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Results for “Scleroderma, Systemic”

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PubMed1978

[Phoniatric and laryngological aspects of systemic scleroderma (author's transl)].

Organs which contain collagen can have connective tissue new growth in systemic scleroderma. The occurrence of scleroderma of the larynx and organs of speech is rare. The disease develops in the mucous membrane in three stages: oedema; infiltration and induration; and atrophy. The oedema stage is generally the shortest. Three patients with systemic scleroderma with involvement of larynx and organs of speech have been seen. In one is described (graphically, sonographically and with stroboscopy) the progress of the disease from an initial severe hoarseness to later resolution of the oedema. In another patient involvement of the soft palate produced rhinolalia. In spite of the limitation of tongue movement in 2 patients there was no disorder of articulation.

Aged↗
PubMed1986

[Physiopathologic bases of the treatment of systemic scleroderma].

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.

Blood Platelets↗
PubMed1976

Ultrastructure of the microvasculature in skin affected by systemic scleroderma.

The microvasculature of the upper dermis in cases of systemic scleroderma was examined with an electron microscope. Microvessels in clinically uninvolved skin areas showed nearly normal structure. On the other hand, in moderately involved skin, enlargement of the vascular lumen, endothelial swelling, increase in the pinocytotic vesicle and microvilli and reduplication of the basal lamina were observed. Along with these, Ruthenium red-positive granules, microfibrils and fine collagen fibrils were perivasculary increased. Markedly involved skin revealed a degenerative change in the endothelial cells with interendothelial gap; in the perivascular region the number of microfibrils and Ruthenium red-positive granules decreased, and were replaced by increased collagen fibrils.

Adult↗
PubMedDate not supplied

[Decreased blood fluidity in progressive systemic scleroderma].

The aim was to define blood rheology in progressive systemic scleroderma (PSS). 55 patients were compared to controls. Blood and plasma viscosity, hematocrit, red cell aggregation, and deformability were measured. Except for hematocrit, all these variables are significantly altered, indicating a loss of blood fluidity in PSS. Drugs had no obvious effect on blood rheology, but the clinical picture did. The loss of blood fluidity in PSS is suggested to play a pathophysiological role in the initiation of Raynaud phenomena, from which all patients suffered.

Adult↗
PubMed1990

[The mechanisms of intravascular blood coagulation in patients with systemic scleroderma].

Hemostasis was investigated in 2 groups of patients with systemic scleroderma (SSD) with minimal (12 patients) and moderate (9 patients) activity of the process. It has been shown that in SSD, the triggering factor of intravascular blood coagulation is the release of Willebrand's factor, an activator of platelets, from the impaired endothelium. Hyperaggregation and labilization of platelets characterizes the course of SSD irrespective of the disease activity. The main changes in coagulation hemostasis are related to the dramatically accelerated triggered thrombin formation and deficiency of the antithrombin potential. The status of fibrinolysis confirming the thrombogenic situation is marked by a number of features: depression of contact lysis is maximally pronounced in chronic SSD with minimal activity, accumulation of the soluble complexes of fibrin monomer only correlates with the disease activity, and no significant rise of the level of fibrin/fibrinogen degradation products has been discovered.

Adult↗
PubMed1989

[Nuclear medicine diagnosis of heart involvement in progressive systemic scleroderma].

The frequency and extent of heart involvement in progressive systemic scleroderma (PSS) can be underestimated when current clinical diagnostic methods are used. We therefore studied 53 PSS patients with planar (201Tl) chloride myocardial scintigraphy, and 44 PSS patients with radionuclide ventriculography using red blood cells labeled in vitro with 99mTc. Comparison of maximal exercise and resting myocardial scans demonstrated pathologic findings in 18 of 38 patients. In 10% of the patients abnormal ejection fraction values (less than 50%) and Fourier analysis were found on radionuclide ventriculography. Myocardial scintigraphy in conjunction with other diagnostic modalities thus provides useful information for the evaluation of heart involvement in PSS.

Cardiac Output↗
PubMedDate not supplied

[Systemic scleroderma and malignant diseases. A review of the literature].

Whether or not the association of systemic scleroderma and carcinoma is fortuitous is a much debated subject. Since 1886, approximately 320 cases of this association have been published. The most frequent malignancy involved is lung cancer (102 cases), followed by breast cancer (52 cases), malignant blood diseases (46 cases), cancer of the oesophagus (20 cases) and other gynaecological or gastrointestinal malignant tumours. A recent epidemiological study has shown that lung cancer is significantly more frequent than other malignancies, but statistical data on the latter are lacking. However, it would be wise to recommend that patients with systemic scleroderma should be regularly examined for gynaecological, haematological and gastrointestinal malignant diseases.

Humans↗
PubMed1996

[Systemic scleroderma and cancers: 21 cases and review of the literature].

Twenty one cases of the association systemic scleroderma and cancer are reported. Neoplasic localisations were the following: pulmonary five cases; breast two cases; esophageal cancer one case; stomach one case; colon one case; uterus four cases; ovarian cancer one case; prostatic cancer one case; renal cancer one case; malignant hemopathies six cases. In the literature, more than three hundred cases of such an association have been reported since 1886, essentially lung cancers (more than 100). Recent epidemiological studies allow to conclude to a higher frequency of lung and breast cancers. We suggest that systemic scleroderma patients should be examined attentively and carefully for these risks.

Adolescent↗
PubMed1998

Immunological characterization of heterochromatin protein p25beta autoantibodies and relationship with centromere autoantibodies and pulmonary fibrosis in systemic scleroderma.

Anticentromere antibodies (ACA) are immunological markers for the subset of systemic scleroderma with the symptoms calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia (CREST). In Western blotting, some ACA-positive sera also recognize a doublet of 23 kDa (p23) and 25 kDa (p25) in addition to centromere protein antigens A (17 kDa), B (80 kDa), and C (140 kDa). Two forms of p25 have been shown to be human homologues of Drosophila heterochromatin-associated protein HP1. One form of p25 (p25beta) which was recently cloned in this laboratory was used to evaluate anti-p25beta antibody response in scleroderma sera. Of 318 scleroderma sera 42 had ACA (13.2%), and 16 of the 42 sera (38%) had anti-p25beta antibodies. On the other hand, 5 of 276 ACA-negative sera (1.8%) showed anti-p25beta antibody response, demonstrating that anti-p25beta antibody is significantly associated with the ACA response (P < 10[-8]). Clinically the anti-p25beta response was significantly associated with the CREST syndrome. Fourteen (36.8%) of 38 CREST patients compared to seven (2.5%) of 280 patients with other forms of scleroderma were anti-p25beta antibody positive (P < 10[-8]). The 14 CREST patients with anti-p25beta antibodies had significantly more interstitial lung disease than those without anti-p25beta antibodies (P < 0.003). There was also a tendency to increased liver involvement. Two dominant autoepitopes in p25beta were determined by Western blotting using p25beta recombinant fragments. In immunofluorescence C-terminal specific antibodies showed staining of heterochromatin, but N-terminal specific antibodies showed no staining. Interestingly, the majority of sera reacted preferentially with one or the other of the two dominant autoepitopes.

Autoantibodies↗
PubMed1996

Differential regulation of transcription and transcript stability of pro-alpha 1(I) collagen and fibronectin in activated fibroblasts derived from patients with systemic scleroderma.

Activated fibroblasts were derived from the skin of patients with systemic scleroderma (SSc), used as a model for fibrosis. Such cells are characterized by increased production of collagens and other matrix constituents. Increased collagen and fibronectin production has been correlated with similarly elevated mRNA steady-state levels. In the present study we analysed the contribution of transcriptional activity and post-transcriptional transcript stability to the increases in pro-alpha 1(I) collagen and fibronectin mRNA steady-state levels in activated (scleroderma) fibroblasts. Fibroblasts, when cultured in close contact with a three-dimensional collagenous matrix, down-regulate collagen synthesis. Culture of skin fibroblasts from two patients with SSc in three-dimensional collagen lattices, however, showed 4-fold elevated pro-alpha 1(I) collagen mRNA levels over fibroblasts from healthy donors. Transcription of the COL1A1 gene in SSc fibroblasts was induced 2-3-fold over that in controls in both monolayer and lattice cultures, accounting in part for the elevated steady-state level. A 50% decrease in transcription rate in lattice compared with monolayer culture occurred, as in control cells. In contrast, whereas control cells in lattices responded with decreased (50%) pro-alpha 1(I) collagen mRNA stability, in SSc cells these transcripts were found to be more stable (half-life of 5 h compared with 2 h in control cells). Fibronectin steady-state mRNA levels, in contrast, were not significantly regulated by the three-dimensional environment. In SSc fibroblasts, fibronectin mRNA levels were induced 1.5-4.9-fold over controls. In part, this increase appears to be due to elevated transcription, and an increase in fibronectin transcript stability was also detected. We therefore conclude that activated fibroblasts such as those derived from scleroderma patients utilize transcriptional and posttranscriptional mechanisms to maintain increased collagen and fibronectin production, which contribute to the pathogenesis of the disease.

Base Sequence↗
PubMed1992

[Pulmonary clearance of 99mTc-DTPA aerosol in patients with progressive systemic scleroderma].

Alveolar epithelial permeability was assessed in 32 patients with progressive systemic scleroderma (PSS), using 99mTc-DTPA aerosol. Immediately after the inhalation of 99mTc-DTPA aerosol for 3 to 6 minutes under normal tidal breathing, lung was imaged sequentially for 30 minutes from the posterior by a gamma camera and exponential fitting was processed on the time activity curve. T1/2 (min) was used as a parameter for the evaluation of permeability of alveolar epithelium. Patients with collagen disease showed shorter T1/2 (T1/2 = 43.7 +/- 23.8 min) than the normal volunteers (T1/2 = 76.8 +/- 8.7 min). No significant difference was observed between patients with or without interstitial changes on the chest CT. Significant correlation was not observed between T1/2 and %VC or %DLco. In 8 cases, studies were repeated in the interval of 3 to 19 months. Improvement of T1/2 was seen in 4 cases, independent of CT findings. These results suggest that 99mTc-DTPA aerosol clearance study provides information independent from other lung examinations, and may be useful for the assessment of lung interstitial changes in patients with PSS.

Administration, Inhalation↗