Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Scleroderma, Limited”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

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

At least 703 records · Page 39Linked to original sources
PubMed2000

Quantitation of microcirculatory abnormalities in patients with primary Raynaud's phenomenon and systemic sclerosis by video capillaroscopy.

OBJECTIVE: : To assess nailfold capillary density and dimensions in patients with primary Raynaud's phenomenon (PRP), limited cutaneous systemic sclerosis (LSSc) and diffuse cutaneous SSc (DSSc), and healthy control subjects. METHODS: : Using the technique of nailfold video capillaroscopy, capillary density and dimensions were averaged from all visible capillaries in a 3 mm length of the nailfold from right and left ring fingers of each subject. Twenty healthy control subjects, 15 patients with PRP, 13 patients with DSSc and 21 patients with LSSc were examined. Intra-observer and inter-observer variability were calculated in 18 and 23 patients, respectively. RESULTS: : There were significant trends for capillary density to fall and for all dimensions to rise across the four groups (P < 0. 0001 for density and all dimensions, order healthy controls, PRP, DSSc and LSSc). Intra- and inter-observer reproducibility studies showed that although there was good correlation between and within observers, the limits of agreement were between +/-25-50% indicating lack of reproducibility. CONCLUSIONS: : Microcirculatory abnormalities can be quantified using the technique of video capillaroscopy and were most marked in patients with LSSc.

Adult↗
PubMed1997

Nuchal fibroma.

Nuchal fibroma was diagnosed in a 54-year-old diabetic woman with a two year history of increased skin thickness of her low-posterior neck and interscapular region, causing discomfort and limitation of neck and arm motion. Physical and laboratory examinations excluded further disorders. The patient was released, free from symptoms, after complete excision of the soft tissue tumor.

Adipose Tissue↗
PubMed1988

Plasma somatomedin-C levels in systemic sclerosis.

We have measured the plasma levels of somatomedin-C (SM-C) or insulin-like growth factor I (IGF-I) in 13 patients with progressive systemic sclerosis (PSS) and age and sex matched healthy controls. We found the plasma SM-C levels to be within normal limits in all the patients. Thus, if somatomedin-C plays a role in the pathogenesis of PSS, it is more likely to be at the fibroblast receptor level or in the synthetic response of fibroblasts to SM-C.

Adult↗
PubMed2003

Idiopathic circumscripta calcinosis cutis of the knee.

BACKGROUND: Calcinosis cutis, a disease characterized by the presence of calcium deposits in the skin, is classified into four types according to etiology: dystrophic, metastatic, iatrogenic and idiopathic. The dystrophic form is the most common while the idiopathic one is the rarest, but specific incidence and frequency data are not available in scientific literature. OBJECTIVE: Calcinosis cutis circumscripta is a very rare form of idiopathic calcinosis cutis arising in the second half of life. It typically involves the extremities and is associated with prior trauma and scleroderma. We dealt with a very rare form of calcinosis cutis circumscripta in a healthy patient, for whom surgical excision revealed to be an effective and successful treatment. METHODS AND MATERIALS: We present the case of a 46-year-old woman affected by idiopathic circumscripta calcinosis cutis of the left knee, successfully treated by surgical removal. DISCUSSION: Medical and surgical treatment are options to cure calcinosis cutis. Medical therapy is not very effective. Surgical excision has shown to be beneficial, as it can provide a symptomatic relief. However, since calcinosis cutis limits are not always well defined a recurrence of the lesions may occur.

Adult↗
PubMed1981

Investigation of type I and type III collagens of the lung in progressive systemic sclerosis.

The interstitial collagens, type I and type III, were investigated in lung tissue from patients with progressive systemic sclerosis (PSS) with pulmonary involvement. By use of CNBr digestion of whole tissue, the relative content of type I versus type III collagen was unchanged. This contrasts with idiopathic pulmonary fibrosis. Limited pepsin digestion released greater amounts of collagen (55%) than normal (16%), but the individual collagen chains were chemically indistinguishable. A reduced amount of the more stable collagen crosslink, hydroxylysinonorleucine, was observed which was consistent with the relatively greater degree of solubilization.

Adult↗
PubMed1996

Colon involvement in systemic sclerosis: clinical-radiological correlations.

About one third of all patients with systemic sclerosis (SS) presents colon abnormalities, although these may be underestimated because they frequently remain asymptomatic for a long time. Thirty-five patients (33 women and 2 men; mean age 56.5 years; mean disease duration 11.9 years) affected by SS (25 with limited and 10 with diffuse pattern of skin involvement) were investigated using barium enema to detect radiological changes in the colon, and to correlate them with other visceral involvement, autoantibody profile, abdominal symptoms and duration of the disease. Ten patients (28.6%) showed X-rays abnormalities (excluding isolated diverticula), wide-mouthed sacculations being the most frequent finding. Our data confirm that the colon is frequently involved in SS, even in the limited form of the disease. The most relevant finding was the dissociation between clinical symptoms and radiological features which proved to be more evident among the patients with limited SS. No correlations were found between the radiological picture and any other parameter, thus suggesting that careful evaluation of the colon should be performed in any patient suffering from the disease.

Adult↗
PubMed1993

RNA B is the major nucleolar trimethylguanosine-capped small nuclear RNA associated with fibrillarin and pre-rRNAs in Trypanosoma brucei.

RNA B is one of three abundant trimethylguanosine-capped U small nuclear RNAs (snRNAs) of Trypanosoma brucei which is not strongly identified with other U snRNAs by sequence homology. We show here that RNA B is a highly diverged U3 snRNA homolog likely involved in pre-rRNA processing. Sequence identity between RNA B and U3 snRNAs is limited; only two of four boxes of homology conserved between U3 snRNAs are obvious in RNA B. These are the box A homology, specific for U3 snRNAs, and the box C homology, common to nucleolar snRNAs and required for association with the nucleolar protein, fibrillarin. A 35-kDa T. brucei fibrillarin homolog was identified by using an anti-Physarum fibrillarin monoclonal antibody. RNA B and fibrillarin were localized in nucleolar fractions of the nucleus which contained pre-rRNAs and did not contain nucleoplasmic snRNAs. Fibrillarin and RNA B were precipitated by scleroderma patient serum S4, which reacts with fibrillarins from diverse organisms; RNA B was the only trimethylguanosine-capped RNA precipitated. Furthermore, RNA B sedimented with pre-rRNAs in nondenaturing sucrose gradients, similarly to U3 and other nucleolar snRNAs, suggesting that RNA B is hydrogen bonded to rRNA intermediates and might be involved in their processing.

Animals↗
PubMed1995

Guidelines for clinical trials in systemic sclerosis (scleroderma). I. Disease-modifying interventions. The American College of Rheumatology Committee on Design and Outcomes in Clinical Trials in Systemic Sclerosis.

OBJECTIVE: To develop guidelines for therapeutic trials designed to improve the overall course of systemic sclerosis (SSc), that is, to reduce the development of significant organ damage or death. METHODS: A committee developed general guidelines for patient inclusion and exclusion criteria, randomization, blinding of patients and physicians, controls, duration of the trial, investigator training, responses, samples size, study dropouts, statistical analyses, data management, and safety monitoring. Delphi and nominal group techniques were used. RESULTS: Briefly, patients with diffuse cutaneous SSc of less than 24 months' duration should be included because they are at greatest risk for the development of severe organ damage and death. Patients should be excluded if they have other connective tissue diseases, SSc-like illnesses related to exposures or ingestions, severe existing internal organ damage, an unacceptable risk of side effects, or concurrent therapies that might independently influence the outcome. Randomized, double-blind, placebo-controlled trials are preferred. The treatment and followup period must be long enough to permit observation of any disease modification, which is likely to require 18-36 months, unless an extraordinarily effective therapy is identified. Responses selected should be quantitative, consistently and accurately reflect activity of SSc in major target organs (not solely the skin), be sensitive to change, and be standardized, with limited variability. An example of a set of responses is given. Surrogate responses are desirable, but none have been validated as correlating with organ damage. CONCLUSION: Guidelines have been established for trials of disease-modifying interventions in SSc. These guidelines will need to be altered as additional information becomes available. Any given protocol will be individualized based on the nature of the intervention and objectives of the study. Nonetheless, each study team should develop a protocol that meets the spirit of these guidelines.

Clinical Protocols↗
PubMed1995

Anticentromere antibody--clinical associations. A study of 44 patients.

The objective of this study was to determine the clinical features of 44 patients with anticentromere antibody (ACA) positivity. We undertook a retrospective review of 44 ACA-positive patients (1 male and 43 females with a mean +/- SD age of 53.6 +/- 12.2 years). There were 25 patients with limited systemic sclerosis, 12 with Raynaud's disease, 2 with Sjögren's syndrome, 2 with systemic lupus erythematosus and 3 with polyarthritis. ACA was more frequently found in patients affected by limited systemic sclerosis with mild visceral involvement and in patients with Raynaud's disease. Moreover, ACA was detected in other connective tissue diseases that were characterized by an atypical autoantibody profile.

Adult↗
PubMed1978

[The clinical significance of soluble nuclear antigen specific antibodies (author's transl)].

Anti-ENA antibodies have been found in 176 sera which nearly all contained antinuclear antibodies giving a speckled pattern of nuclear fluorescence. The charts of 134 of these 176 patients were available for a thorough clinical study. Among these 134 patients, 59 had a well defined Connective Tissue Disease including 40 SLE, 31 had a limited clinical syndrome made of Raynaud's phenomenon, inflammatory polyarthritis, swollen fingers and hyperglobulinemia and 34 had a complex clinical picture associating signs of more than one connective tissue disease. Some of the patients in this third group could be considered as-having the Mixed Connective Tissue Disease (MCTD) described by Sharp et al. Anti-RNP antibodies were more common in this series than the other anti-ENA antibodies. However, no narrow specificity could be assigned to any of these antibodies. This is true of the non anti-RNP antibodies, the anti-Sm in particular, which were found in 49 patients of whom 32 had SLE existing alone or in association with features of other connective tissue diseases and 17 had another connective tissue disease or the afore-mentioned limited clinical syndrome. In any case, the anti-ECT antibodies never reach the diagnostic value of the anti-DNA antibodies.

Adolescent↗
PubMed1999

Cardiac involvement in limited systemic sclerosis: non-invasive assessment in asymptomatic patients.

Nineteen patients with limited systemic sclerosis (SSc) and without any cardiac symptoms were evaluated non-invasively for silent cardiac involvement using electrocardiography; M-mode, two-dimensional and Doppler echocardiography, and resting and post exercise radionuclide ventriculography. Left anterior hemiblock and mild pericardial effusion were seen in two patients. The interventricular septal thickness and left ventricular posterior wall thickness were greater in patients compared with controls. Patients with limited SSc also exhibited low early diastolic filling velocities and a low early diastolic atrial filling ratio. However, these values did not correlate with the age of the patient or disease duration. Eight patients (42.1%) were found to have cardiac dysfunction, of which four had combined systolic and diastolic dysfunction. Isolated systolic and diastolic dysfunction was seen in three patients and one patient, respectively. The age and disease duration in patients with cardiac dysfunction did not differ from patients without cardiac dysfunction. Functional cardiac involvement occurs in a large proportion (42.1%) of patients with limited SSc, and can be easily picked up by non-invasive methods such as echocardiography and radionuclide ventriculography. The prognostic significance of these findings requires further long term studies.

Adult↗
PubMed1994

Intravenous dexamethasone pulse therapy in diffuse systemic sclerosis. A randomized placebo-controlled study.

Thirty-five patients with diffuse systemic sclerosis were studied in a randomized, placebo-controlled, double-blind study. Seventeen patients received intravenous dexamethasone "pulse" therapy, while 18 patients received placebo. Each "pulse" consisted of 100 mg dexamethasone in 250 ml 5% dextrose infused intravenously over 1 h. Pulse therapy was repeated every month for 6 months. Assessment of disease status with various parameters was done at entry and at completion of trial, i.e. after 6 months. Significant improvement in skin involvement was seen in the study group, with the total skin score (TSS) decreasing from 28.5 +/- 12.2 to 25.8 +/- 12.8, while in the control group, TSS increased from 30.6 +/- 13.2 to 34.7 +/- 10. Similarly, significant improvement was noted in the flexion index. Other parametres that included extension index, maximum oral opening, range of movement of joints, functional disability score, Raynaud's phenomenon (frequency and duration), ESR, proteinuria, chest X-ray, ECG, lung function tests, barium swallow and antinuclear antibody were unchanged. Adverse effects of therapy were limited to an increased incidence of minor chest infections. It is concluded that intravenous pulse dexamethasone may be useful in the treatment of diffuse systemic sclerosis.

Adult↗
PubMed1990

Penicillamine in systemic sclerosis: a reappraisal.

In a 36-month prospective trial 21 patients with systemic sclerosis (diffuse systemic sclerosis 16 patients and 5 subjects with limited cutaneous subtype) were treated with D-penicillamine. In all patients with diffuse systemic sclerosis there was objective improvement. The degree and extent of skin involvement decreased significantly (p less than 0.001), whereas no objective improvement was noted in patients with limited cutaneous subtype. Further, no systemic progression of the disease was observed during the study period. Our results suggest that a prolonged treatment with D-penicillamine in small doses is not only beneficial and effective but also free of side-effects, if used at an earlier stage.

Adult↗
PubMed2005

Chronic graft-versus-host-disease-like dermopathy in a child with CD4+ cell microchimerism.

We report the case of an 11-year-old boy suffering from a severe progressive chronic skin disease with clinical features of progressive systemic scleroderma, systemic lupus erythematosus and dermatomyositis. Skin biopsies revealed fibrosis and lichenoid changes and muscle biopsy a myositis. Immunohistology of the skin showed a lichen-ruber-like pattern. Despite repeated extensive investigations, no autoantibodies were detectable. Some of these findings looked like those described in juvenile dermatomyositis. Finally, it could be demonstrated that the boy showed microchimerism with approximately 1% maternal CD4+ lymphocytes in his peripheral blood leukocytes. Furthermore maternal cells could be demonstrated in inflamed muscle tissue. So a graft-versus-host-disease-like pathomechanism appears to be likely. Several systemic therapies have been used with limited success to improve the condition including corticosteroids, azathioprine, cyclosporine A and mycophenolate mofetil. A distinct improvement of erythemas and sclerosis could be achieved by means of low-dose UVA1 phototherapy which was applied with escalating single doses of 3-12 J/cm2 for 35 consecutive days.

CD4-Positive T-Lymphocytes↗
PubMed1989

Clinical distribution of anticentromere antibody in Japanese patients.

Serum samples from 401 subjects were screened for the presence of anticentromere antibody using HEp-2 cells as the substrate for an indirect immunofluorescence method. Anticentromere antibody was found in 16 cases; 8 out of 62 patients with systemic sclerosis, 3 out of 7 patients with primary Raynaud's disease, 2 out of 41 patients with systemic lupus erythematosus, 1 out of 54 asymptomatic relatives of systemic sclerosis and 2 out of 50 patients with miscellaneous diseases. In systemic sclerosis, the patients with anticentromere antibody were limited in CREST (calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactylia and telangiectasia) variant. As reported previously by many investigators, anticentromere antibody is considered as a useful immunologic marker for CREST variant of systemic sclerosis, although this antibody is widely distributed in other conditions with less frequency.

Autoantibodies↗
PubMed2002

Clinical utility of common serum rheumatologic tests.

Serum rheumatologic tests are generally most useful for confirming a clinically suspected diagnosis. Testing for rheumatoid factor is appropriate when rheumatoid arthritis, Sjögren's syndrome or cryoglobulinemia is suspected. Antinuclear antibody testing is highly sensitive for systemic lupus erythematosus and drug-induced lupus. Anti-double-stranded DNA antibodies correlate with lupus nephritis; the titer often corresponds with disease activity in systemic lupus erythematosus. Testing for anti-Ro (anti-SS-A) or anti-La (anti-SS-B) may help confirm the diagnosis of Sjögren's syndrome or systemic lupus erythematosus; these antibodies are associated with the extraglandular manifestations of Sjögren's syndrome. Cytoplasmic antineutrophil cytoplasmic antibody testing is highly sensitive and specific for Wegener's granulomatosis. Human leukocyte antigen-B27 is frequently present in ankylosing spondylitis and Reiter's syndrome, but the background presence of this antibody in white populations limits the value of testing. An elevated erythrocyte sedimentation rate (ESR) is a diagnostic criterion for polymyalgia rheumatica and temporal arteritis; however, specificity is quite low. ESR values tend to correlate with disease activity in rheumatoid arthritis and may be useful for monitoring therapeutic response.

Antibodies, Antinuclear↗
PubMed1993

[Evaluation of small intestinal motility].

Disturbances of small bowel motor function are increasingly recognized in clinical practice, either in the setting of an underlying disease that may affect the neuro-hormonal control of gut motility, such as diabetes or scleroderma, or as part of unexplained intestinal dysfunctions such as the irritable bowel syndrome or chronic idiopathic intestinal pseudo-obstruction. In the absence of endoscopic or radiological mucosal disease, it is often clinically helpful to define the motor function of the small bowel to understand the origin of the patient's symptoms. The hydrogen breath test after a lactulose oral load is currently used to measure mouth to caecum transit time. However, the reproducibility of this test is poor, and the range of normal values is wide. Scintigraphic determination of small intestinal transit time overcomes some of the limitations of the hydrogen breath test. This is however a time consuming procedure--up to 10 hrs when the time for acquisition, processing and analysis is included--and the costs prohibit widespread application of the technique. It is further restricted by the exposure to ionising radiation, particularly if repeated evaluations are necessary, for example in drugs trials. Manometry records mechanical activity of the bowel and detects quantitative and qualitative changes of small intestinal motility. As with scintigraphy, high costs and radiation exposure limit its usefulness. The major clinical application of the technique is in the diagnosis of chronic intestinal pseudo-obstruction.

Breath Tests↗
PubMed2006

Evaluation of cardiac laboratory markers in patients with systemic sclerosis.

OBJECTIVES: Myocardial involvement is frequent in systemic sclerosis, but symptoms are usually delayed and non-specific, thus often misrecognized. The aim of this study was the evaluation of the early subclinical cardiac involvement in patients with systemic sclerosis by means of non-invasive laboratory cardiac markers. DESIGN AND METHODS: Cardiac troponin T (cTnT), ischemia modified albumin (IMA) and NT-prohormone-brain natriuretic peptide (NT-proBNP) were measured in 40 female patients with systemic sclerosis and in 40 matched healthy controls. RESULTS: Patients with systemic sclerosis displayed significantly increased concentrations of serum IMA (106 versus 93.5 kunits/l, P < 0.0001) and NT-proBNP (89 versus 37 pg/ml, P < 0.0001), whereas no significant differences could be observed in both IMA and NT-proBNP values in limited versus diffuse pattern of disease. CONCLUSIONS: The increased levels of NT-proBNP and IMA could be considered a sign of early myocardial involvement, warranting further heart examination and a regular follow-up.

Aged↗