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PubMed1982

Prostaglandin-mediated inhibition of lymphokine secretion in normal individuals and patients with progressive systemic sclerosis (scleroderma, PSS).

The sensitivity of peripheral blood lymphocytes to E-type prostaglandin-mediated inhibition of lymphokine secretion was examined in 3 groups of individuals; normal controls, hospitalized patients, and patients with progressive systemic sclerosis (PSS, scleroderma). Leukocytes were stimulated by a polyclonal T-cell activator, phytohemagglutinin, and the release of the lymphokine, leukocyte migration inhibitory factor (LIF), was measured in the presence or absence of exogenous PGE2 using a direct agarose droplet migration inhibition technique. Leukocytes of scleroderma patients were found to be hyporesponsive to E-type prostaglandin (i.e., lymphokine secretion by these cells was not inhibited at concentrations of PGE2 between 2.8 X 10(-8) and 2.8 X 10(-5) M). In addition, a marked sex difference in PGE responsiveness was found to exist among normal controls, whereby females were hyporesponsive during the latter half of the menstrual cycle. It is possible that this deficit may facilitate, in part, the development of connective tissue diseases in women of childbearing age. The inability to suppress lymphokine production and arrest persistent immune reactivity, coupled with the known ability of lymphokines to augment fibroblast collagen production, offers a a reasonable explanation for the accumulation of tissue collagen in scleroderma.

Adult↗
PubMed1989

An automatic analysis method for in situ hybridization using high-resolution image analysis.

Specific mRNA for alpha 1 (I) collagen has been detected on a cellular level by in situ hybridization using radioactively labelled alpha 1 (I) antisense RNA probes. We here present an automatic, quantitative, evaluation technique for the determination of grain densities using the high-resolution image analysis system IPS KONTRON. The reliability and objectivity of this method were evaluated by comparing the values of grains per cell obtained by conventional and automatic techniques following in situ hybridization with alpha 1 (I) collagen DNA probes in various specimens. The correlation coefficients between conventional and automatic analysis of grain densities were 0.97 for fibroblasts embedded into a three-dimensional collagen gel, 0.94 for dermal fibroblasts in skin obtained from a patient with progressive systemic scleroderma, and 0.90 for fibroblasts in a 2-week-old scar. All correlation coefficients were significant on the P less than 0.0001 level. This new analysis technique therefore allows a more rapid and reliable quantitative evaluation of in situ hybridization and may also be helpful in differentiating between various cell populations characterized by different biosynthetic capacities.

Autoradiography↗
PubMed2006

[Systemic sclerosis].

Systemic sclerosis (SSc; syn. systemic scleroderma) is a rare autoimmune disorder with characteristic cutaneous manifestations. Prevalence in women is fivefold higher than in men. The course of the disease is slowly progressive with a variable degree of internal organ involvement due to fibrosis and obliteration of small vessels. The diffuse form shows more frequent and severe organ manifestations compared to the limited form. Increased mortality is particularly related to a cardiopulmonary involvement leading to a 5-year survival of around 75%. Treatment indications are dependent on the severity of the disease. In the acute state, immunosuppressive agents are needed in case of significant organ involvement. Vasodilative drugs are often used for the symptomatic treatment of Raynaud's phenomenon.

Autoantibodies↗
PubMed1993

[Arteriopathies as occupational diseases].

Arteriopathies of occupational diseases predominate in the upper limbs and are usually expressed as Raynaud's phenomenon ("white finger"), sometimes complicated by digital gangrene. Hand-arm vibration disease, hypothenar hammer syndrome, exposure to silica even without silicosis and vinyl chloride intoxication are the principal causes of occupational diseases acknowledged as such by the laws. In a personal series of 60 patients with complicated upper limb arteriopathies, 25% had a recognized occupational disease. Since December 1992, Erasmus' syndrome and systemic scleroderma due to silica without silicosis have been accepted in France as being occupational diseases. The therapeutic problems are discussed, notably the surgical indications in hypothenar hammer syndrome, the possible medical treatments of finger necrosis occurring in Erasmus' syndrome and, in particular, the role played by spinal cord neurostimulation.

Air Pollutants, Occupational↗
PubMed2002

[Bidirectional cell traffic between mother and fetus: new insights in pediatric and adult disorders].

There is a heavy traffic of cells and DNA through the placenta during pregnancy. The rate of fetal cells in the maternal blood is correlated with abnormalities, such as aneuploidy and pre-eclampsia. Studying and quantifying these cells could improve antenatal diagnosis techniques, especially for Down syndrome. Maternal-fetal microchimerism is frequently observed in several auto-immune diseases in adulthood, such as systemic scleroderma. Studies suggest a rather allo-immune pathophysiology, involving maternal-fetal HLA compatibility. Microchimerism is also found in auto-immune diseases in children. Thus, the cells traffic offers new insights for antenatal diagnosis techniques and pathophysiology of auto-immune diseases.

Adult↗
PubMed2000

Pseudoscleroderma associated with lung cancer: correlation of collagen type I and connective tissue growth factor gene expression.

Pseudoscleroderma as a paraneoplastic syndrome is a rare disease. We report here a patient with lung cancer (undifferentiated squamous cell carcinoma), who developed acrosclerosis. Using in situ hybridization, marked expression of alpha1(I)-collagen and connective tissue growth factor (CTGF) mRNA was found in fibroblasts scattered throughout the dermis. However, transforming growth factor (TGF)-beta1 expression was not detected. The pattern of CTGF gene expression and collagen synthesis was similar to that in systemic scleroderma. The absence of TGF-beta1 mRNA could indicate that tumour-derived factors induce the expression of CTGF.

Aged↗
PubMed1998

[The value of electrogastrography for the diagnosis of gastroenterologic function].

BACKGROUND AND OBJECTIVE: Abnormalities of gastric myoelectric activity can be measured by electrogastrography (EGG). Such dysfunctions can be associated with disorders of gastric motility. It was the aim of this study to investigate the relationship between gastric electrical activity and motility in patients with dyspepsia due to gastrointestinal or extraintestinal disease. PATIENTS AND METHODS: 135 consecutive patients with dyspepsia (standardized score) were enrolled in this prospective study including patients with functional dyspepsia (FD) (n = 25), dyspepsia in diabetes mellitus type II (n = 27), hyperthyroidism (n = 23), progressive systemic scleroderma (PSS) (n = 20), chronic alcoholism (n = 20), and 20 patients with gastric lesions: gastric ulcer (n = 10) or gastric cancer (n = 10). The EGG measurements were performed over 60 min pre- and post-prandially state after ingestion of a solid-liquid test meal (370 kcal). The following parameters were measured: dominant electrical frequency (DF), percentage of DF in the normal frequency range (2-4 cpm), bradygastria (< 2 cpm), tachygastria (4-10 cpm), dominant frequency instability coefficient (DFIC), and power ratio. The data were compared with results in 40 healthy persons. The gastric emptying was determined by the gastric retention of 99mTc colloid after 60 min (gamma camera). RESULTS: Nearly 50% of FD patients had delayed gastric emptying (gastric retention after 60 min > 68%): they patients exhibited significantly more tachygastrias than those with normal gastric emptying (P < 0.05). Patients with diabetes mellitus type II, PSS and chronic alcoholism showed normal electrical activity, although gastric emptying was delayed in nearly 50%. The hyperthyroid patients had increased tachygastria without abnormal gastric motility. Gastric lesions did not produce pathological electrogastrograms. The dyspepsia score did not correlate with either EGG or radioscintigraphy in the various patient groups. CONCLUSIONS: Electrogastrography can detect tachygastrias, which are significantly increased in some patients with functional dyspepsia. Because of therapeutic consequences electrogastrography seems to be indicated in patients with functional dyspepsia.

Adult↗
PubMed2001

Pseudoainhum of all fingers associated with Reynolds' syndrome and breast cancer: report of a case and review of the literature.

Pseudoainhum is a rare disorder characterized by digital constrictions leading to autoamputation. We report a bilateral pseudoainhum of all fingers in a 64-year-old fair-skinned woman with breast cancer, systemic scleroderma, and primary biliary cirrhosis. The overlap between scleroderma and primary biliary cirrhosis with antimitochondrial antibodies M2 is also known as Reynolds' syndrome. Although pseudoainhum has been associated with many conditions, this particular association is exceptional and has yet not been described.

Ainhum↗
PubMed1998

A case study and national database report of progressive systemic sclerosis and associated conditions.

We report the case of a 34-year-old white woman with a history of progressive systemic scleroderma (PSS) and diffuse alveolar hemorrhage (DAH) that may be either a rare complication of PSS or induced by D-penicillamine. The DAH progressed to hemoptysis and led to intubation for airway protection. The patient progressed to acute renal failure. Her chest x-ray revealed diffuse bilateral infiltrates. She developed pulmonary fibrosis with secondary pulmonary hypertension. She experienced a brief period of improvement of her respiratory status after steroid treatment. We also report a database of 21,442 decedents with PSS over a 15-year period from 1979 to 1994. Our report demonstrates that of over 21,000 decedents, only 0.2% had pulmonary hemorrhage or hemoptysis or both listed as a cause of death. The data also demonstrate that PSS was the underlying cause of death more frequently in younger people. Age-adjusted mortality rates were higher for blacks than for whites and for women than for men.

Acute Kidney Injury↗
PubMed1992

Nodular scleroderma in systemic sclerosis under D-penicillamine therapy.

A case of systemic sclerosis (SS) which developed keloidal lesions (nodular scleroderma) on the chest during D-penicillamine (DPC) therapy is reported. The 36-year-old woman showed rapidly progressing skin sclerosis with lung and esophageal involvement, and DPC was started at the age of 38. Skin sclerosis as a whole had improved to some extent, when keloidal nodules developed on the upper chest at the age of 44. Since there were no other findings suggestive of adverse reactions caused by DPC, we speculate that activation of the fibroblasts in these lesions occurred despite the suppressive effect of DPC.

Adult↗
PubMed1999

Topical tocoretinate improved hypertrophic scar, skin sclerosis in systemic sclerosis and morphea.

Four patients with systemic scleroderma (SSc), 4 patients with morphea, and 4 patients with hypertrophic scar were treated with topical tocoretinate for 6 months to 3 years and studied clinically and histopathologically. Clinically, all of the lesions responded to this therapy. The stiffness of the skin lesions, glossy appearance of the lesions, and telangiectasia improved. Histopathologically, the proliferated collagen fibers decreased in thickness, and the inter-fiber spaces increased. Immunoreactive tenascin-C expressed in the proliferated deep dermal fibers of the SSc and hypertrophic scar lesions was markedly decreased compared with the level before the topical tocoretinate therapy. Topical tocoretinate has been used for the treatment of ulcers; it is also a potent treatment for sclerotic skin diseases.

Administration, Topical↗
PubMed1990

Are ACA and Scl 70 antibodies mutually exclusive?

Serological studies using indirect immunofluorescence, double immunodiffusion and immunoblot with recombinant Topo I and kinetochore antigens were performed in 180 patients with systemic scleroderma. In the sera of 10 patients, we found coexisting ACA and Scl 70 antibodies, and this was confirmed by all three methods. The relationship between the coexistence of these antibodies and the clinical features of the disease is not clear, although in all these cases there was pronounced vascular involvement.

Adult↗
PubMed1989

Immunopathologic findings in systemic sclerosis patients: clinical and immunopathologic relationships.

Direct immunofluorescence (DIF) of the skin biopsy specimens was performed in 62 patients with systemic scleroderma: 26 cases of acrosclerosis (group 1), 26 cases of diffuse scleroderma (group 2) and ten cases of transitional form acrosclerosis-diffuse scleroderma (group 3). If epidermal nuclear fluorescence and lupus band test (LBT) were considered, the results were positive in 15.3% in group 1, 42.3% in group 2, and 10% in group 3. LBT was positive in 13.5% of the cases in groups 1 and 2 and negative in group 3. The immunoglobulin deposits at the dermoepidermal consisted mainly of IgM or a combination of IgG and IgM. There was no correlation between the LBT and antinuclear antibodies as detected by indirect immunofluorescence (IIF) or double immunodiffusion (DID).

Adult↗
PubMed1996

Electron-microscopic study of sclerodermatous chronic graft-versus-host disease.

BACKGROUND: Sclerodermatous chronic graft-versus-host disease (SC-GVHD) resembles systemic scleroderma (SSD) closely, both clinically and histologically. Our purpose was to try to define the morphologic differences of collagen fibers between SC-GVHD and SSD. MATERIALS AND METHODS: Using electron microscopy, we compared the morphology of collagen fibers in a 15-year old girl with SC-GVHD with those of three patients with SSD. RESULTS: In SC-GVHD, sclerosis is located in the superficial dermis and collagen fibers of irregular diameter are seen in the subepidermal area. In SSD, sclerosis is seen in the lower dermis and subcutaneous fatty tissue, and collagen fibers of irregular diameter are located in the deep dermis. Some of the collagen fibers were degenerative in the superficial dermis in SC-GVHD. We observed low-density, round structures in cross sections of collagen fibers. CONCLUSIONS: The difference in initial location and morphologic appearance of collagen fibers may indicate a different pathogenesis in SC-GVHD compared to SSD.

Adolescent↗
PubMedDate not supplied

Detection of enhanced monohydroxyeicosatetraenoic acid and F2-isoprostane levels in human plasma samples after extracorporeal photoimmunotherapy.

To investigate the involvement of reactive oxygen species in extracorporeal photoimmunotherapy (photopheresis), we have introduced two highly sensitive and specific techniques for the detection and quantitative measurement of oxygenated nonenzymatically formed arachidonic acid isomers [mono-hydroxyeicosatetraenoic acids (HETEs) and F2-isoprostanes] by gas chromatography-mass spectrometry/negative ion chemical ionization (GC-MS/NICI) in plasma samples of patients suffering from cutaneous T-cell lymphoma and progressive systemic scleroderma II. The analysis of HETEs involved hydrogenation, solid phase extraction on a C18 cartridge, formation of pentafluorobenzyl bromide and trimethylsilyl ether derivatives. In the case of F2-isoprostanes, the analytical procedure was similar to that of HETEs except that the hydrogenation step was omitted. In the plasma of healthy volunteers picomole amounts of 2-, 5-, 8-12-, 15-HETEs, 8-iso-PGF(2alpha) and 9alpha,11alpha-PGF(2alpha) were quantified by using 12-hydroxy-heptadecatrienoic acid and PGF(2alpha)-d4 as internal standards of HETEs and isoprostanes, respectively. Analysis of plasma samples obtained from patients before and after extracorporeal photoimmunotherapy revealed characteristic increases in both, HETE and isoprostane levels. The enhancement of indicators of lipid peroxidation is in correspondence with a moderate loss of alpha-tocopherol, the most important lipid-soluble antioxidant in human plasma. Thus, our data confirm the involvement of lipid peroxidation in extracorporeal photoimmunotherapy.

Arachidonic Acid↗
PubMed1992

Carpal tunnel syndrome and systemic sclerosis.

Carpal tunnel syndrome (CTS) was observed in 4 cases of a consecutive series of 16 patients admitted for the initial diagnosis or follow-up of systemic scleroderma from 1986 to 1990. We also observed one case of ulnar nerve compression. Neurological involvement was confirmed by electromyogram, and motor and sensory nerve conduction studies. The neurological signs appeared at the beginning of the disease in two cases and preceded the discovery of scleroderma in two. CTS is not rare in scleroderma and must be carefully sought, both clinically and electrophysiologically. Patients with 'idiopathic' CTS might need clinical follow-up for early diagnosis of scleroderma.

Adult↗
PubMed2004

Microcirculation abnormalities in patients with fibromyalgia - measured by capillary microscopy and laser fluxmetry.

This unblinded preliminary case-control study was done to demonstrate functional and structural changes in the microcirculation of patients with primary fibromyalgia (FM). We studied 10 women (54.0 +/- 3.7 years of age) with FM diagnosed in accordance with the classification criteria of the American College of Rheumatology, and controls in three groups (n = 10 in each group) - age-matched women who were healthy or who had rheumatoid arthritis or systemic scleroderma (SSc). All 40 subjects were tested within a 5-week period by the same investigators, using two noninvasive methods, laser fluxmetry and capillary microscopy. The FM patients were compared with the healthy controls (negative controls) and with rheumatoid arthritis patients and SSc patients (positive controls). FM patients had fewer capillaries in the nail fold (P < 0.001) and significantly more capillary dilatations (P < 0.05) and irregular formations (P < 0.01) than the healthy controls. Interestingly, the peripheral blood flow in FM patients was much less (P < 0.001) than in healthy controls but did not differ from that of SSc patients (P = 0.73). The data suggest that functional disturbances of microcirculation are present in FM patients and that morphological abnormalities may also influence their microcirculation.

Arm↗