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

A number of reports of potential etiologic agents of localized and systemic scleroderma appeared in the past year, including alterations in tryptophan metabolism, use of appetite suppressants, and exposure to silicone. An infectious agent, Borrelia burgdorferi, was found not to be implicated in localized scleroderma. The improvement in outcome of systemic scleroderma complicated by renovascular hypertension was highlighted in several papers, as was the emerging importance of cardiac and pulmonary involvement. Recent advances in the early detection and evaluation of cardiac and pulmonary complications of scleroderma are discussed.
 ...
PMID:Clinical aspects of localized and systemic scleroderma. 177 53

We present the case of a 52-year-old woman who developed diffuse induration of the skin and severe edema of the subcutaneous tissue involving the extremities and the trunk, sparing hands, feet and face after 10 years of almost constant oral tryptophan medication. The skin manifestations were similar to those of eosinophilic fasciitis (Shulman syndrome). The patient complained of severe muscle pain and weakness. Laboratory studies revealed an elevated Westergren erythrocyte sedimentation rate and eosinophilia. There were no signs of internal organ involvement and no immunological parameters of progressive systemic scleroderma. Eosinophilia and myalgia resolved in response to intermittent systemic therapy with glucocorticosteroids, whereas the progressive scleroderma and the edema showed only slight improvement after the discontinuation of L-tryptophan.
 ...
PMID:[Eosinophilia-myalgia syndrome and L-tryptophan intake]. 182 70

We investigated the relationship between use of L-tryptophan and development of eosinophilic fasciitis by two methods: a retrospective patient survey and a case-control study of patients with eosinophilic fasciitis diagnosed at the Mayo Clinic between 1977 and 1989. Before 1986, no traceable patients with eosinophilic fasciitis had taken L-tryptophan. Between Jan. 1, 1986, and July 31, 1989, 8 of 34 patients had ingested L-tryptophan (P less than 0.001). In the case-control study, traceable patients with eosinophilic fasciitis were matched with patients who had systemic sclerosis or rheumatoid arthritis and healthy control subjects who had undergone a general medical examination. Of the 60 matched control subjects, 2 had used L-tryptophan. Thus, the odds ratio was 19, indicating a 19-fold greater likelihood of use of L-tryptophan in patients with eosinophilic fasciitis than in the control group. A retrospective assessment of clinical features, response to treatment, and blinded review of biopsy specimens of skin and fascia in patients who had eosinophilic fasciitis with or without exposure to L-tryptophan disclosed no significant differences in the two groups. This retrospective study confirms a strong association between consumption of L-tryptophan and development of eosinophilic fasciitis in some patients. No clinical or histopathologic features were detected that distinguished this disorder on the basis of previous exposure to L-tryptophan.
 ...
PMID:Eosinophilic fasciitis associated with use of L-tryptophan: a case-control study and comparison of clinical and histopathologic features. 192 98

Scleroderma developed in six women who were taking L-tryptophan. Fasciitis and morphea were most common, but one patient had pleural effusion, hypertension, and signs of cardiac and kidney failure. In five patients the biopsy findings were characteristic of scleroderma; the sixth patient had Crohn's disease and developed fasciitis; her biopsy specimen showed inflammatory arteritis. All patients' conditions improved after cessation of their L-tryptophan intake, initiation of corticosteroid therapy, or both. These findings confirm previous data that show altered tryptophan-kynurenine metabolism in some patients with scleroderma and fasciitis, particularly with tryptophan loading.
 ...
PMID:Scleroderma and L-tryptophan: a possible explanation of the eosinophilia-myalgia syndrome. 221 43

Numerous drugs have been suggested for the treatment of systemic scleroderma. They may be studied and classified according to their site of action on the chain of events that leads from vascular abnormalities to sclerosis of the skin. Thus, proline analogues, colchicine, lathyrogenic agents, D-penicillamine, coagulation factor XIII and oestrogens are thought to act on collagens and their metabolism. Ketanserin has been suggested by the discovery of tryptophan abnormalities. Corticosteroids exert an inhibitory effect on fibroblasts. The use of calcium antagonists, angiotensin-converting enzyme inhibitors, prostacyclin and anti-platelets rests on the presence of vascular abnormalities. The purpose of treatments with immunosuppressive drugs or plasma exchanges is to act on possible lymphocytic and/or macropageal factors.
 ...
PMID:[Physiopathologic bases of the treatment of systemic scleroderma]. 353 53

Eosinophilia myalgia syndrome (EMS) has been related to intake of "contaminated" L-tryptophan, and an alteration in tryptophan 5-hydroxytryptamine (5-HT, serotonin) metabolism has been reported in EMS patients. Recently we found that a defined autoantibody pattern consisting of antibodies to nucleoli, gangliosides, and phospholipids is closely related to the fibromyalgia syndrome (FS) which clinically resembles the EMS. We were therefore interested to see whether these antibodies can also be detected in patients with EMS. Studied were 27 patients with acute EMS (13 of whom were also examined 2 years after acute onset), 100 patients with FS, and 40 patients with progressive systemic sclerosis (PSS). As controls, sera from 100 blood donors were analyzed. Antibodies to nucleoli were demonstrated by immunofluorescence test on cell cultures in 52% of patients with acute EMS, 62% of patients with chronic EMS, and 37% of FS patients. Western blotting with a nuclear extract from HeLa cells revealed in both diseases the same epitopes at 63, 57, and 53 kDa. Antibodies to 5-HT, gangliosides (Gm1), and phospholipids were determined by enzyme-linked immunosorbent assay. Among patients with FS 73% had antibodies to 5-HT, in contrast to only 19% of patients with acute EMS. However, 77% of the 13 EMS patients analyzed 2 years later had become anti-5-HT antibody positive during that time. Also the incidence of antibodies to Gm1 increased from 37% at acute onset to 69% in patients with chronic EMS (30%). The various antibodies were detected in only 18% of healthy controls.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:A comparative study on antibodies to nucleoli and 5-hydroxytryptamine in patients with fibromyalgia syndrome and tryptophan-induced eosinophilia-myalgia syndrome. 798 84

Saccharomyces cerevisiae cho1/pss mutants, which are severely impaired in phosphatidylserine (PS) synthesis, do not have detectable amounts of PS in their lipid fractions. Their derivatives with mutations that cause defects in tryptophan synthesis grew poorly in a medium containing 5 micrograms/ml of L-tryptophan, a concentration that met the requirements of tryptophanauxotrophic CHO1/PSS strains. The rates of tryptophan uptake of trp1 cho1/pss mutants were low at low tryptophan concentrations. This defect in the use of tryptophan was restored either by expression of CHO1/PSS or by introduction of a gene encoding tryptophan transporter, TAT1 or TAT2. These results indicate that PS synthesis is required for the maximal tryptophan-transporting activity of S. cerevisiae at low tryptophan concentrations.
 ...
PMID:Phosphatidylserine synthesis required for the maximal tryptophan transport activity in Saccharomyces cerevisiae. 1070 62

There is growing concern about the association between systemic sclerosis and certain environmental and occupational risk factors, including exposures to vinyl chloride, adulterated cooking oils, L-tryptophan, silica, silicone breast implants, organic solvents, and other agents such as epoxy resins, pesticides, and hand/arm vibration. This article highlights the current medical research that has examined these associations in scleroderma-like disorders and in systemic sclerosis.
 ...
PMID:Systemic sclerosis: environmental and occupational risk factors. 1109 2

First time eosinophilic fasciitis (EF) was described by Shulman. It occurrences predominantly in young men, rarely older women, sporadic in children. The illness was characterised by swelling of the extremities, skin changes, myalgia, and elevation of the peripheral blood eosinophil count and hypergammaglobulinemia. It is followed by rapidly progressive cutaneous and subcutaneous induration. The histopathologic examination in the region of affected skin shows thickening of the fascia, deep dermal fibrosis and infiltration mainly consists of lymphocytes, plasmocytes, eosinphils. It is suggested that the cells from infiltration induce fibrosis. The tryptophan disturbance has significant influence on development of EF in predisposed persons. Eosinophilic fasciitis is classified to wide spectrum of scleroderma disorders by many authors. The authors tried to show differences between EF, morphea and systemic scleroderma.
 ...
PMID:[Eosinophilic fasciitis--current knowledge]. 1293 59

For the first time eosinophilic fasciitis (EF) was described by Shulman. It occurs predominantly in young men, rarely in older women and sporadically in children. The illness was characterised by swelling of the extremities, skin changes, myalgia, and elevation of the peripheral blood eosinophils count and hypergammaglobulinemia. It is followed by rapidly progressive cutaneous and subcutaneous indurations. The histopathologic examination of affected skin shows thickening of the fascia, deep dermal fibrosis and infiltration mainly consists of lymphocytes, plasmocytes, eosinophils. It is suggested that the cells from infiltration induce fibrosis. The tryptophan disturbance has significant influence on development of EF in predisposed persons. Eosinophilic fasciitis is classified into wide spectrum of scleroderma disorders by many authors. The authors tried to show differences between EF, morphea and systemic scleroderma.
 ...
PMID:[Eosinophilic fasciitis--current database]. 1551 Sep 3


<< Previous 1 2 3 Next >>