial history of thyroid disease was found in 7 out of 18 patients with systemic scleroderma, and 8 out of 18 patients had a thyreopathy (one Hashimoto's disease, one Graves' disease, one toxic adenoma, one hypothyroidism, two euthyroid goiters, two nodular thyroids). The TRH test only revealed a toxic adenoma. A thyroid disease preceded systemic scleroderma in 5 out of 18 cases with delays ranging from 1 to 38 years. In 3 cases, the thyroid disease occurred within two years after the onset of systemic scleroderma. The prevalence of thyroid disease was significantly higher than in a comparable population sample of subjects from the same geographic region (p less than 0.01). The authors review the literature and discuss the physiopathologic relationships between the two clinical entities. The authors suggest to perform a thyroid screening (ultrasensitive TSH assay) on all patients with systemic sclerosis and especially on those whose clinical follow-up is atypical and on those with a familial and/or personal history of thyroid disease.

Aged↗
PubMedDate not supplied

Development of a device for removing a partial denture in a patient with scleroderma.

Scleroderma is a chronic disease that has been associated with immune dysfunction. One of the oral manifestations is microsomia, a result of collagen deposition in the perioral issues. The complexity of treating these patients includes limited mouth opening ability, and difficulty inserting and removing dentures due to finger deformity. This article will describe an appliance specially designed especially for scleroderma patients, which facilitates treatment of the patient with removable partial dentures (RPD).

Adult↗
PubMed2006

Unilateral generalized morphea is a rare variant of localized scleroderma.

Localized scleroderma (LS) is a rare connective tissue disorder generally involving the entire dermis and usually limited to the subcutaneous tissue. However, it may progress to large indurated plaques, growth retardation, muscle atrophy, and even to flexion deformities or poorly healing ulcerations. LS has been classified as plaque, generalized, bullous, linear, and deep forms exhibiting different clinical subtypes. Recently, an unusual case of unilateral generalized morphea (UGM) in childhood extending from the middle dermis to the subcutaneous fat tissue has been reported. We here describe four young patients exhibiting a similar subtype of LS. All patients demonstrated a prominent unilateral skin involvement starting in childhood or adolescence. Histology revealed prominent accentuation of intradermal involvement. Except for positive anti-nuclear antibodies, no specific antibody pattern could be observed. In presenting these clinically homogenous cases we hereby introduce UGM as an extreme variant of the linear form of LS in childhood. As the onset of UGM usually occurs in pediatric patients, pediatricians should be cognizant of the presentation of this uncommon condition. Treatment with combined low-dose methotrexate and pulsed high-dose corticosteroid therapy might represent a promising treatment option for UGM.

Adolescent↗
PubMed2005

Severe refractory fingertip ulcerations in a patient with scleroderma: successful treatment with sildenafil.

Systemic sclerosis (scleroderma) is a multisystem fibrotic disease that commonly manifests with severe Raynaud's phenomenon and slow-healing cutaneous ulcerations. Reduced nitric oxide levels have been proposed to play a role in the pathogenesis of vascular disease in scleroderma, and therefore sildenafil (which increases nitric oxide levels) is an attractive therapeutic prospect. We describe a patient with limited cutaneous systemic sclerosis who presented with severe nonhealing finger ulcerations despite conventional management, who showed marked improvement with oral sildenafil.

Administration, Oral↗
PubMed1975

Surgical management of strictures of the lower thoracic esophagus.

An obstructing lesion of the lower thoracic esophagus should be evaluated carefully and appropriate surgical therapy planned to correct the abnormal physiology. Gastroesophageal reflux is the most frequent cause of esophageal stricture and usually can be managed effectively by dilatation of the esophagus, restoration of a competent lower esophageal sphincter, and repair of an associated hiatal hernia. Collis gastroplasty and a Belsey herniorrhaphy are useful when the esophagus is excessively shortened. Firm, fixed esophageal strictures may be treated by the Thal procedure accompanied by Nissen's fundoplication or by resection of the stricture and interposition of a colon graft or an achlorhydric tube. The management of other benign strictures secondary to scleroderma, ingestion of caustic substances, or benign neoplasms must be individualized. Most benign strictures may be cared for by dilatation; however, firm, fixed strictures should be resected. Wide surgical resection is indicated for primary malignant lesions of the lower thoracic esophagus that are localized or have limited lymph node metastasis.

Esophageal Neoplasms↗
PubMed2002

Recognition of Granzyme B-generated autoantigen fragments in scleroderma patients with ischemic digital loss.

OBJECTIVE: To examine whether autoantibodies recognizing granzyme B (GB)-cleaved autoantigens are associated with ischemic digital loss (IDL) in limited systemic sclerosis (SSc). METHODS: Fifteen of 19 patients with limited SSc and IDL were matched by age, sex, race, and duration of disease to controls with limited SSc but without IDL. The sera were used to immunoblot HeLa cell lysates and chromosome preparations that had been incubated in vitro in the absence or presence of GB. Anticentromere antibodies (ACAs) were assayed by immunofluorescence and immunoprecipitation of in vitro-translated centromere proteins (CENP-B and CENP-C). Immunoprecipitation of GB-cleaved CENPs was also performed. RESULTS: GB-cleaved autoantigens were immunoblotted by 16 of 19 IDL sera (84.2%) compared with 6 of 15 non-IDL sera (40.0%) (odds ratio 8.0, 95% confidence interval 1.6-40.0). This association persisted after adjustment for ACA status. Furthermore, the presence of antibodies to centromere proteins as well as to GB-cleaved antigens was highly specific for IDL, occurring in 12 of 19 IDL patients (63.2%) and in none of 15 controls (P < 0.0001). An identical 60-kd GB-generated fragment was recognized by 5 of 16 IDL sera (31.3%) and was demonstrated to arise through GB-mediated cleavage of CENP-C. GB-cleaved CENP-C fragments were recognized preferentially over the intact CENP-C molecule by antibodies from patients with IDL. CONCLUSION: The striking recognition of GB-generated autoantigen fragments by sera from patients with limited SSc and IDL constitutes the first in vivo evidence that antibodies against GB-generated centromeric peptide fragments identify a distinct clinical subset.

Autoantibodies↗
PubMed2000

Validity of HAMIS: a test of hand mobility in scleroderma.

OBJECTIVE: Hand Mobility in Scleroderma (HAMIS) is a hand function test for persons who have systemic sclerosis (scleroderma). The purpose of HAMIS is to obtain an estimation of the hand mobility that is precise enough to detect limitation of motion at the same time as it indicates the ability to use the hand in daily occupations. The aim of this study was to test psychometric properties of the HAMIS, and the following aspects of HAMIS were examined: 1) the applicability of HAMIS, 2) concurrent validity, and 3) discriminating ability. METHODS: Forty-five patients with scleroderma were assessed for range of motion (ROM), HAMIS, and skin thickness. In addition, 15 healthy individuals completed HAMIS. RESULTS: The applicability of HAMIS was good for items assessing finger and thumb mobility and moderate for items assessing mobility of the wrist and the forearm. The relationships of HAMIS to ROM and skin score were statistically significant for all items except for pronation and supination of the forearm. There were also statistically significant differences between the patients and the healthy individuals for all items except these two. CONCLUSION: HAMIS has a demonstrated concurrent validity compared with ROM and skin score, and it showed a good ability to discriminate between healthy individuals and persons with scleroderma, although a lack of variation in the items measuring pronation and supination inferred worse psychometric properties for these two items.

Activities of Daily Living↗
PubMed2003

Differential responses of three fungal species to environmental factors and their role in the mycorrhization of Pinus radiata D. Don.

Three ectomycorrhizal (ECM) isolates of Rhizopogon luteolus, R. roseolus and Scleroderma citrinum were found to differ markedly in their in vitro tolerance to adverse conditions limiting fungal growth, i.e. water availability, pH and heavy metal pollution. S. citrinum was the most sensitive, R. luteolus intermediate and R. roseolus the most tolerant species. Pinus radiata D. Don seedlings were inoculated in the laboratory and in a containerised seedling nursery with spore suspensions of the three ECM species. Colonisation percentage was considerably lower under nursery conditions, probably due to competition by native fungi. The effects of nursery ECM inoculation on seedling growth depended on the fungal species. Only R. roseolus-colonised plants showed a significantly higher shoot growth than non-mycorrhizal plants. All three fungi induced significantly higher root dry weights relative to control plants. Despite the low mycorrhizal colonisation, mycorrhization with all three species improved the physiological status of nursery-grown seedlings, e.g. enhanced root enzyme activity, shoot nutrient and pigment content, net photosynthesis rate and water use efficiency. Of the three fungal species, R. roseolus was the most effective; this species was also the most adaptable and showed the greatest range of tolerance to adverse environmental conditions in pure culture. It is, therefore, proposed as a promising fungal species for ECM inoculation of P. radiata in the nursery.

Basidiomycota↗
PubMed2003

Bronchiolitis obliterans with organizing pneumonia associated with scleroderma and scleroderma spectrum diseases.

Systemic sclerosis (SSc) is an autoimmune disorder with a high mortality rate from pulmonary manifestations, such as interstitial lung disease and pulmonary hypertension, seen at autopsy in 90% and 75% of patients, respectively. Bronchiolitis obliterans with organizing pneumonia (BOOP) has numerous causes, but it has only been reported in three patients with SSc. We present three cases of biopsy-proven BOOP in patients with scleroderma spectrum disease, including diffuse cutaneous SSc, mixed connective tissue disease with SSc features, and limited cutaneous SSc that later evolved to dermatomyositis/SSc overlap. BOOP and SSc alveolitis have similar findings on spirometry, radiographs, and BAL; the definitive diagnosis of BOOP was therefore made by open lung biopsy. Cyclophosphamide is the current treatment of alveolitis associated with SSc, whereas high-dose prednisone is used to treat BOOP. As a result of this difference, we emphasize the importance of a thorough investigation of pulmonary disease in SSc patients to exclude BOOP and treat appropriately.

Journal Article↗
PubMedDate not supplied

Medical use of dimethyl sulfoxide (DMSO).

DMSO is a clear odorless liquid, inexpensively produced as a by-product of the paper industry. It is widely available in the USA as a solvent but its medical use is currently restricted by the FDA to the palliative treatment of interstitial cystitis and to certain experimental applications. Cutaneous manifestations of scleroderma appear to resolve (albeit equivocally) following topical applications of high concentrations of DMSO. A limited number of small clinical trials indicate that intravenous DMSO may be of benefit in the treatment of amyloidosis, possibly by mobilizing amyloid deposits out of tissues into urine. Dermal application of DMSO seems to provide rapid, temporary, relief of pain in patients with arthritis and connective tissue injuries. However, claims for antiinflammatory effects or acceleration of healing are currently unwarranted. There is no evidence that DMSO can alter progression of degenerative joint disease, and, for this reason, DMSO may be considered for palliative treatment only and not to the exclusion of standard antiinflammatory agents. The safety of DMSO in combination with other drugs has not been established; neurotoxic interactions with sulindac have been reported. In experimental animals, intravenous DMSO is as effective as mannitol and dexamethasone in reversing cerebral edema and intracranial hypertension. An initial clinical trial in 11 patients tends to support this latter application. DMSO enhances diffusion of other chemicals through the skin, and, for this reason, mixtures of idoxuridine and DMSO are used for topical treatment of herpes zoster in the UK. Adverse reactions to DMSO are common, but are usually minor and related to the concentration of DMSO in the medication solution. Consequently, the most frequent side effects, such as skin rash and pruritus after dermal application, intravascular hemolysis after intravenous infusion and gastrointestinal discomfort after oral administration, can be avoided in large part by employing more dilute solutions. Most clinical trials of DMSO have not incorporated the components of experimental design necessary for objective, statistical evaluation of efficacy. Randomized comparisons between DMSO, placebo and known active treatments were rarely completed. Final approval of topical DMSO for treatment of rheumatic diseases in particular will require a multi-center, randomized comparison between high and low concentrations of DMSO and an orally-active, nonsteroidal antiinflammatory agent.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗
PubMed1996

Isolated pulmonary hypertension in systemic sclerosis with diffuse cutaneous involvement: association with serum anti-U3RNP antibody.

OBJECTIVE: To describe a group of patients with systemic sclerosis (SSc) and diffuse cutaneous (dc) involvement with isolated pulmonary hypertension (IPHT) and to compare them to 2 other SSc patient groups, i.e., one with limited cutaneous (lc) involvement with IPHT and another with dcSSc without IPHT. METHODS: The Pittsburgh Scleroderma Databank was screened to identify appropriate patients. SSc specific serum autoantibodies were determined using published methods. Chi-squared tests and Kaplan-Meier survival analysis were performed. RESULTS: During the period 1975-92, 60 of 580 (10%) consecutive new patients with lcSSc and 14 of 677 (2%) with dcSSc have developed IPHT (p < 0.0001). The patients with IPHT did not differ from their parent dc or lc groups by age, race, sex, or cutaneous manifestations. All 14 patients with dcSSc IPHT had minimal or no pulmonary interstitial fibrosis on chest radiograph. The 12 tested had a striking reduction in DLCO (mean 50% of predicted normal), similar to results for patients with lcSSc with IPHT. Of note, 5 of these 14 patients had survived "scleroderma renal crisis" 1.3 to 10.7 (mean 5.5) years before onset of IPHT. Six of 13 (46%) patients with dcSSc with IPHT were positive for serum anti-U3RNP antibody by immunoprecipitation, and one had the typical nucleolar staining pattern of anti-U3RNP, but was not tested for U3RNP by immunoprecipitation. In contrast, only 13 (6%) of the 244 patients with dcSSc without IPHT (p < 0.0001) had anti-U3RNP antibody. Survival from the first reported symptom consistent with pulmonary hypertension (usually dyspnea on exertion) was uniformly poor in both IPHT groups, with the vast majority of patients dying during the first 2 years. CONCLUSION: Patients with SSc with dc involvement can develop severe, fatal isolated pulmonary arterial hypertension. This complication occurs disproportionately more often in patients with serum anti-U3RNP antibody.

Adult↗
PubMedDate not supplied

Early detection of scleroderma-spectrum disorders by in vivo capillary microscopy: a prospective study of patients with Raynaud's phenomenon.

The condition of 25 patients with Raynaud's phenomenon, initially examined by in vivo capillary microscopy, was followed to determine how those who had shown scleroderma-pattern (SD-pattern) capillary abnormalities compared with other patients later in the course of disease. Limited follow-up information was obtained on 19 patients (10 with and 9 without the SD-pattern); the interval between the initial examination and the follow-up ranged from 9 months to 5 years. The results revealed that during the interval, 5 of the 10 patients who had initially displayed SD-pattern capillary abnormalities had developed SD (systemic sclerosis) or a closely related disease, but none of the 9 others. The length of the follow-up was comparable in both groups.

Adolescent↗
PubMed1994

[Pulmonary involvement in sclerodermia].

Progressive systemic sclerosis (PSS) is a connective tissue disease characterized by fibrosis and thickness of cutis and subcutis (scleroderma) and deterioration of small arteries and capillary vessels, with changeable visceral renal, cardiac, intestinal and pulmonary involvements. The disease is characterized by cutaneous sclerosis, that is to say by the increase of consistence and thickness of cutis that lose her usual elasticity. The sclerosis can be limited to the fingers (sclerodactyly) or can involve otherwise (acrosclerosis); many other time is diffuse also to upper limbs and to thorax (diffuse scleroderma). The disease is rare and its incidence is variable from 2 to 10 x 10(6); more frequently hits women than men in the ratio of 3 to 1 or 8 to 1 (according to certain Authors). In the elderly is more frequently represented by the CREST syndrome (calcinosis, Raynaud's phenomenon, oesophageal dismotility, sclerodactyly, teleangiectasia). The authors have considered nine patients with pulmonary involvement of progressive systemic sclerosis: five with CREST syndrome and four with diffuse scleroderma. In addition to the common routine examinations, we performed chest radiography (CXR), pulmonary function tests, fiberoptic bronchoscopy and bronchoalveolar lavage (BAL), Gallium 67 scanning and high resolution computed tomography (HRCT) with the purpose to consider the role of these medical examinations in diagnosis and the follow-up of pulmonary involvement in scleroderma. From the results we have observed that: 1) in early disease chest radiography may be silent even if the patient shows a restrictive syndrome and reduction of diffusion capacity of carbon monoxide (DLCO); 2) chest radiography, pulmonary function tests and Gallium 67 scanning have traditionally been used to measure the degree of lung involvement in SSP but unfortunately, none of these reliably predicts disease activity or future course; 3) bronchoalveolar lavage shows the degree of lung inflammation (alveolitis) characterized by the increase of total BAL cell counts and by higher neutrophil cells in the initial stage of the disease; 4) even HRCT shows in this stage, the typical aspect of higher endoalveolar cellularity with ground glass appearance. With the progression of anatomical damage, there can be found even appearance of complete destruction of pulmonary architecture with the typical alteration of honeycomb. These findings support the hypothesis that a ground glass appearance on HRCT is the radiographic equivalent of alveolitis found in BAL. Furthermore HRCT shows itself useful in the follow-up the disease and in detecting of its activity condition.(ABSTRACT TRUNCATED AT 400 WORDS)

Bronchoalveolar Lavage Fluid↗
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↗
PubMed1992

Localized cutaneous scleroderma.

Localized scleroderma in children includes morphea mainly on the trunk and linear scleroderma on the limbs, scalp, and face. Progressive systemic sclerosis is very rare in children and change from localized to progressive disease is extremely rare. Laboratory abnormalities occur with localized scleroderma, including eosinophilia, positive antinuclear factor (ANF), and increased immunoglobulin (Ig)G. The diseases are usually self limited, but involvement over bones may lead to marked functional impairment. Those on the face may be associated with underlying abnormalities and, occasionally, seizures. Treatment regimens are difficult to assess as there is no chemical marker, but penicillamine has gained wide use. It has numerous side effects and patients should be carefully monitored, particularly with regard to renal function. Other modalities have been used with variable success, but series of patients are small. Many medications produce skin softening in patients with progressive systemic sclerosis. The pathogenesis of skin sclerosis involves complicated interactions between vascular responses, lymphokines, and connective tissue proliferation. The etiology is completely unknown, despite a few cases associated with increased Borrelia titers.

Child↗
PubMed1986

Scleroderma in childhood.

Scleroderma is a rare disease in children. Heterogeneous in its many forms, it ranges from circumscribed and self-limited pigmentary disorders to disabling and disfiguring involvement of an extremity. It also may include diffuse skin disease, multiple internal organ derangements, and a rapidly fatal outcome. Outcome varies widely and is related to the location and extent of cutaneous involvement, evaluation of disfigurement or impairment, and which treatment resources are available.

Child↗
PubMedDate not supplied

Epidemiology of systemic sclerosis.

Systemic sclerosis is a family of disorders most appropriately considered in the category of the connective tissue diseases. Two major forms are recognized (diffuse cutaneous and limited cutaneous involvement subtypes), each with distinctive clinical and serologic findings as well as natural history. Scleroderma is characterized epidemiologically by several distinctive features. From a demographic viewpoint, the disease spares children and its incidence increases steadily with age among adults. If occurs much more frequently in women, especially during the child-bearing years, and most often and most severely in young black women, though there is no overall prominent racial predilection. The annual incidence approaches 20 per million population, and may be considerably underestimated. Both incidence and mortality have increased during the past several decades, but these changes are most likely a result of improved case detection rather than a true increase in incidence. Prevalence studies have not been undertaken, but 500 per million population may be a reasonable estimate. Family and genetic studies suggest a weak genetic predisposition, but several strong HLA associations with scleroderma-specific serum autoantibodies are evident. Certain environmental agents may be implicated in pathogenesis of scleroderma and closely related systemic illnesses associated with cutaneous fibrosis. The widespread pathologic process in systemic sclerosis leads to vascular insufficiency and fibrosis, which diminishes the reserve function of many organ systems. The result is considerable disability, especially affecting hand function, and a significant reduction in life span, with an overall 10-year survival from first physician diagnosis of under 70%. Further epidemiologic studies should take full advantage of established and newly proposed subsets of patients with homogeneous clinical, laboratory, serologic, and natural history features. The environment-host interactions noted here must be fully explored, especially in early untreated disease, where primary rather than secondary mechanisms are most likely to be operative.

Adolescent↗