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Results for “Scleroderma, Systemic”

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PubMed1995

[Cardiac manifestations of diffuse systemic scleroderma. Apropos of 4 cases].

Based on a series of four cases and a review of the literature, the authors describe the lesions of the various cardiac tissues in the course of systemic scleroderma. Pericardial involvement presents in the form of either acute pericarditis or chronic pericarditis. Pericardial tamponade is exceptional. Sclerodermal cardiomyopathy is frequent and serious and can be responsible for heart failure. Arrhythmias are frequent and may be either ventricular or supraventricular. Involvement of the conduction tissue often requires implantation of a pacemaker. Endocardial and valvular involvement is very rare. Lastly, coronary involvement appears to be fairly rare and responsible for vasospastic episodes in the coronary artery territory.

Adult↗

PubMed1976

Skin capillary abnormalities as indicators of organ involvement in scleroderma (systemic sclerosis), Raynaud's syndrome and dermatomyositis.

Forty-four study patients with scleroderma (systemic sclerosis) (28 patients), Raynaud's syndrome (13 patients) or dermatomyositis (three patients) were observed for skin capillary abnormalities by widefield microscopy and compared with three control groups of 20 subjects each: (1) patients with other rheumatic disease, (2) hospitalized patients with nonrheumatic conditions, and (3) healthy volunteers. The distinctive microvascular pattern (dilated and distorted capillary loops alternating with avascular areas) previously reported in scleroderma and dermatomyositis was observed almost exclusively in the study patients. The severity of capillary abnormalities varied among the diagnostic subgroups, and a positive correlation was found between the degree and extent of abnormal microvascular patterns and multisystem involvement. On this basis, widefield nailfold capillary observations are proposed as a simple, inexpensive, reproducible technic for making an improved early diagnosis and predicting multisystem involvement in scleroderma, Raynaud's syndrome and dermatomyositis, presently a group of loosely associated and overlapping connective tissue disorders which often defy early and precise diagnosis.

Adult↗

PubMedDate not supplied

[Digestive manifestations during systemic scleroderma].

A 3 years' study was performed on a group of 32 patients with systemic scleroderma. 23 of them had digestive manifestations involving the whole digestive tract. The diagnosis was based on the clinical, radiological, echographic, endoscopic and anatomopathological examinations. In the prolonged forms of the disease, successive associations appeared in various segments of the digestive tract, the esophageal one being always the first. In all cases, the digestive manifestations followed the cutaneous involvement and were constantly correlated to Raynaud's syndrome presence. Digestive manifestations did not influence the prognosis of the substrate disease.

Digestive System Diseases↗

PubMed2006

[Autonomic nerve function in patients with systemic scleroderma using heart rate variability analysis].

We examined autonomic nerve function in patients with systemic scleroderma (SSc) using power spectral analysis of heart rate variability. In the SSc group, both in nighttime (0:00a.m.-5:00a.m.) and daytime (10:00a.m.-5:00p.m.), HF elements, a parasympathetic nerve index, were lower and LF/HF ratios, a sympathetic nerve index, were higher than in the control group. This suggests that in SSc patients, sympathetic nerve activity was increased and parasympathetic nerve activity was decreased throughout the day.

Adult↗

PubMed1989

[The regulation of DNA synthesis by fibronectin and its proteolytic products in the skin fibroblasts of healthy donors and patients with systemic scleroderma and rheumatoid arthritis].

To study the effect of fibronectin isolated from plasma and culture media and the effect of its tryptic hydrolyzates on DNA synthesis, cultured skin fibroblasts of healthy donors and these of patients with systemic scleroderma (SSD) and rheumatoid arthritis (RA) were employed. It was shown that both fibronectin and total products of its proteolysis markedly stimulated DNA synthesis only in skin fibroblasts of patients with SSD. Fibronectin fragments inhibited DNA synthesis in all fibroblast strains studied. The effect of fibronectin and all its Gel fragments on the DNA synthesis in skin fibroblasts of patients with SSD was dose-dependent. The activity of total fibronectin tryptate, Gel-fragment-free tryptate, and Gel fragments themselves depended on the duration of fibronectin proteolysis, i. e. on the size of the fragments obtained. Culture media collected after treatment of fibroblast monolayer with trypsin and subsequent removal of fibronectin Gel fragments had mitogenic effect on skin fibroblasts, especially on those of patients with SSD and RA. It is supposed that fibronectin Gel fragments are inhibitors of growth factors produced by fibroblasts. The results suggest that fibronectin and its fragments have an important regulatory role in fibroblast proliferation.

Arthritis, Rheumatoid↗

PubMed1992

[Systemic scleroderma and scleroderma-like disease after silicone implants].

Systemic sclerosis or clinical patterns indistinguishable from systemic sclerosis have previously been demonstrated in workers in the polyvinylchloride industry and in mine-workers exposed to silica dust. In recent years, an increasing number of cases of systemic sclerosis, localized scleroderma, and scleroderma-like disease have been diagnosed in women after implantation of silicone gel prosthesis either following operations for breast cancer or cosmetic augmentation mammoplasty. We present two cases of systemic sclerosis or scleroderma-like disease appearing after silicone implants. Together with the already reported cases these results should lead to reduced use of silicone for cosmetic augmentation mammoplasty and a search for another material for patients operated on for breast cancer. The cases described, together with the reports concerning industrially provoked scleroderma, are of interest for the continued efforts to clarify the pathogenesis of scleroderma. Other types of exposure than those described here may also be of interest, i.e. occupational exposure to silicone spray.

Female↗

PubMed1990

[New markers of collagen and basal membrane metabolism and kidney involvement in systemic scleroderma].

The study was made within the framework of the Soviet-Finnish cooperation and represents a fragment of work pertaining to the clinical trials of new markers of metabolism of collagen and basal membranes in patients afflicted with systemic scleroderma (SSD). Sufficient clinical material (84 SSD patients) and radioimmunoassays were employed to study the clinical significance of aminoterminal propeptide of type III procollagen. In SSD patients with renal impairment (n = 34) and without it, PIIINP and PIIINP-Fab, serum galactosyl hydroxylysylglucosyltransferase and two antigens of basal membranes-7S-domene of type IV collagen and PI-fragment of laminine were determined. Renal impairment was established to correlate with serum concentration of PIIINP-Fab (p less than 0.007). PI-fragment of laminine. The parameters under study correlated as well with the disease course, the presence of arthritis and the Raynaud's syndrome gravity and can be regarded as markers of the disease activity and high fibrous formation lying at the basis of SSD.

Adolescent↗

PubMedDate not supplied

[Variability of the clinical picture and the classification of progressive systemic scleroderma].

Fiftythree patients with progressive systemic sclerosis were studied. Four of them (3 males) had the diffuse form of the disease. The skin manifestation of this clinical picture is characterized by diffuse progression of the cutaneous sclerosis over almost the whole body surface, except for the hands where it eventually may appear late. The prognosis for these patients it especially poor. Fortyfive patients (44 females) had acrosclerosis in the widest sence. Twentyseven of these ("acrosclerosis stricto sensu") had cutaneous sclerosis of the hands, face, and often other parts of the body, but not on the abdomen, arms or thighs. The remaining 18 patients had sclerotic alterations on these surfaces also. In this syndrome (which the authors call "the intermediary syndrome"), i.e. where the abdomen, arms, and thighs also are affected, certain internal organs and the joints are more involved than in "acrosclerosis stricto sensu". With rare exceptions, a symptomatic tetrade (REST-syndrome) occurred in acrosclerosis and all the intermediary syndromes. This consisted of Raynaud's syndrome (R), esophagopathy (E), cutaneous sclerosis (S), and telangectasia (T). Fifty % of the patients in addition had calcinosis (C), either subcutaneous or para-articular. The tetrade "REST syndrome" becomes in these cases the pentade "CREST syndrome". The addition of calcinosis to the other four phenomena of the REST syndrome does not alter the frequency of internal organ involvement or the prognosis of the disease. The term "REST syndrome" and its variant "CREST syndrome" should replace the conservative term "acrosclerosis" because they add to the purely cutaneous phenomena other characteristic manifestations of the disease. Two patients could neither be classified under the REST syndrome nor the progressive diffuse syndromes. Two other patients had no cutaneous phenomena ("scleroderma sine scleroderma").

Adolescent↗

PubMed1978

Migraine and systemic scleroderma.

Scleroderma with typical migraine headaches occurred in 16 well-documented cases observed over a 25-year period. Although the number of cases is within the expected range of coincidence of both diseases, in 13 of the 16 patients the scleroderma developed after 15 years or more of therapy with ergot or methysergide preparations. Eleven of the 13 patients had Raynaud's vasospastic phenomenon as part of their systemic scleroderma. The vascular pathology of scleroderma, Raynaud's phenomenon, and ergotism is similar enough to suggest caution in the administration of these drugs to patients with migraine and extra caution in observing for signs of Raynaud's phenomenon or early vascular scleroderma.

Adult↗

PubMed1994

Mucin deposits in morphea and systemic scleroderma.

BACKGROUND: Though rarely reported, mucin deposition may be observed in scleroderma. OBJECTIVE: To verify the frequency of significant amounts of mucin in the biopsy specimens. METHODS: Biopsies from 20 patients with scleroderma were reviewed and stained to verify the presence of mucin. RESULTS: Mucin deposits were found in all of the 20 specimens. CONCLUSION: Mucin deposition is probably a constant feature in both morphea and systemic scleroderma. Its relevance in differential diagnosis between scleredema and scleroderma is debatable.

Adolescent↗

PubMed2006

[A case of systemic scleroderma complicating pulmonary hypertension].

The patient was a 7-year-old girl. At the age of 6, deposits of pigment had appeared on the skin of her face and limbs, the skin had become sclerosed, and she had developed dyspnea on exertion. Her previous physician had hospitalized her. She was diagnosed as systemic scleroderma that accompanied pulmonary hypertension by her symptoms and laboratory findings. She was referred to our hospital at 7 years of age, and she was hospitalized. At that time, the entire skin showed deposition of brown pigment, the skin of the limbs was sclerotic. And the face was mask-like, flexion of the joints of the fingers and knees was limited, and the fingertips were ulcerated. Raynaud's phenomenon was present. She was positive for antinuclear antibodies, and negative for other autoantibodies. Echocardiography revealed pulmonary hypertension. After admission, steroid pulse therapy and cyclophosphamide (CY) pulse therapy were initiated, and for aftercare, 15 mg/day of prednisolone (PSL) and mizolibin (MZB) were administered orally. After several months, the sclerosis of the skin improved and the restriction of limb flexion was almost eliminated. The pulmonary hypertension advanced temporarily (maximum: 70 mmHg), but after oral administration of a PGI2 preparation and low-flow supplemental oxygen therapy and the initiation of anticoagulant therapy, the systolic pressure of the pulmonary artery improved to 34 mmHg. The CY pulse therapy was terminated after two years, and internal use of PSL and MZB was continued. The patient's condition is now stable. This case was treated from an early stage with steroid pulse therapy and CY pulse therapy, accompanied with oral administration of a PGI2 preparation for the pulmonary hypertension. The dermal symptoms improved, and it was possible to maintain a state of remission.

Child↗

PubMed1989

[Respiratory-hemodynamic features in patients with systemic scleroderma].

The authors provide the results of the clinical and functional studies in 25 patients with systemic scleroderma. The patients were examined for the diffusion lung capacity separated into the constituent components (membrane and blood ones), for the uniformity of alveolar ventilation, for pulmonary artery pressure and pulmonary blood flow. They also underwent echocardiography. The use of the combination of non-invasive research methods makes it possible to obtain quantitative and quantitative information pertaining to the involvement of the lungs at the level of the alveolocapillary membranes and microvascular bed, myocardium and to their interrelations at the early stages of systematic scleroderma. In patients with the pronounced clinical picture of the disease, of paramount importance is the use of these methods for proper interpretation of certain symptoms in the estimation of lung and heart injuries and of their prevailing significance in the general clinical picture of the disease. The new trends in the present work are related to the demonstration of the compensatory and pathological changes in the respiratory and hemodynamic system in sclerodermia systematica at the early enough stages of the illness.

Adult↗

PubMedDate not supplied

Echographic diagnosis of cardiac involvement in systemic lupus erythematosus and systemic scleroderma.

The great frequency and severity of cardiac involvement in collagen diseases have proved to be an important diagnostic, prognostic and therapeutic problem. In the last few years the value of echographic examination in the estimation of the pathologic cardiac changes in the course of systemic lupus erythematosus (SLE) and of systemic scleroderma (SS) has considerably increased making the object of numerous studies. In this paper the results obtained in some of these studies are estimated comparatively.

Cardiomyopathies↗

PubMed1986

In-vivo capillary-microscopical findings in patients with thrombangiitis obliterans, progressive systemic scleroderma, and rheumatoid arthritis, respectively.

Fourty patients with thrombangiitis obliterans were examined to find out whether in-vivo capillary microscopy can contribute to the establishment of a diagnosis. No capillary-microscopical signs could be detected that are exclusive to thrombangiitis obliterans. Nevertheless typical signs as capillary lengthening, capillary branching (53%) and haemorrhagic margin (75%) are of considerable differential-diagnostic value when it comes to distinguishing a case of thrombangiitis obliterans from a case of degenerative arterial occlusive disease. In a further series of examinations, the nailfold area of patients with progressive systemic scleroderma was examined by in-vivo capillary microscopy and with an ophthalmoscope. The coincidence of the findings between the two methods was 80% and more, so that the examination of the nailfold capillaries with an ophthalmoscope can aid verify the diagnosis of scleroderma. In patients with rheumatoid arthritis capillary tortuosities (85%), capillary branching (53%) and increased venule visibility (55%) often occur. These are unspecific signs. Typically capillary-microscopical symptoms for the disease are not detectable.

Adult↗

PubMed1975

[Combined lung cancer and systemic scleroderma].

The article presents data of the literature and the author's personal two observations on combination of systemic scleroderma and cancer of the lungs. According to the data obtained, the most frequent forms of neoplasms in this combination were tumours of an adenomatous structure. A decisive role in the machanism of development of adenomatosis of the lungs was played by septo-alveolar sclerosis which led to rearrangement of the lung tissue. Potential malignancy of adenomatosis of the lungs in systemic sclerodermia was closely connected with participation of a "defective" collagen of the embryonic type in the formation of the basal membrane of the alveolar epithelium, as well as with the predominance among the tumour cells of granular pneumocytes of the B type characteristic of the embryonal period of mammals.

Adenocarcinoma↗

PubMed1990

[The fibrillarin (Scl-34) autoantibody in systemic scleroderma].

Reexamination of ANA positive sera with nucleolare fluorescence staining on HEp-2 cells and hamster liver imprints revealed 3 sera with an uniform characteristic pattern. It is characterized by: 1. Strong clumpy fluorescence of nucleoli in the interphase. 2. Missing nucleoplasma staining (pure nucleolar). 3. Characteristic granular staining of the cytoplasma from the condensed chromosoma in mitosis. 4. Additive fluorescence of a nucleolus-like body. 5. Extremely high antibody titers. 6. Negative immunodiffusion. This nucleolar staining pattern was described by Bernstein et al. as a clumpy nucleolar pattern. It is produced through an antibody to the dense fibrillar component of the nucleolus (fibrillarin). This antigen is a protein of the U3 RNP with a molecular weight 34 kDa. Clinically is the fibrillarin antibody highly specific for systemic scleroderma especially with diffuse skin involvement. We found it in 1% of scleroderma patients. Its pathogenetic importance is not known.

Antibodies, Antinuclear↗

PubMed1988

Theophylline-resistant and theophylline-sensitive "active" and "total" E rosette-forming lymphocytes in patients with systemic scleroderma.

Using the E rosette test and its modification with theophylline, we have studied T regulatory lymphocytes in various forms of systemic scleroderma. Mean percentages of active rosette-forming cells (ARFC) as well as the fraction resistant to theophylline incubation (ARFC-res) were significantly decreased, irrespective of the variety of the disease, compared to the age-matched controls. Late ("cold") rosette-forming fractions were unimpaired. The theophylline-sensitive fraction of total rosette-forming cells (TRFC-sens), which contains mainly cells from the suppressor circuit, was found to be lowered in all patients groups studied, whereas the ARFC-sens fraction was significantly decreased only in patients with diffuse scleroderma over 50 years of age, in whom there was a tendency to a more severe course, as manifested by pronounced systemic organ involvement. The lowered values of E rosette tests were found in a majority of SSc patients and were correlated with the appearance in the sera of factors capable of inhibiting ARFC formation by normal human peripheral blood T lymphocytes. Normal values of the E rosette test were related to the presence in the patients' sera of factors stimulating ARFC formation by normal lymphocytes. We surmise from the results that in SSc patients the T-cell defect is not only restricted to T suppressor cells but also refers to the active theophylline-resistant fraction containing mainly T inducer and T cytotoxic cells.

Adult↗

PubMed1999

Expression of TGF-beta 1, -beta 2 and -beta 3 in localized and systemic scleroderma.

Scleroderma is a generalized or localized disorder which leads to fibrosis of the affected organs. TGF-beta has been implicated as a causal agent in its pathogenesis. In mammals, TGF-beta comprises a family of three members, beta 1, beta 2 and beta 3. Since cutaneous wound healing is thought to result either in formation of a scar or in scar-free tissue regeneration, depending on the relative amounts of the beta 3 isoform, the expression of all three isoforms was studied in skin biopsies of patients with either localized or systemic scleroderma. mRNA for all three isoforms was detected in inflammatory skin areas of both disease forms, but never in sclerotic or healthy skin. Immunohistochemical analysis confirmed expression of beta1 and beta 2 proteins in inflammatory skin of patients, whereas beta 3 protein appeared to be present in the subepidermal area and also found throughout the dermis of patients and healthy dermis as well.

Adult↗