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)

The morphological and haemodynamic findings in the vessels of the forearm and hand in 21 patients with progressive scleroderma are described. In these patients, brachial arteriograms have been carried out before instituting fibrinolytic therapy. Injections were carried out before and after intra-arterial Priscolin and the results were compared. Obliterative changes in the digital arteries are already present during the oedematous stage. Between the oedematous and advanced stages, there are vascular changes of progressive severity. In the sclerotic stage, the changes may be less marked, but more often consist of advanced obliterative vascular disease; this cannot be distinguished from the atrophic stage. The haemodynamics of the digital arteries can be influenced by Priscolin and this provides some information regarding the arterial side of the dermal micro-circulation. Even during the oedematous stage one can observe contrast stasis which is irreversible and not influenced by Priscolin.
Rofo 1976 Sep
PMID:[Pharmaco-angiography with Priscolin in progressive scleroderma (author's transl)]. 13 49

This presentation describes effects of oral tryptophan loading (5.0 g DL) on tryptophan metabolism in healthy subjects (n = 10) and persons with progressive scleroderma. N1-methylnicotinamide (N1MN), 3-hydroxyanthranilic acid (3 HAA), kynurine (KN), tryptamin (TA), xantheurenic acid (XA) were determinated. Alterations of tryptophan metabolism were evaluated by 24 h urinary excretions of the following metabolites: 5-hydroxy indolacetic acid (5 HAA) and indole-3-acetic acid (IAA). The pathological pathways were discussed, especially the way and influence of serotonine.
Klin Wochenschr 1976 Sep 15
PMID:[Tryptophan-load in progressive scleroderma (author's transl)]. 13 25

The behavior of proteoglycanes was examined by histochemical methods in excised particles of skin from patients with progressive (n = 24) and circumscribed (n = 74) scleroderma. A method described by Ishikawa was principally used. An increase of the proteoglycanes was shown preferentially in the vascular and perivascular regions in progressive scleroderma and in the interfibrous space of the connective tissue in circumscribed scleroderma. While the vascular sclerosis is prominent from the clinical point of view in progressive scleroderma, the disease processes are found in collagen connective tissue of the corium in morphea.
MMW Munch Med Wochenschr 1976 Sep 24
PMID:[Histochemical investigations in scleroderma (author's transl)]. 13 7

This report calls attention to the complete resorption of the mandibular condyle in progressive systemic sclerosis (scleroderma), a previously unreported finding. This was associated with osteolysis of the ipsilateral coronoid process, both mandibular angles, and autoamputation of the fingertips. The Panorex provides a simple, effective method for studying the mandible in systemic sclerosis. Similar mandibular osteolysis with vinyl chloride exposure is noted.
Oral Surg Oral Med Oral Pathol 1978 Sep
PMID:Total osteolysis of the mandibular condyle in progressive systemic sclerosis. 27 33

Baby hamster kidney cells fixed in acetone on glass slides were used as antigen for demonstration of antinuclear antibodies. Where certain storage conditions were observed (drying agent, 4 degrees C) they have kept for 12 months up to now. As regards specificity, sensitivity, reproducibility, and differentiation of fluorescent types the baby hamster kidney cell test appears superior to other immunofluorescence methods used (chicken erythrocytes, rat liver sections, and crithidiae). These results were obtained in 73 sera from patients with disseminated lupus erythematodes, drug-induced lupus erythematodes, discoid erythematodes, allergic vasculitis, progressive scleroderma, dermatomyositis, and 36 control sera.
Dtsch Med Wochenschr 1978 Sep 15
PMID:[Baby hamster kidney cells as antigen for demonstration of antinuclear antibodies (author's transl)]. 30 47

In a controlled study of barium swallow radiographs in systemic sclerosis, longitudinal oesophageal mucosal folds were absent in eight of 30 patients and in one of 30 control subjects (P less than 0.03). Patients without mucosal folds developed Raynaud's phenomenon at an earlier age than those who retained their mucosal folds (P less than 0.05). A loss of oesophageal mucosal folds did not necessarily signify more severe visceral or cutaneous disease.
Clin Radiol 1979 Sep
PMID:Absence of oesophageal mucosal folds in systemic sclerosis. 46 50

Eosinophilic fasciitis, originally reported as a syndrome distinct from scleroderma, appears now to be an early inflammatory variant of scleroderma. No less than one half of the cases reported as eosinophilic fasciitis have convincing features of scleroderma, including Raynaud's phenomenon, esophageal dysmotility, restrictive lung disease, diffuse hyperpigmentation, synovitis, flexion contractures, dermal sclerosis, colonic diverticula, scleroderma kidney, positive latex fixation test, and the presence of serum antinuclear antibodies (ANA). Clinical presentations of scleroderma range from isolated acrosclerosis to rapidly progressive systemic sclerosis. As clinical experience and long-term follow-up data on eosinophilic fasciitis accumulate, it appears that the syndrome may well represent another variant in the scleroderma spectrum. Reported here is a case which presented clinically and histologically as eosinophilic fasciitis, but which progressed over 3 years to diffuse, histologically confirmed scleroderma.
J Am Acad Dermatol 1979 Sep
PMID:Eosinophilic fasciitis: an early variant of scleroderma. 51 71

A woman with clinical manifestations of progressive systemic sclerosis had liver disease with histologic and immunologic features of primary biliary cirrhosis. Biopsy specimens of salivary gland showed necrosis and lymphocytic infiltrates in and around ducts similar to those observed in hepatic ducts, whereas neither of these tissues exhibited immunoglobulin or complement deposition. The ultrastructural and immunohistochemical studies suggest a common cell-mediated immunologic mechanism for the two disorders.
Arch Pathol Lab Med 1979 Sep
PMID:Primary biliary cirrhosis and scleroderma. The possibility of a common pathogenetic mechanism. 58 63

Progressive systemic sclerosis may be associated with focal myocardial fibrosis. Electrocardiographic abnormalities including conduction block are common in progressive systemic sclerosis but whether they are due to direct destruction of the specialized conduction tissue of the heart is uncertain. The conduction systems of 35 patients with progressive systemic sclerosis were studied. Of these 35 patients, 17 (50 per cent) had myocardial fibrosis of the type seen in progressive systemic sclerosis. In 10 of the 17, it was severe. Sinus node fibrosis was present in 13 patients and was nearly as frequent in those with as in those without the progressive systemic sclerosis myocardial lesion. Overlying pericarditis may have contributed to the fibrotic changes within the sinoatrial nodes in seven of the 13 patients. The atrioventricular node and main His bundles were normal. However, fibrotic changes were found in the proximal bundle systems in six patients. In three of the six, severe myocardial progressive systemic sclerosis was present, two had focal fibrous atrophy of the left bundle, and one had complete interruption of the right bundle. In only the latter patient was this reflected in the electrocardiogram which showed a right bundle branch block. Three patients without progressive systemic sclerosis myocardial lesions also had fibrous atrophy of a portion of the proximal left bundle branch, and in one the electrocardiogram showed an isolated left anterior hemiblock. Thus, morphologic abnormalities within the conduction system in our patients are difficult to attribute to progressive systemic sclerosis per se. Furthermore, although conduction abnormalities were more frequent in patients with myocardial disease, specific conduction system disease was not the cause in most patients. As has been noted in ischemic heart disease, the conduction system appears to be relatively spared from the myocardial changes of progressive systemic sclerosis, and the high incidence of conduction disturbances in this condition may be a consequence, rather, of damage to working myocardium.
Am J Med 1976 Sep
PMID:The cardiac conduction system in progressive systemic sclerosis. Clinical and pathologic features of 35 patients. 96

Thymus derived (T) and bone-marrow derived (B) lymphocytes were quantitated in 11 untreated patients with progressive systemic sclerosis (PSS) and in 11 age and sex matched controls. T and B lymphocytes were identified according to their receptor sites for sheep red blood cells and activated complement, respectively. All patients had a normal lymphocyte count. The mean per cent value of circulating T lymphocytes was less in PSS patients (38 per cent) as compared with controls (56 per cent) (P less than 0.005), while no significant difference existed for the circulating B lymphocytes (PSS 14 per cent; controls 11 per cent). The mitogenic response to phytohemagglutinin (PHA) was assessed by comparing dose response curves in PSS with those obtained from matched controls. No significant difference could be detected between these groups.
J Rheumatol 1975 Sep
PMID:Circulating T and B lymphocytes in progressive systemic sclerosis. 108 Nov 43


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