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Results for “Scleroderma, Diffuse”

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At least 469 records · Page 26Linked to original sources
PubMed2006

Functional hyperprolactinemia and hypophyseal microadenoma in systemic sclerosis.

OBJECTIVE: Hyperprolactinemia (HPRL) has been identified in more than half of patients with systemic sclerosis (SSc). However, the association with pituitary adenoma and the status of hypothalamic dopaminergic tone using metoclopramide (MTC) test has not been studied. We investigated the prevalence of prolactin (PRL)-secreting pituitary adenoma and evaluated production of PRL by dynamic testing with MTC in SSc. METHODS: We studied 30 patients with SSc (mean age 38 +/- 10 yrs) and 20 healthy controls (mean age 37 +/- 11 yrs). Serum PRL concentrations were determined by radioimmunoassay in all subjects, and PRL response was measured 30, 60, 90, and 120 min after injection of 10 mg of MTC. Computed tomography (CT) of the sella turcica was performed. RESULTS: The mean basal serum PRL levels before and after stimulation with MTC in SSc patients versus controls were: basal 18.2 +/- 5.4 versus 8.7 +/- 1.6 ng/ml, p = NS; 30 min: 175.0 +/- 5.4 versus 61.0 +/- 42 ng/ml, p < 0.001; 60 min: 160 +/- 64 versus 52 +/- 30 ng/ml, p < 0.001; 90 min: 125 +/- 57 versus 42 +/- 21.0 ng/ml, p < 0.05; 120 min: 108.0 +/- 57 versus 30.0 +/- 10 ng/ml, p < 0.005. CT scan showed microadenomas in 24/30 SSc patients and 1/20 controls (p = 0.001). CONCLUSION: Our study suggests that a group of patients with SSc have a high prevalence of HPRL with increased central dopaminergic tone, and microadenomas. PRL may have a role in the pathogenesis of SSc. Further studies are necessary to confirm our results.

Adenoma↗
PubMed2006

Ischemic scleroderma wounds successfully treated with hyperbaric oxygen therapy.

Hyperbaric oxygen therapy (HBOT) has been used to treat refractory wounds for the last several decades, with the majority of research focusing on wounds secondary to arterial insufficiency. We describe 2 patients with scleroderma with intractable bilateral extremity ulcers. Local ischemia was identified using transcutaneous oximetry. Each patient then underwent 30 treatments of HBOT at a relative depth of 2.4 ATA with resulting wound healing. This is the first reported successful use of HBOT to treat scleroderma ulcers, and may represent an unrecognized treatment option for these notoriously difficult chronic wounds.

Adult↗
PubMed2006

Natural remedies for scleroderma.

Scleroderma is an autoimmune disease of the connective tissue characterized by fibrosis and thickening of various tissues. It can be limited to the skin or affect multiple organs, and its course ranges from slowly to rapidly progressive. Penicillamine, glucocorticoids, and other drugs are used to treat scleroderma, but none of these treatments has a high degree of efficacy. This article reviews several promising natural treatments for scleroderma, including para-aminobenzoic acid, vitamin E, vitamin D, evening primrose oil, estriol, N-acetylcysteine, bromelain, and an avocado/soybean extract.

Humans↗
PubMedDate not supplied

[Clinical usefulness of determining the antibodies to soluble nuclear antigens in various collagen diseases].

The purpose of the study was evaluation of the clinical usefulness of determination of antibodies to soluble nuclear antigens. The study was carried out in 71 cases of various collagen diseases. Antibodies dsDNA (IIF method with Crithidium luciliae as substrate) were found only in patients with SLE and renal involvement. RNP antibodies (double immunodiffusion method) were demonstrated in 83.3% of cases of mixed connective tissue disease, and Sm antibodies in 8% of SLE patients. It is worth stressing that in the presented material Sm antibodies were present only in association with RNP antibodies. Antibodies Ro and/or La were present most often in the sera of patients with SCLE, while Scl 70 antibodies were a marker of systemic sclerosis, more frequent in patients with diffuse scleroderma, while their demonstration in acroscleroderma suggested a more severe course of the disease. The study showed a high diagnostic and prognostic value of antibodies to soluble nuclear antigens in collagen diseases.

Antibodies, Antinuclear↗
PubMed1990

[Anticardiolipin antibodies in diffuse connective tissue diseases with IgG, IgM and IgA isotypes].

Anticardiolipin (aCL) antibodies were assessed in isotypes IgG, IgM and IgA by the enzyme immunochemical technique in serum of 86 subjects with diffuse connective tissue affections and in 75 subjects of three control groups (syphilis, syndrome of common variable immunodeficiency and blood donors). In systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) and syphilis the mean values of the three isotypes of aCL antibodies were significantly higher than in blood donors (p = 0.05 to 0.001); in diffuse scleroderma and primary polymyositis/dermatomyositis in isotype IgG (p = 0.01-0.001). Positive findings of aCL antibodies (isolated or in combinations of Ig isotypes (were found most frequently in SLE (34.4%), RA (33.3%) and syphilis (66.6%); sera of blood donors were positive in 8.7%. Venous thrombosis was recorded in the case-records of 28% patients with SLE but only in 5.4% of those with RA. Spontaneous abortion terminated 8/66 pregnancies in 28 women with SLE. In one female patient with SLE the aCL syndrome was detected. On account of frequent positivity of aCL antibodies in syphilis, the authors consider it essential to rule out the coincidence of this disease. Examination of aCL-IgA antibodies extends the detection of positive cases (isolated or in combinations of Ig) in SLE and RA.

Adult↗
PubMed1990

[A case of progressive systemic sclerosis associated with mutilans-type arthropathy and suspected Felty's syndrome].

A patient who developed mutilans-type arthropathy, splenomegaly, leukopenia, leg ulcer and massive hydroxyapatite accumulation during the course of progressive systemic sclerosis (PSS) was reported. A 56-years-old female had suffered Raynaud's phenomenon since the beginning of her third decade. She developed multiple symmetrical arthritis and morning stiffness at the age of 29, and was treated with NSAIDs and low dose corticosteroids under the diagnosis of rheumatoid arthritis (RA) Because of dysphagia and diarrhea, she was admitted in Niigata-Kenritsu Senami Hospital in September, 1987. Physical and roentgenographic examinations revealed diffuse scleroderma, mutilans-type arthropathy, lung fibrosis, splenomegaly and right leg ulcer. Laboratory examinations showed leukopenia, high titer of anti-DNA antibody, positive anti-Scl-70 antibody and mild hypocomplementemia. These findings suggested that she had PSS and Felty's syndrome. Furthermore, massive subcutaneous and intraarticular hydroxyapatite accumulation were noticed. The leg ulcer and laboratory data gradually improved with the combination therapy of corticosteroids, D-penicillamine and plasmapheresis. Although it has been well recognized that PSS patients reveal frequently the articular lesions similar to these of RA, severe mutilans-type arthropathy seen in this case is extremely rare. The joint contracture might be induced by hydroxyapatite accumulation, of which the early diagnosis seems to be very important in long-standing PSS patients.

Antibodies, Antinuclear↗
PubMedDate not supplied

[Angiopathy caused by vibration and CREST syndrome: beware of hidden pathologies!].

The etiologic definition of an occupational disease should be achieved via an accurate differential diagnosis. A careful assessment of any clinical features that are atypical or unusual for the disease in question can sometimes lead to the identification of other morbid states that are either hidden or show few symptoms, and may or may not interfere with the occupational picture. The case described is of a man who, after working for 10 years with vibrating tools, developed a vibration disease with typical circulatory alterations in the hands and osteoarticular and neurological alterations. However, the appearance of a necrotic ischaemic lesion at the tip of the second finger of the right hand and signs of sclerodactylia in the second finger of both hands led us to suspect the presence of another concomitant disease. The finding of anticentromere antibodies suggested the presence of a CREST syndrome, which is a serologic variant of diffuse scleroderma. More in-depth investigations and examination of the state of other organs and apparatuses confirmed the diagnostic suspicion, with evidence of impairment also of the cardio-pulmonary system. The presence of the CREST syndrome was interpreted as a state of hypersusceptibility to the traumatic action of vibrating tools on the vascular system.

Arm↗
PubMed1989

[Peritoneal dialysis, the method of treatment for end-stage renal insufficiency: development of indications during the past 10 years in relation to the initial renal disease and extra-renal pathology].

A decade after its first introduction, the advantages and drawbacks of continuous ambulatory peritoneal dialysis over hemodialysis remain controversial. This present paper is a review of the literature, focused on the indications of this dialysis modality in different circumstances: extra-renal pathology, systemic diseases (lupus erythematosus--diffuse scleroderma--plasma cell disorders--amyloidosis--HIV infected patients) and complications related to hemodialysis.

Humans↗
PubMedDate not supplied

Cineradiography identifies esophageal candidiasis in progressive systemic sclerosis.

Cineradiography of the esophagus showed signs of esophageal candidiasis in 11 out of 71 patients with progressive systemic sclerosis (PSS) - both in diffuse scleroderma and the CREST syndrome. Culture of esophageal brushings confirmed the presence of Candida albicans in eight of these 11 patients. Antimycotic treatment decreased the cineradiographic signs of candidiasis and the degree of dysphagia. Since impaired esophageal motility and treatment with immunosuppressive drugs may predispose to candida esophagitis, and since dysphagia will decrease after antimycotic treatment esophageal mycosis should always be sought in patients with PSS.

Candidiasis↗
PubMed1989

[Radiological changes in feet of patients with progressive systemic sclerosis].

Forty six patients (41 female and 5 male) with progressive systemic sclerosis were analysed through X-ray photographs of feet. Age ranged from 33 to 74 (mean: 48 +/- 13) years old, and durations of the disease were one to 28 (mean: 9.8 +/- 8) years. Abnormal radiological findings of feet were noted as following; bone absorption of the terminal tufts of the first phalanx (6 out of 46 cases: 13%), bone absorption of metatarsals and/or mid, basal phalanges (2 cases: 4.3%), bone erosion (6 cases: 13.0%), calcification (7 cases: 15.2%), diffuse osteoporosis (2 cases: 4.3%) and cystic lesion (one case: 2.2%). All patients with abnormal findings on X-ray photographs of feet simultaneously showed radiological changes of hands. It took 13.3 +/- 7 years until the radiological findings appeared in feet. On the other hand, it took much shorter 8.8 +/- 6 years in hands. Patients with radiological changes in feet tended simultaneously to have pulmonary and digestive tract involvements, and widespread skin lesions such identified as diffuse scleroderma. Anti-Scl 70 antibody were positive in a half of these patients.

Adult↗
PubMed1987

[Motility disorders of the esophagus].

The paper describes the physiology of swallowing, the methods for the assessment of esophageal motility, and the motility disorders of the tubular part and the lower sphincter of the esophagus, except for gastroesophageal reflux disease. Primary esophageal motility disorders are achalasia (incomplete relaxation of the lower sphincter in response to swallowing), diffuse esophagospasm (simultaneous repetitive contractions), and the nutcracker esophagus (propulsive peristalsis with abnormally high amplitude). Besides, there are non-specific as yet unclassified contraction abnormalities. Since hypermotile contraction abnormalities can mimic chest pain of cardiac origin, differential diagnosis of anginal chest pain should include esophageal motility disorders. Contraction abnormalities of the esophagus may occur in diffuse scleroderma, after therapeutic radiation of the mediastinum, and possibly after sclerotherapy of esophageal varices.

Esophageal Achalasia↗
PubMedDate not supplied

The value of echocardiography in the early diagnosis of myocardial impairment due to connective tissue diseases.

The study comprised 14 patients with systemic lupus erythematosus (SLE) and 12 patients with diffuse scleroderma. Echocardiography ascertained 5 cases of congestive and 1 case of restrictive cardiomyopathy among the patients with SLE, as well as 6 cases with restrictive cardiomyopathy among those with scleroderma. The authors specify two important echocardiographic variables: the E-septum distance and the septal and wall motion after amyl-nitrite test, which reveal the intrinsic contractile properties of the myocardium, allowing an early diagnosis of myocardial impairment, as well as the selection of patients for an efficient treatment with vasodilator drugs.

Cardiomyopathy, Dilated↗
PubMedDate not supplied

[Streptococcus antibodies in a rural population and in patients with inflammatory rheumatic diseases].

An account is given of assessed titres of anti-streptolysin O (ASO) and antihyaluronidase (AH) in rheumatic fever, in rheumatic patients of inactive phase and in tonsillitis. The ASO titre was examined in 1739 members of the general public and in 360 patients with chronic rheumatic diseases. The author analyses 6235 examinations of ASO titres and 1210 AH examinations. In rheumatic fever the mean maximum ASO titre was 397 u, while the mean maximum AH titre was 3583 u. In inactive phase rheumatic patients the mean ASO titre was 187 u, the mean AH titre 630 u. The mean ASO titre at the onset of the disease in tonsillitis was 172 u. During the second examination after 3-4 weeks, the mean rise of the ASO titre was 205 u. A increased AH titre above 1280 u was recorded during the first examination in 35.2%, during the second examination in 52.3%. The mean ASO titre in the population group comprising 1739 subjects was 148 u. A titre increased above 200 u was recorded in 20.7%. The mean ASO titre in rheumatoid arthritis was 139 u, in ankylosing spondylitis 199 u, in systemic lupus erythematosus and diffuse scleroderma 128 u. In all groups the ASO and AH levels were inversely proportional to the age of the examined subject. Attention is drawn to the correct evaluation of the assessed antibody titres.

Adolescent↗
PubMed1977

[An case of acute diffuse seleroderma in an infant].

The authors report a case of diffuse scleroderma in a 15 months old infant. Dermatologic (clinical and pathological) findings are quite typical of the disease. On the other hand, in this case some particularities were observed: the age of the infant (second published case beginning before the age of two); the presence of a durable eosinophilia, the absence of visceral lesions and of biological abnormaliteis (of auto-immune nature specially), the evolution towards athrepsica and death within one year. Thus, because of these particularities, the diagnosis of scleroderma remains questionable and the diagnosis of progeria has been considered. The affection appeared in the course of a hepatitis leaving a hepatic fibrosis without inflammatory signs; no conclusion can be drawn about the relations between the hepatic affection and the fatal dermatologic disease.

Acute Disease↗
PubMedDate not supplied

Progressive systemic sclerosis and penicillamine.

Retrospective evaluation of 26 patients with progressive systemic sclerosis and diffuse scleroderma who were treated with D-penicillamine for periods of 6-50 months (average = 19 months) revealed a reduction in skin thickening, which was confirmed in 7 cases by a diminution in the weight of skin biopsy cores obtained from the forearm before and after therapy. There was little or no change in skin thickness in 18 otherwise similar patients who received other medications. Treatment with D-penicillamine also appeared to be associated with a decrease in the rate of development of new visceral involvement and prolongation in life expectancy in this series.

Collagen↗
PubMed1995

Pericardial effusion and vasculitis in a patient with systemic sclerosis.

We describe a case of diffuse scleroderma with a large pericardial effusion, pleural effusions, and subsequent oliguric renal failure. Histology of the pericardium and pleura revealed the presence of leukocytoclastic vasculitis. Pleuropericarditis in systemic sclerosis may occur on the basis of vasculitis. Large pericardial effusions may predispose to subsequent renal failure.

Female↗
PubMed1975

[The indirect immunofluorescence test for the identification of auto-immune skin diseases (author's transl)].

The indirect immunofluorescence test has acquired great importance in the diagnosis of auto-immune skin diseases. Since it is sufficient only to send a sample of whole blood or blood serum to carry out the test, it is also of practical interest to the general practitioner. The test should be called upon if any of the following auto-immune skin diseases are suspected: pemphigus vulgaris (including p. vegetans, p. foliaceus, p. erythematosus), bullous pemphigoid (including benign mucosal pemphigoid) lupus erythematodes (systemic) and diffuse scleroderma.

Autoimmune Diseases↗
PubMed1997

Systemic sclerosis with pulmonary involvement and right ventricular failure in a child.

We report a very rare case of systemic sclerosis in a 6-year-old girl. She presented with diffuse scleroderma, Raynaud's phenomenon, pulmonary interstitial fibrosis, pulmonary hypertension, and right ventricular failure. The diagnosis was confirmed by skin manifestations, high resolution computed tomography, cardiac catheterization, and anti-nuclear antibodies. Nifedipine, prednisolone, digoxin, and furosemide were given. There was remission of the right ventricular failure and dyspnea, and the skin showed partial improvement. The patient remained asymptomatic for a year. The symptoms of respiratory and right heart failure developed again after an episode of lower respiratory tract infection and she eventually died. We discuss the clinical manifestations, treatment, and outcome.

Child↗