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)

A 43-year old man with CRST syndrome (calcinosis, Raynaud's phenomenon, sclerodactyly and telangiectasia) and progressive systemic sclerosis presented with a four-year history of relapsing abdominal pain, the result of chronic pancreatitis, not associated with alcoholism, biliary disease, or any of the known causes of pancreatitis. He had a good response to retrograde pancreatic duct drainage but exhibited management problems and complications that may be peculiar to the systemic sclerosis patient with pancreatitis. A cause and effect relationship between progressive systemic sclerosis and pancreatic disease is not proven but we believe there is evidence to suggest such a relationship.
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PMID:Idiopathic calcific pancreatitis, CRST syndrome and progressive systemic sclerosis. 43

Progressive systemic sclerosis, especially prior to distinctive internal organ involvement can be difficult to distinguish from vinyl chloride disease. A 50-year-old male developed classic scleroderma while working with polyvinyl chloride. Early in his disease he displayed a number of features common to both disorders, including acroosteolysis, sclerodactyly, polygammopathy and circulating immune complexes. Characteristic visceral involvement was the cardinal feature which distringuished his disease from the pseudoscleroderma of vinyl chloride disease.
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PMID:Scleroderma simulating vinyl chloride disease. 46 7

The CREST syndrome refers to a disorder comprising the manifestations of calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. Thirteen CREST patients (two with CRST) were compared with 26 patients with systemic sclerosis but without the full manifestations of the CRST syndrome. No significant difference was found between the groups in the age of onset of Raynaud's phenomenon, degree of multiphasic digital color changes, ulcerations of fingers, sclerodactyly, or in the frequency of abnormal esophageal peristalsis or dysphagia. Laboratory results were similar, including the frequency of an elevated ESR. However, the CREST patients had a significantly lower frequency of arthralgia (54%) and arthritis (15%) than did those with scleroderma (88% and 65%, respectively). All but one of the CREST patients were women, which was a greater proportion than found among scleroderma cases (69%), and all were white (P less than .05). Most patients with the CREST syndrome had rather severe acrosclerosis. At last evaluation, four patients were chronically ill and three had died. The CREST and CRST syndromes are closely related disorders that seem to be part of the spectrum of systemic sclerosis.
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PMID:The 'CREST' syndrome. Comparison with systemic sclerosis (scleroderma). 50 20

Lesions of circumscribed scleroderma clinically appeared as pseudoxanthoma elasticum. The histological structure, however, revealed circumscribed scleroderma. The sclerodactyly, Raynaud's phenomenon, acrosclerosis and the laboratory results suggested systemic sclerosis.
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PMID:[Morphea resembling pseudoxanthoma elasticum in progressive scleroderma]. 72 40

Severe pulmonary hypertension without pulmonary fibrosis occurred in 10 patients with the CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia), reputedly a benign variant of progressive systemic sclerosis. Time from the initial symptom, Raynaud's phenomenon, to the recognition of pulmonary hypertension was as long as 40 years. Pulmonary hypertension and increased pulmonary vascular resistance was shown in all patients. Autopsy examination in three of six deaths attributable to pulmonary hypertension showed intimal proliferation with myxomatous change in the small- and medium-sized pulmonary arteries similar to changes in the digital arteries of patients with scleroderma and Raynaud's phenomenon, and interlobular renal arteries of those with "scleroderma kidney." It is concluded that the CREST syndrome is not entirely benign but may be complicated, after a long clinical course, by progressive pulmonary vascular obliteration, pulmonary hypertension, and death in the absence of significant pulmonary fibrosis.
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PMID:Pulmonary hypertension in the CREST syndrome variant of progressive systemic sclerosis (scleroderma). 84

Eighty-four patients with systemic sclerosis have been investigated and observed at intervals for up to 15 years. The prognosis is worse in males than in females although in any individual case the clinical course is unpredictable. Those patients with the combination of calcinosis, Raynaud's phenomenon, sclerodactyly and telangiectasia have the same degree of systemic involvement and the same prognosis as other patients with systemic sclerosis, and the term 'CRST syndrome' should be abandoned.
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PMID:The prognosis of systemic sclerosis. 95 40

Three patients seen with similar findings of progressive systemic scleroderma. Two of the patients, a father and son, had very similar skin changes, sclerodactyly, Raynaud phenomenon, gastrointestinal involvement, and pulmonary symptoms. The three patients were from the highly inbred Brandywine triracial isolate. This isolate is a group of families who have been inbreeding since 1660 and now have the highest gene frequencies for sickle cell anemia and oculocutaneous albinism in the United States. There have been only a few reported cases of familial scleroderma and the hereditary aspect of the disease has not been well established. This report shows that the mortality for scleroderma in this isolate is at least 250 times the mortality of the general population, thus suggesting a probable genetic predisposition for the disease.
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PMID:Familial progressive systemic scleroderma. 111 27

Gastrointestinal transit times were measured in 12 patients with progressive systemic sclerosis. The CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) was found in all patients. None of the patients reported complaints referable to specific gastric, small intestinal, or colonic involvement. The patient group had an increased mean gastric emptying time of 99mTc-labeled cellulose fiber when compared with 16 healthy controls [1.17 (0.89-1.38) hr [median (range)] vs 0.84 (0.56-1.88) hr; P less than 0.02], whereas mean gastric emptying time of 2- to 3-mm 111In-labeled plastic particles was unaffected [1.86 (0.99-2.74) hr vs 1.50 (0.92-2.51) hr; NS]. No difference was observed in mean small intestinal transit time of cellulose fiber [4.33 (0.50-7.04) hr vs 3.74 (2.09-7.59) hr; NS] or plastic particles [4.21 (2.00-6.25) hr vs 3.53 (1.50-6.70) hr; NS] between patients and controls. The patient group had an increased mean colonic transit time of plastic particles [47 (24-116) hr vs 29 (18-46) hr; P less than 0.01]. These findings suggest that asymptomatic delay in gastric emptying and colonic transit is frequent in patients with progressive systemic sclerosis.
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PMID:Gastrointestinal transit times of radiolabeled meal in progressive systemic sclerosis. 132 42

The authors used nailfold capillary microscopy (NCM) to evaluate 112 patients with systemic sclerosis spectrum disorders (SSc). Patients were classified as S1 if they had skin involvement proximal to the metacarpo-phalangeal joints. S2 if they had at least two minor SSc American Rheumatism Association criteria, and S3 if they had at least two CREST criteria (calcinosis, Raynaud's, esophageal motility disorder, sclerodactyly, telangiectases), without S1 or S2 criteria. Disease duration from the first symptom was similar in all groups (7.17 +/- 8.98 years). Disease severity was determined by a total score of seven target organ involvements. S1 patients had a greater degree of skin and pulmonary involvement, with a mean score of 26.2 +/- 17.3. S2 patients had a mean score of 13.8 +/- 12.4, and had mostly vascular and digestive involvement, in comparison with S3 patients (7.2 +/- 7.2; p less than 0.001 and p less than 0.01 respectively). NCM sensitivity for S1 and S2 was 93.6%. NCM correlated with the degree of target organ involvement (p less than 0.01). Three NCM profiles established were: "mild," normal or borderline capillaries; "moderate," other abnormalities with no capillary telangiectases; and "severe," abnormalities other than those of the mild profile, with capillary telangiectases.
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PMID:Evaluation of the severity of systemic sclerosis by nailfold capillary microscopy in 112 patients. 157 68

Systemic Sclerosis is a multisystemic disease characterized by sclerosis of the skin and visceral organs, vasculopathy (Raynaud's phenomenon) and autoantibodies. The criteria for the classification of the disease requires either proximal scleroderma (major criteria) or the presence of 2 of the 3 minor features namely sclerodactyly, digital pitting scars and bibasilar pulmonary fibrosis. There are 3 subsets of this condition--diffuse variant, limited variant (CREST syndrome) and Overlap Syndrome (where patients have features of other rheumatic diseases). There are localized forms of scleroderma and pseudoscleroderma states. The presenting features of Systemic Sclerosis are usually Raynaud's, skin changes and arthralgia. Systemic complaints like breathlessness, dyspepsia, etc depending on the organ involved may be present. Management starts with patient education regarding the disease, skin care, exercises and regular medical check-up. There is no miracle cure but much can be done to improve the quality of life of the patient. Nifedepine and other drugs may improve Raynaud's phenomenon. Drugs can be used to treat other complications. Various medication have been tested as disease modifying drugs for scleroderma. These include drugs which inhibit collagen like D-penicillamine, colchicine, and immunosupressive drugs like cyclosporin. Ketotifen, a mast cell stabilizer has been reported to be effective in scleroderma. As it is a relatively safe drug, clinical trials are underway.
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PMID:Systemic sclerosis. 162 Nov 27


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