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Systemic scleroderma is a general disease of the connective tissue which may affect practically any organ. The authors describe the case of a man where in the course of the disease affection of the heart with symptoms of severe cardiac failure became the dominating symptom.
The aim of the study was to determine the pulmonary 67-gallium uptake, bronchoalveolar lavage (BAL) cell differentiation and the activity of BAL cells, measured as release of superoxide anion (O2-), and to investigate the results whether there are relations. In 11 nonsmoking systemic scleroderma (SS) patients and 11 systemic lupus erythematosus (SLE) patients with lung involvement double-sided BAL were performed, mainly in regions with increased 67-gallium uptake. Release of O2- was measured by INT-assey. BAL cell differentiation was in SS and SLE pathological in 68.2% without side-difference. In contrast to SS, O2(-)-release in SLE depends on BAL cell differentiation and is most increased in normal BAL cell differentiation. There is no correlation between 67-gallium uptake and both BAL cell differentiation and O2(-)-release. The results suggest, that in contrast to SS, BAL cells in SLE with pathological differentiation are less activated than BAL cells with normal differentiation probable due to autoimmunological factors. Pulmonary 67-gallium scan and BAL seem to be independent from each other.
The aim of the study was to determine the bronchoalveolar lavage (BAL) cell differentiation and the activity of beta-glucuronidase and N-acetyl-beta-D-glucosaminidase in alveolar macrophages. In 12 patients with systemic sclerosis (SS), 4 with systemic lupus erythematosus and 4 healthy controls BAL was performed. The activity of beta-glucuronidase and N-acetyl-beta-D-glucosaminidase was measured semiquantitative by means of cytochemical methods. Lymphocytes and neutrophils in BAL cell differentiation are increased, also the activity of beta-glucuronidase. The activity of N-acetyl-beta-D-glucosaminidase is decreased in SS and SLE in comparison with controls. The activity of beta-glucuronidase seems to be a marker of activity of alveolar macrophages in SS and SLE.
Patients with systemic lupus erythematosus (SLE), systemic scleroderma (SSD) and donors were examined for the blood levels of adrenocorticotropic hormone, hydrocortisone, follicle-stimulating hormone, luteinizing hormone, prolactin, estradiol, testosterone, progesterone, thyroid-stimulating hormone, triiodothyronine, thyroxin, and insulin. The corticotropin load test was carried out in 38 SLE patients, 32 SSD patients and 24 donors. The prednisolone test was made in 15 SSD patients and 27 donors. The studies were made with the aid of RIA. The patients with SLE manifested a decline of the basal level of hydrocortisone as well as a reduction of the reserve potentialities of the pituitary-adrenal system. The patients with SSD demonstrated a negligible decrease of the basal level of hydrocortisone with an evident lowering of the reserves of the same system. The treatment of SLE and SSD patients with glucocorticoids was followed by marked hyperinsulinemia.
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A case of systemic scleroderma with peculiar morphaea-like lesions is reported. The patient at first started with typical acrosclerosis, some parts of which later became softened and apparently resumed the appearance of normal skin, so that morphaea-like cutaneous plaques which remained hard, were soft. These changes were surrounded by erythema resembling the 'lilac ring' as noted in characteristic active lesions of morphaea.
Atrophy of orbital fat is a reported complication of localized scleroderma but not of systemic scleroderma. Here we present a case of systemic scleroderma with unilateral enophthalmos. Orbital fat atrophy was the presumed cause on computerized tomography.
As a result of intraperitoneal injections in mice of crude glycosaminoglycans obtained from the urine of patients with systemic scleroderma, a fibrotic process similar to the intial changes of human scleroderma was frequently observed, especially in the skin and esophagus when the glycosaminoglycans contained a probable variant of heparan sulfate. Furthermore, the scleroderma-like change was also observed, to some extent, in the ultrastructure of collagen fibers and glycosaminoglycans of the skin.
Two cases of systemic scleroderma in girls are reported. One patient, aged 11 years, has systemic scleroderma with Raynaud's phenomenon, and pulmonary involvement. The other, aged 8 years, has systemic scleroderma with lung involvement. The specific features of pediatric systemic scleroderma are reviewed briefly.
49 unrelated subjects suffering from systemic scleroderma were typed for 28 HL-A antigens, without any particular significant association of an antigen with the disease or one of its manifestations being noted. In addition, 13 patients from the same family were genotyped for HL-A. Transmission of the disease through 4 generations does not seem to be linked to a particular haplotype and no pair of sibling HL-A identical patients were seen in the same generation. By contrast, two pairs of sibling patients were HL-A different. Nevertheless, other cases, and in particular familial, will be necessary before an association between the genes of susceptibility to S.S. and a gene in the chromosomal HL-A region may be definitely eliminated.
In 24 patients with progressive systemic scleroderma the respiratory disturbances, X-ray, scintigraphic and clinical deviations as well as the statistical correlations between them are described. The functional examination of respiration revealed lowered diffusion capacity, restrictive type of ventilatory failure and hypoxemia as the most frequent disturbances. The functional examination of respiration is correlated with the X-ray changes in the lungs in these patients.
A glycosaminoglycan with scleroderma-inducing effect was isolated and partially purified from the urine of patients with systemic scleroderma. The glycosaminoglycan was an N-sulfated glycosaminoglycuronan and its high total sulfate and 2,5-anhydromannose contents suggest that the glycosaminoglycan is a degradation product of heparin or polysulfated heparin sulfate. Furthermore, the composition of the above glycosaminoglycan was similar to that of the N-sulfated glycosaminoglycan which we observed previously in uninvolved skin of scleroderma.
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.
Antibodies against nuclear ribonucleoprotein (RNP) were found in 5 of 63 cases of systemic scleroderma, whereas they were present in all but one case of mixed connective tissue disease and in 15 of 67 cases of systemic lupus erythematosus. In all RNP positive cases of systemic scleroderma there were some features of other collagen diseases, and their course was relatively more benign. Studies of RNP antibodies in systemic scleroderma may be of importance for treatment and prognosis.
Liver electron microscopic studies were performed in 14 patients with systemic scleroderma. In 13 of these patients, giant mitochondria were demonstrated in the hepatocytes. This ultrastructal abnormality was present whatever the type and duration of the disease and was also present even when the liver was histologically normal. The mechanism of formation of giant mitochondria in systemic scleroderma is unknown.
This study was performed on seven children with systemic scleroderma, three with the diffuse and four with the limited type. All three patients with diffuse scleroderma had high titers of clumpy pattern antinucleolar antibody on HEp-2 cells. The course of the disease was severe, and two children died. Four children with limited scleroderma had mild disease, and Scl-70 antibody, an immunologic marker that in adults is associated mostly with diffuse scleroderma. In one child Scl-70 antibody and anticentromere antibody coexisted, although previously the two were believed to be mutually exclusive. This study shows that limited scleroderma of childhood with slight cutaneous involvement may be associated with the Scl-70 marker. The findings in 10 adults in whom Raynaud's phenomenon developed in childhood and indurations appeared some years later, point to the significance of careful observation of these children, with repeated testing for immunologic markers of SSc. An important new finding is the association of different types of systemic sclerodermas with specific immunologic markers.
From the urine of patients with systemic scleroderma, we previously isolated a glycosaminoglycan, which could induce a scleroderma-like change in the skin of mouse. In the present work, the cell-mediated response to urinary glycosaminoglycans was examined by lymphocyte transformation test. The specific response was observed in lymphocytes of patients with scleroderma, when the above scleroderma-inducing glycosaminoglycan was added. By contrast, lymphocytes of patients with SLE or dermatomyositis and of normal persons hardly showed a response to this glycosaminoglycan. On the other hand, the glycosaminoglycans from normal urine could not stimulate lymphocytes of patients with scleroderma or healthy persons.
Three patients seen with similar findings of progressive systemic scleroderma. Two of the patients, a father and son, had very similar skin changes, sclerodactyly, Raynaud phenomenon, gastrointestinal involvement, and pulmonary symptoms. The three patients were from the highly inbred Brandywine triracial isolate. This isolate is a group of families who have been inbreeding since 1660 and now have the highest gene frequencies for sickle cell anemia and oculocutaneous albinism in the United States. There have been only a few reported cases of familial scleroderma and the hereditary aspect of the disease has not been well established. This report shows that the mortality for scleroderma in this isolate is at least 250 times the mortality of the general population, thus suggesting a probable genetic predisposition for the disease.