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[Study of bone density in systemic scleroderma].

BACKGROUND: Osteopenia in systemic sclerosis (scleroderma) patients was reported in X-ray studies of hands and by proximal and distal forearm bone mass measurement. It has been suggested that bone loss in these patients might be due to chronic ischemia, immobilization and early menopause. Nevertheless, it is not established if these patients present generalized osteopenia. To shed light into this point we studied bone mineral density in the spine, proximal femur and total body in patients with systemic sclerosis. PATIENTS AND METHOD: Twenty-five Caucasian women were evaluated. Mean age of patients was 48 +/- 12 years and mean time of disease was 7 +/- 7 years; 13 were postmenopausal (8 +/- 8 years). Bone mass was measured in the spine, proximal femur and total body by using a dual-photon absorptiometry with X rays source (Lunar-Model DPX). RESULTS: Bone mass in different sites was not statistically different from the age-matched control healthy women. Mean bone mass of patients with limited form was not different from patients with diffuse form of systemic sclerosis. Patients with calcinosis had lower bone mass at proximal femur than those without this alteration. CONCLUSIONS: Patients with systemic sclerosis do not present bone loss and this disease in not a risk factor for generalized osteoporosis.

Adult↗
PubMed2003

Endothelium-dependent regulation of cutaneous microcirculation in patients with systemic scleroderma.

The microinjection method used for the first time in this study makes it possible to measure in vivo the endothelium-dependent vasomotion in the nailfold capillaries of systemic scleroderma patients. Aceylcholine (blood concentration of 10(-5) M) and sodium nitroprusside (blood concentration of 1.6 microg per kg per min) were used as test substances to examine capillary vasomotion. Defined quantities of medication in the range of microliters were administered as a bolus using the Panomat V-3 medicine pump. The boluses were injected into the capillaries at the capillary loop in 10 scleroderma patients and 10 randomized healthy volunteers. Blood flow velocity and the diameter of the skin capillaries at the venous loop were measured and tested for correlation with the effects of injections of test substances. The intracapillary administration of sodium nitroprusside brought about a significant decrease in capillary blood flow velocity and a significant increase in vascular diameter in comparison with baseline values, both in systemic scleroderma patients (p<0.005 and p<0.0001) and in the healthy volunteers (p<0.001 and p<0.0001). The intracapillary administration of acetylcholine led to a significant decrease in blood flow velocity (p<0.001) and increase in vascular diameter (p<0.0001) only in the healthy volunteers. In systemic sclerosis patients capillary diameter and blood flow velocity were unaffected. In two systemic scleroderma patients this procedure was repeated in identical fashion at the end of a 1 wk infusion therapy cycle with prostacyclin. The infusion therapy led to a normalization of endothelium-dependent vasomotion in the two scleroderma patients. The microinjection technique used in this study for the first time made it possible to establish in vivo that endothelium-dependent vasomotion is disturbed in systemic scleroderma patients.

Acetylcholine↗
PubMed2002

A variant of acrokeratoelastoidosis in systemic scleroderma: report of 7 cases.

We describe acrokeratoelastoidosis-like lesions on the palms of the patients with systemic scleroderma. Histology showed a focal hyperkeratosis with or without epidermal concavity, regular acanthosis, and hyalinization of collagen fibers and, in some cases, fragmentation and diminution of elastic fibers in the deep dermis. A slight degree of fibrotic change of collagen in the uninvolved neighboring skin was found in one case. The lesions were found in 7 of 26 patients with systemic scleroderma who were analyzed here, and were not found in the unrelated connective tissue disorders (n = 32) and normal controls (n = 27). The cause of the unique skin lesions may be related to the altered connective tissue metabolism similar to that of systemic scleroderma.

Adult↗
PubMed1997

Is there circadian variation of plasma endothelin (ET-1) in patients with systemic scleroderma (SSc)?

Forty-three patients with systemic scleroderma (SSc), 10 with non-SSc (6 cases of systemic lupus erythematosus and 4 cases of dermatomyositis), 14 cases of mild- or non-sclerotic type of scleroderma with capillaroscopic abnormalities of nailfolds (SSD; scleroderma spectrum disorders) and 10 healthy volunteers (HC) were subjected to examination of plasma levels of endothelin-1 (ET-1). The sex ratios (male/female) in the patients with SSc, non-SSc and HC were 7:36, 4:6 and 0:10, and the ranges of their ages were 22-74, 19-78 and 33-62 years old, respectively. The plasma levels of ET-1 in SSD, SSc (Barnett I;15), SSc (Barnett II;16), SSc (Barnett III;12 cases), non-SSc and HC were 1.67 +/- 0.37 2.04 +/- 0.58 2.04 +/- 0.68 1.85 +/- 041 191 +/- 0.7 and 1.31 +/- 0.34 pg/ml, respectively, confirming previous results from other laboratories. The plasma levels of ET-1 statistically differ between each collagen disease (SSD, SSc and non-SSc) and HC using Student's t-test (P < 0.05). Although a statistically significant difference was obtained in the plasma levels of ET-1 between the SSc group (6 cases) and HC (6 cases) measured at 06:00, 12:00, 18:00 and 24:00 h, there was no significant circadian variation of plasma levels of ET-1 at these times in both the SSc group and HC. The present study revealed that (1) the ET-1 level in HC showed no circadian fluctuation, and remained at a low level (0.8-1.6 pg/ml). (2) When compared to HC, ET-1 in blood plasma of patients with SSc was elevated (0.3-3 pg/ml) throughout the day and night (P < 0.05). (3) ET-1 tended to increase more at midnight (24:00 h) in the SSc group without PSL treatment, though no statistical significance was obtained. (4) TAT showed a significant increase at noon (12:00 h) suggesting coagulation activity in patients with SSc, but PlC did not show a significant increase compared to HC. In conclusion, the observed increase of vasoconstrictive ET-1 in the patients with SSc throughout the day and night may make maintenance of peripheral blood flow more difficult, may have some biological origin and should be further investigated.

Adult↗
PubMed1979

Lymphocyte responsiveness to urinary glycosaminoglycan in systemic scleroderma.

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.

Antigens↗
PubMed1990

Cytotoxic effects of sera from patients with systemic scleroderma: comparison of three different in vitro methods.

Sera from 93 patients with systemic scleroderma including incipient or prodromal acroscleroderma and from 43 healthy individuals were studied for cytotoxic effects on endothelial cells by means of three different in vitro methods. Inhibition of 3H-thymidine incorporation by endothelium was caused by about 33% of sera, almost exclusively from patients with diffuse scleroderma and the transitional form: acroscleroderma--diffuse scleroderma. An antibody-dependent cellular cytotoxicity assay revealed cytotoxicity of about 37% of sera from patients with diffuse scleroderma and the transitional form but also of a proportion of sera from patients with CREST syndrome with pronounced vascular changes. Serum cytotoxic activity, revealed by both methods, was related with more frequent involvement of muscle and kidney in the patients. A direct 51Cr release assay showed the cytotoxicity only in 4 of 68 cases in diffuse scleroderma with pronounced internal organ involvement. Thus, depending on the method used, various types of cytotoxicity could be detected in sera from patients with systemic scleroderma.

Adult↗
PubMed1995

Histopathological and capillaroscopical features of the cuticles and bleeding clots in ring or middle fingers of systemic scleroderma patients.

Sixty-three patients with systemic scleroderma (SSc) (Barnett I, 41; Barnett II, 17; Barnett III, 5), 14 with systemic lupus erythematosus (SLE), 9 with dermatomyositis (DM) and 10 healthy controls (HC) were subjected to histopathological examinations of the cuticles of ring or middle fingers. The sex ratios (male/female) in the patients with SSc, SLE, DM and HC were 7:56, 5:9, 5:4 and 5:5, and the ages were 22-74, 19-78, 45-70 and 13-78 years old, respectively. Biopsy samples were taken from the central portion of the cuticles, which showed the most severe change of elongation with or without bleeding clots of cuticle-proximal nailfolds (BC). Histopathologically, 61 (96.8%) cuticles of SSc patients consisted of the upper (U), middle (M) and lower (L) layers, which represent obliquely stacked, parabolic, and parallel stacked layers, respectively. The middle parabolic layer appeared to discharge homogenous eosinophilic globular deposits (ED). On the other hand, this typical three-layer-nail pattern was seen only in 9 (64.3%) of SLE, 3 (33.3%) of DM and none of HC, in total 12 (36.4%) of the non-SSc group, which included SLE, DM and HC. In SSc, there were statistical correlations (R2) between ED and BC, ED and cuticle-elongation, cuticle-layer and cuticle-elongation, ED and cuticle-layer, BC and cuticle-elongation. Capillaroscopically, bleeding clots located in the middle layer with ED of the cuticles in eight patients with SSc were transported rapidly within 1-2 weeks.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1975

Familial progressive systemic scleroderma.

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.

Adult↗
PubMed1991

Current options for the treatment of systemic scleroderma.

The epidemiology, pathology, diagnosis, and clinical manifestations of systemic scleroderma (SSc) are described, and therapeutic options are discussed. SSc is a rare condition of unknown etiology that occurs in a subset of scleroderma patients. It is distinguished by involvement of the small arteries, microvessels, and diffuse connective tissue. The degree of internal organ involvement is the main determinant of morbidity and mortality. Management of SSc may entail supportive, palliative, remittive, and immunosuppressive therapies. Supportive therapy involves maintaining the affected extremities at warm temperatures and the use of emollient creams. Results of palliative treatment are mixed. Toxic reactions may be associated with many of these medications. Dermatologic manifestations have been treated with nonsteroidal anti-inflammatory agents, low-dose corticosteroids, dimethyl sulfoxide, and edetate disodium; peripheral and internal-organ vascular obstruction, with alpha-adrenergic blockers, angiotensin-converting-enzyme inhibitors, and calcium-channel blockers. Antacids with alginic acid, histamine H2-receptor antagonists, sucralfate, and cholinergic-acting agents may be used to relieve GI symptoms. Lung infections should be treated promptly with antibiotics. No drug therapy has been successful in reducing the incidence of fatal cardiac arrhythmias or in preventing cardiac fibrosis. Captopril and enalapril are essential in the control of SSc renal crisis. Penicillamine may hold promise as a remittive therapy. The immunosuppressive agents fluorouracil, cyclosporine, and methotrexate, which have shown limited effectiveness in preliminary studies, merit further investigation. No therapeutic agent has yet been shown to alter the course of SSc on a consistent or long-term basis. Toxicity and drug interactions remain a major concern in patient management, and aggressive monitoring is essential.

Adult↗
PubMed1985

[Clinico-morphological characteristics and working classification of kidney diseases in patients with systemic scleroderma].

Based on a study of 110 patients with systemic scleroderma (SSD) (74 had involved and 36 uninvolved kidneys) the authors provide the clinico-functional and morphological characteristics of renal lesions and the working classification of sclerodermic nephropathy (SN). The two basic variants of SN were recognized: acute and chronic. The latter one was subdivided into clinical, moderate and pronounced SN. A correlation was revealed between the functional (glomerular filtration lowering) and ultrastructural (reticulation of endotheliocytes and thickening of the basal membrane of the glomerular capillaries) signs evidencing derangement of the microcirculatory bed. Morphological examination of the kidneys disclosed alterations that mirror the complex pathogenesis of SSD: derangement of the microcirculatory bed, immunopathological disorders, activation of connective tissue components.

Adolescent↗
PubMed1982

[Clinical value of bronchoalveolar lavage in progressive systemic scleroderma (author's transl)].

As an enlargement of conventional diagnostic methods the cell spectrum of bronchoscopically obtained bronchoalveolar lavage was investigated in 10 patients with progressive systemic scleroderma. Two cases showed marked lymphocytosis, one increased numbers of granulocytes, two patients had marginally increased percentages of inflammatory cells. Four of these patients were in a progressive active phase of scleroderma with humoral inflammation signs and round cell infiltrates of the skin. In contrast, the lavage cell picture of the other five patients was unremarkable. One of these had advanced pulmonary fibrosis, however without signs of progression. The results show that the lavage cell pattern reflects the acute phase with cellular inflammation of lung parenchyma disease such as occurs in progressive systemic scleroderma. Staging of pulmonary organ manifestation in progressive systemic scleroderma can be markedly improved by low-risk bronchoalveolar lavage.

Adult↗