Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036421 (PSS)
 10,989 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

One of the most appealing current pathogenetic concepts is that progressive systemic sclerosis (PSS) is a reaction to repeated episodes of endothelial cell injury. Injury of small arteries and capillary endothelium initiates reactions which involve increased permeability of the vessels, platelet adherence, myointimal cell proliferation, luminal narrowing and heightened sensitivity of the vessel wall. Clinical evidence of the vessel damage is Raynaud's phenomenon, involving both skin and viscera. The Authors evaluated the effects of iloprost on Raynaud's phenomenon in patients with PSS. This drug provides prolonged vasodilation, reduces platelet aggregation and promotes endothelial lining function repair. This last pattern is of primary importance because it may stop the vicious circle: endothelial injury-platelet hyperaggregation-microangiospasm. Five females were recruited, aged 41-66 years, suffering from well-documented (ARA criteria) PSS, associated with typical Raynaud's phenomenon. The trial provided for intravenous infusion of iloprost at a rate of 1-2 ng/kg/min. First treatment consisted of six-hour infusions on six successive days. After this first treatment, weekly infusions during the winter months were carried on. Drug effectiveness was considered through subjective and objective parameters. All patients showed prominent reduction of number, duration and severity of attacks of Raynaud's phenomenon, improvement of prehensile strength, healing of finger ulcerations and improvement or normalization of digital photoplethysmography. So far, the treatment has been prolonged for years in our patients and still goes on. The side effects of iloprost (headache, flushing, nausea) have been very poor. Therefore, iloprost proved to be a valid drug in the management of Raynaud's phenomenon in patients with PSS, but the inconvenience of intravenous administration may limit its routine use.
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PMID:[Effects of long-term iloprost therapy on Raynaud's phenomenon in progressive systemic sclerosis]. 128 Dec 97

To evaluate the usefulness of recently proposed schemes of classification for systemic sclerosis an extensive cross-sectional study of a series of 164 consecutive patients with long-term systemic sclerosis was undertaken. There were 47 cases of proximal sclerosis, 93 of distal sclerosis and 24 of complete CREST syndrome. The study included clinical, visceral, immunological and follow-up data. In addition, a quantitative clinical score was calculated for each patient, thus providing indications for prognosis. Data were expressed according to three conventional systems of classification: The ARA system, the diffuse versus limited systemic sclerosis system and the early cutaneous involvement system. The most reliable indications of severe outcome were: proximal sclerosis, trunk skin involvement, presence of anti Scl 70 autoantibody, pulmonary and/or heart involvement and age. Diagnosis and prognosis were not generated by the same items. Prognosis indicators proved more accurate for groups than for individuals. Mortality was 1 death per 149 patient X years of follow-up from diagnosis. We conclude that the ARA criteria for classification should be recognized as a standard, but patients with complete CREST syndrome should be included in the distal group. Other systems of classification, principally 2-way versus 3-way criteria, allow different subsets of patients that correlate with prognosis and the severity of the disease, and could be used for therapeutic purposes.
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PMID:Long-term follow-up study of 164 patients with definite systemic sclerosis: classification considerations. 145 83

In an open study conducted over 6 months, gamma interferon was administered to 12 patients suffering from progressive systemic sclerosis. The dosage used was three injections, each of 50 micrograms s.c. in the first 2 weeks, followed by 50 micrograms twice a week. All patients fulfilled the criteria required for classification as type II in the ADF system or the major criterion of the ARA (proximal sclerosis). Therapeutic effects were noted as a decrease in of skin thickness (10/12 = 83%), improvement in muscle and joint pains (5/8 = 63%) and reduction of the procollagen-type III peptide in serum by more than 20% (8/12 = 66%). No effects on fibrosis or dysfunction of internal organs were seen. Disease progression did not occur in any of the patients. Side-effects of gamma interferon were noted in 4 patients, in the form of slightly elevated body temperature and transient influenza-like symptoms, but these occurred only at the beginning of therapy and soon passed.
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PMID:[Therapy of progressive scleroderma with gamma-interferon]. 191 61

We looked for anti-Scl-70 and anti-centromere antibodies in 109 patients (26 men and 83 women). Mean age was 43 +/- 15 years. Forty patients had systemic sclerosis according to ARA criteria. The extension of cutaneous involvement was defined by using Barnett and Coventry criteria: 12 patients were type I (sclerodactyly), 20 type II (acrosclerosis) and 8 type III (diffuse scleroderma). Among the 12 patients with type I, there were 8 cases of CREST syndrome defined as follows: presence of sclerodactyly, Raynaud's phenomenon and 2 of the 3 following criteria: oesophageal dysmotility, calcinosis, telangiectasia. Other organ involvement was recorded. Control patients had idiopathic Raynaud's phenomenon (n = 22), other connective tissue diseases (n = 20), and miscellaneous diseases (n = 28). Ninety-nine patients were prospectively included in this study. Patients' sera were stored at -20 degrees C. Ten previously stored sera obtained from patients with systemic sclerosis were also analyzed. Immunological tests were performed simultaneously and with no information on the diagnosis. When antinuclear antibodies were detected by indirect immunofluorescence, double immunodiffusion and immunoblotting were performed. Anti-Scl-70 antibodies were detected in systemic sclerosis only: 1 of 12 type I, 11 of 20 type II and 4 of 8 type III. One serum negative by immunodiffusion was positive using immunoblotting. We found that the specificity of anti-Scl-70 antibodies for systemic sclerosis was 100 p. 100 and their sensitivity 40 p. 100. There was a correlation between the presence of anti-Scl-70 antibodies and the presence of antinuclear antibodies (p less than 0.05) and the extent of cutaneous involvement (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Anti-Scl-70 antibodies in systemic scleroderma]. 211 50

Three hundred and sixty two Japanese patients with rheumatoid arthritis (RA) and 455 patients with other rheumatic diseases (SLE 233, PSS 63, MCTD 51, PM-DM 41 Behcet's disease 33 and OA 33) were examined for the evaluation of the 1987 revised ARA criteria for RA. In our cases sensitivity was slightly decreased and specificity was markedly increased 5 out of 7 criterions compared with the results reported by ARA. In the investigation how many number of criterions at least which the patients with RA should satisfy, 4 out of 7 criterions in the 1987 criteria turned out the highest figure in the accuracy (the mean of sensitivity and specificity values). So the patients who satisfied at least 4 criterions were classified to have RA. Sensitivity of the 1987 criteria decreased to 90% although that of the 1958 ARA criteria was 93%. Specificity were markedly increased from 88% to 95% (the 1958 and 1987 criteria, respectively). These results might be based on the revision of the duration of "morning stiffness" (lasting at least 1 hour) and on the deletion of "joint pain or tenderness" which was relatively less specific for RA.
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PMID:[Evaluation of the 1987 revised ARA criteria for rheumatoid arthritis in Japan]. 277 58

The overwhelming majority of patients with PSS present with combinations of Raynaud's phenomenon, sclerodactyly, polyarthralgias, or swelling of an extremity. However, the clinical presentation of PSS may be atypical; 14% of patients in the present series initially sought medical attention for symptoms other than Raynaud's phenomenon, tight skin, or joint pain. In the present series, only 31% of patients fulfilled the ARA criteria for PSS at the time of initial medical evaluation. Most patients manifested advanced disease by the time the criteria were fulfilled. The ARA criteria for the classification of PSS appear to have limited value with regard to making the diagnosis in an individual patient. Rapidly progressive PSS occurred in 17.6% of patients in this series and represents a particularly fulminant form of the disease whose course may not be predictable on clinical grounds at the time of initial medical evaluation or diagnosis. Patients destined to develop renal or cardiorespiratory failure usually do so in the first 3 years of disease. Close observation of PSS patients during the first 12 to 18 months may serve to identify those individuals who will undergo an accelerated disease course. Prognosis for patients with rapidly progressive PSS is poor and is associated with significantly higher mortality compared with patients with a more protracted disease course. Future therapeutic trials in PSS should be designed with the recognition that a subgroup of patients with this disorder will have a rapidly progressive disease course.
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PMID:Progressive systemic sclerosis: mode of presentation, rapidly progressive disease course, and mortality based on an analysis of 91 patients. 318 42

We studied 14 patients with PSS, 12 females and 2 males with a mean age of 43.6 and a medium of 8 years disease. All of the patients were selected for this study according to updated ARA criteria and were included in a prospective protocol to investigate digestive involvement. This protocol consists of a complete medical history, physical examination, radiologic and endoscopic studies, parasitological and microbial flora investigation. The symptoms more frequently seen were: pyrosis (78%), gastroesophageal regurgitation (50%), flatulence (50%), dysphagia (42%) and chronic diarrhea (21%). The radiologic findings commonly seen were: distal esophageal aperistalsis (78%), gastroesophageal reflux (57%), dilatation of intestinal loops (35%), changes of the mucosal folds (35%). A mild esophagitis was seen endoscopically in 64% of the patients, moderate and severe in 7% respectively. The study of the microbial flora showed contaminations with enterobacteria in 5 patients (35%). After statistical analysis we concluded that the digestive compromise by PSS is frequent, being the esophagus more commonly affected (80%), at the beginning in the form of reflux esophagitis and later in esophageal stenosis, the compromise of the small intestine (40%) is manifested by chronic diarrhea or dyspeptic flatulence, which correlates well the radiologic findings and the bacterial overgrowth in this organ. The colonic compromise generally is asymptomatic, and the common finding is dilatation os the colonic loops. Finally, the bacterial overgrowth in the small intestine is a secondary involvement to the intestinal compromise of Progressive Systemic Sclerosis.
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PMID:[Digestive involvement in progressive systemic sclerosis]. 322 28

The relationship between anticentromere antibodies (ACA), antitopoisomerase I or Scleroderma 70 (Scl-70) antibodies, HLA-DR antigens, and clinical manifestations of scleroderma were examined in 51 patients defined by ARA criteria. No association between a given HLA-DR antigen and either ACA or anti-Scl-70 was found. Statistically significant associations were noted for patients with ACA who had a lower frequency of arthritis and longer disease duration; anti-Scl-70 patients were more likely to be males with a higher frequency of pulmonary, cardiac and sicca symptoms.
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PMID:Scleroderma: DR antigens, autoantibodies and clinical manifestations. 332 91

The authors conducted a study of 47 patients with a diagnosis of systemic sclerosis in order to determine the sensitivity, specificity and Youden index and consequently the diagnostic value of the generally accepted criteria and of some other common disease features. The control population consisted of 324 patients with rheumatoid arthritis and 35 patients with systemic lupus erythematosus. In addition to proximal scleroderma, the presence of which was required for inclusion in this study, other disease features score a high to moderate Youden index: sclerodactyly, Raynaud's phenomenon, appearance of perioral grooves, cinematographic evidence of esophageal hypomotility, periarticular calcifications, widening of the periodontal membrane on teeth radiographs and specific histologic changes on fingertip biopsy. Finally, a good correlation was found with most of the ARA subcommittee criteria.
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PMID:Disease manifestations of progressive systemic sclerosis: sensitivity and specificity. 344 3

Ninety patients who fulfilled the ARA criteria for systemic sclerosis were divided into 6 groups on the basis of cutaneous areas of sclerosis. Anticentromere antibody (ACA) was found in 14 of 28 patients of the first 2 groups, the first group being pure sclerodactyly, and the second sclerodactyly plus minimal sclerotic lesions (eyelids, neck, armpits). In the other 4 groups (62 patients) in whom skin sclerosis was more diffuse, ACA was found in one case. Fluorescent antinuclear antibodies (FANA) with a diffusely grainy pattern, those with a nucleolar pattern and the anti-Scl-70 antibody were present in all 6 groups, but were significantly more frequent in the last 4 groups than in Groups 1 and 2. The cumulative survival rate as well as disease duration were found to be significantly longer in the first 2 groups designated "limited cutaneous systemic sclerosis," than in the 5th and 6th groups, i.e., patients in whom the trunk also was involved: "diffuse cutaneous systemic sclerosis." The cumulative survival rate and disease duration of patients with skin sclerosis of the face and limbs, but not of trunk, were not significantly different either from those of patients with limited cutaneous systemic sclerosis or from those of patients with diffuse cutaneous systemic sclerosis. Thus these patients show the same antibody pattern as diffuse cutaneous systemic sclerosis, but the prognosis not significantly different from the other 2 subsets. We suggest they constitute a distinct subset of systemic sclerosis for which we propose the name "intermediate cutaneous systemic sclerosis."
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PMID:Different antibody patterns and different prognoses in patients with scleroderma with various extent of skin sclerosis. 354 86


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