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Results for “Scleroderma, Systemic”

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PubMed1989

[Analysis of the distribution of class I HLA-antigens in patients with systemic scleroderma with regard to features of the clinical course of the disease and therapy with D-penicillamine].

The distribution of HLA-A, B, C antigens has been studied in 40 patients with systemic scleroderma and in 200 healthy individuals (all Russians). An increased frequency of the antigens B35 and Cw4 has been discovered in patients, as compared with control. When analysing different clinical and common parameters, lung affection in the systemic scleroderma patients was found to be associated with the antigen A10 (58.9% versus 21% in control, RR = 5.22, EF = 0.476, Pc = 0.0363), the presence of antinuclear antibodies being associated with the antigen B35 (50% versus 17% in control, RR = 4.8, EF = 0.396, Pc = 0.0354). The association with the antigen B8 most commonly mentioned in the literature was characteristic of the patients with an earlier onset of the disease (under 30 years) and those with the rheumatoid factor. The patients having D-penicillamine-induced complications were found to have an increased frequency of the antigen B8, as compared with the alternative group of patients (2P = 0.0430).

Adolescent↗
PubMed1989

[Electrocardiographic and echocardiographic evaluation of cardiac changes in systemic scleroderma].

Cardiac abnormalities were studied using the clinical, electrocardiographic and echocardiographic methods in 66 patients with systemic scleroderma. Abnormal ecg was seen in 41 patients (62.1%), most frequently in form ventricular extrasystole (21.2%), incomplete block of the right bundle branch (12.1%), left ventricular and right atrial hypertrophy (10.6%) each, and the pattern suggesting the history of past myocardial infarction (7.6%). Abnormal echocardiographic results were found in 38 patients (57.6%). The most usual changes, differentiating that group from the controls, were: sluggish diastolic movement of the posterior wall of the left ventricle (40.7%), pericardial effusion (37.0%), elongation of the isovolumic relaxation time diastole phase (99.3 s.m.v. 81.1 ms, p less than 0.001) and right ventricular dilatation. There were no significant differences between the scleroderma and the control group as regards the indices of the contractility of the left ventricle: ejection fraction (EF), velocity of circumferential fibres shortening (VCF), diastolic size of the left ventricle and of the left atrium. The prevalence of the electrocardiographic and echocardiographic abnormalities in particular types of scleroderma (diffuse, acroscleroderma, severe acroscleroderma, CREST) was roughly similar: 50-76%. Finding of the impaired diastolic rather than systolic function of the left ventricle and of the similar prevalence of the cardiac abnormalities in the particular types of scleroderma is new and contradictory to the commonplace opinions.

Adult↗
PubMed1994

[Pathogenesis of anemia in systemic scleroderma].

The paper reports a case of anemia with complicated genesis in a male patient with systemic scleroderma (SS). Antianemia therapy was not easy, as after rapid disappearance of iron deficiency vitamin B12 deficiency remained, hemolytic component of the disease persisted. The case demonstrates that multicomponent pathogenesis of anemic conditions is possible. This must be taken into consideration when planning therapeutic policy in SS.

Adolescent↗
PubMed1999

Global improvement of systemic scleroderma under long-term administration of octreotide.

Octreotide has proven to be effective for the treatment of intestinal dysmotility in patients with scleroderma in short-term administration. We report a global improvement of scleroderma manifestations under long-term administration of octreotide. A 53-year-old black woman was diagnosed with a four-year history of progressive and severe systemic scleroderma, with diffuse skin sclerosis, myositic involvement, impaired carbon monoxide transfer factor (57% of the predicted normal value and severe digestive involvement with pseudo-obstruction and bacterial overgrowth into the intestinal lumen). After one month of octreotide (75 mug/d), oral feeding was restarted and weight gain of 6.5 kg was achieved. After 8 months of treatment, normal weight was obtained and skin induration was spectacularly reduced and pigmentation returned to a normal state. Dyspnea disappeared and physical activity was quite normal. Octreotide effects on intestinal transit are unclear and may be secondary to immunomodulation or neurotransmission effects. Extradigestive effects of octreotide in scleroderma have not been studied. This report suggests that long-term administration of octreotide may be beneficial in the treatment of patients with systemic scleroderma. Long-term trials are required to confirm these preliminary results.

Administration, Oral↗
PubMed1985

Systemic scleroderma and small cell carcinoma of the lung.

A 64-year-old man diagnosed to have systemic scleroderma for 1 year developed small-cell carcinoma of the lung. Six additional cases obtained from the literature describing small-cell carcinoma occurring in patients with scleroderma are reviewed.

Carcinoma, Small Cell↗
PubMed1996

Systemic scleroderma and perforating granuloma annulare: differential diagnosis from calcinosis.

BACKGROUND: Systemic scleroderma is a disorder of unknown etiology with skin sclerosis. Its major histological features are swollen and homogenized collagen bundles. OBJECTIVE AND METHODS: We describe 2 patients with systemic sclerosis who have multiple umbilicated nodules indistinguishable from calcinosis cutis. RESULTS: Histological examinations including Von Kossa staining revealed features of perforating granuloma annulare, but not of calcinosis cutis. CONCLUSION: The association may not be fortuitous but both diseases may be etiologically related.

Adult↗
PubMed1991

Extracellular microfibrils are increased in localized and systemic scleroderma skin.

Extracellular microfibrils, about 10 nm thick with a hollow core have been found in most organs as free bundles or in association with elastic fibrils. Histochemistry of the dermis of 4 patients with localized and 6 with systemic scleroderma revealed numerous fine elastic fibrils in areas of fibrosis. Immunofluorescence and immunoelectron microscopy were performed with antibodies against fibrillin and amorphous elastin. The lower dermis revealed an increase in 10-nm microfibrils interspersed between collagen fibrils. These microfibrils stained for fibrillin but not for amorphous elastin. Fibrosis in localized and systemic scleroderma involves the deposition of collagen fibrils and microfibrils.

Extracellular Space↗
PubMed2001

Surfactant protein D (SP-D) and systemic scleroderma (SSc).

We measured serum levels of SP-D in collagen diseases (110 cases) such as systemic scleroderma (SSc), scleroderma spectrum disorders (SSD), systemic lupus erythematodes (SLE), Sjogren syndrome (Sjs), dermatomyositis (DM), rheumatoid arthritis (RA), and dermatitis (DE) (109 cases) as a control. Additionally, we performad a correlation analysis to determine how these levels were related to pulmonary fibrosis and function test (vital capacity, %DLco). The serum levels of SP-D increased in SSc patients with Barnett type III more than in SSc patients with Barnett type I or II, while they increased slightly in SSD (incomplete type of SSc) patients. The differences in these figures were statistically significant between the SSc (SSc & SSD) and non-SSc (SLE, DM, Sjs & RA) groups (p<0.005). The serum levels of SP-D in SSc patients with anti-topoisomerase I antibodies were statistically higher than those in SSc patients with other types of anti-nuclear antibodies. There was a statistically significant correlation between the severity of pulmonary fibrosis and the serum levels of SP-D, and a statistically negative correlation between SP-D levels and vital capacity or %DLco, but there was no proportional correlation with the forced expiatory volume (FEV1.0%). There was no statistical relationship between pre- and post-therapy with photopheresis; however, there was a statistical correlation between the serum levels of SPD and KL-6. In the group of collagen diseases, plasma levels of SP-D were higher than serum levels of SP-D. Patients with SSc possess higher levels of SP-D than do those with other collagen diseases and dermatitis, which may correspond to the severity of pulmonary fibrosis.

Adolescent↗
PubMed1983

[Pigmented exanthema after spironolactone allergy in progressive systemic scleroderma].

A 37-year-old man developed generalized medium-brown hyperpigmentation in the course of progressive systemic scleroderma. Histologically there were numerous Melanin granules in the basal epidermis, but also in the upper Malpighian layer and in corneocytes. An allergic rash developed on administration of spironolactone given for the scleroderma. After healing there remained slate-grey spots. As a consequence of the drug rash the already present hyperpigmentation was aggravated by marked pigment deposition in the upper corium. At the same time there were numerous melanophages as sign of an absorptive removal of the melanin granules. The drug allergy was thus the occasion for the persisting pigment deposition with circumscribed spotty pigmentation of the skin.

Adult↗
PubMed1975

[Ultrastructure of the skin in systemic scleroderma].

Biopsy specimens of the skin of the back of the forearm and hand in 20 patients with systemic scleroderma were studied using histological and electron microscopy methods. There were revealed evidences of intensified neofibrillogenesis, changes in collagen structures of the derma (destruction and defects of packing of collagenous fibrills, variability of their thickness in a fibre) and in the microcirculatory bed. In 18 out of 20 cases the nuclei of the connective-tissue cells and cells of the basal layer of the epidermis contained the so-called nuclear bodies, which are considered by a number of authors as an indirect evidence of a viral infection. The process of an intensified neoformation of collagen fibrils considerably predominated over destructive processes in collagen structures.

Adult↗
PubMed1986

[Treatment of systemic scleroderma with ketanserin. Randomized, double-blind 6-months study of 27 cases].

Twenty-seven patients with systemic scleroderma and Raynaud's phenomenon underwent a randomised double blind therapeutic trial: monotherapy with Ketanserine (80 mg/day for 6 months) against Placebo. The secondary effects were comparable in both groups as were the withdrawals from the trial for aggravation of Raynaud's phenomenon (one in each group). No significant difference was observed between the two groups as regards the evolution of the Raynaud's phenomenon or skin changes. Dysphagia was improved in the Ketanserine group (p less than 0.05) but not in the Placebo group. Some patients in the Ketanserine group experienced an improvement in the Raynaud's phenomenon at the end of the trial period; there were no improvements in the Placebo group. Three haemorrheological parameters (total blood viscosity, plasma viscosity and thixotropism) were abnormal at the beginning of the trial and did not improve by the end in the Ketanserine group. The K infinity coefficient of Quemada's law was normal at the start of the trial and increased after treatment (p less than 0.05).

Clinical Trials as Topic↗
PubMed2003

[Cardiac tamponade revealing systemic scleroderma].

The frequency of pericardial involvement in Systemic Sclerosis (SSc) is high but usually was asymptomatic and cardiac tamponnade was exceptional. We report a case of systemic sclerosis (scleroderma) revealed by cardiac tamponnade. This case illustrate the value of transthoracic echocardiography in the diagnosis of cardiac tamponnade in systémic sclerosis.

Adult↗
PubMed1991

[Type IV collagen and laminin levels in the sera from patients with systemic scleroderma (PSS)].

Serum type IV collagen 7S and laminin P1 levels were measured with radioimmunoassay in 33 patients with systemic scleroderma (PSS), 6 localized scleroderma (LS), and one mixed connective tissue disease (MCTD). Serum type IV collagen 7S levels were higher in PSS (5.11 +/- 1.11 ng/ml:m +/- SD) and LS (4.68 +/- 0.46 ng/ml) than in normal controls (3.90 +/- 0.85 ng/ml) (p less than 0.001). Serum laminin P1 levels were also significantly higher in PSS (1.75 +/- 0.34 U/ml) and LS (1.38 +/- 0.20 U/ml) compared to the controls (1.19 +/- 0.16 U/ml) (p less than 0.001 and p less than 0.01, respectively). A significant correlation between these two values in PSS was found (r = 0.465, p less than 0.02). These results suggest that the measurements of these values may serve as a marker of PSS.

Adult↗
PubMedDate not supplied

[Studies of the mechanism of disorders of the natural killer cell activity in patients with systemic scleroderma].

The NK activity of peripheral blood mononuclear cells (PBMC) was evaluated in 90 patients with various subsets of systemic scleroderma (SSc). The NK activity, as performed with K-562 as target cell, was found to be significantly lowered in patients with diffuse scleroderma, but did not differ from the healthy control in patients with acrosclerosis. The lowest values in the NK activity assay were obtained in patients with most extensive skin involvement and severe internal organ changes. The NK activity of healthy donors' PBMC was significantly decreased by addition of SSc patients PBMC (50:1) to the cytotoxicity assay, but was not influenced by the patients sera.

Cytotoxicity, Immunologic↗
PubMed1993

[Analyses of ventricular arrhythmias in patients with progressive systemic scleroderma (PSS) by ambulatory electrocardiography].

Disorder of cardiac conduction and automaticity have been widely reported in patients with progressive systemic scleroderma (PSS). In the present study, ventricular arrhythmias were monitored by 24-hour ambulatory electrocardiographic recording (Holter monitor) in 30 female patients with PSS and 20 apparently healthy female controls. The following results were obtained: 1) In the waking state, 8 cases of 30 patients showed grade III, IVA or IVB of ventricular arrhythmias, according to Lown's classification. On the other hand, ventricular arrhythmias, such as grade III, IVA or IVB, were not observed in the control group. 2) In the patient group, grade IVA arrhythmias were observed during defecation, driving or light working, and grade IVB were during defecation, urination, light working or sleeping. These results suggest that it is important to examine arrhythmias by Holter monitors in patients with PSS.

Adult↗