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PubMed1999

Bowel problems in patients with systemic sclerosis.

BACKGROUND: The impact of systemic sclerosis on bowel function is still unknown. The aim of this study was therefore to assess the frequency and severity of colorectal problems among patients with systemic sclerosis and to determine whether these problems are associated with age, gender, type of systemic sclerosis, or time since diagnosis. METHODS: A detailed questionnaire describing diarrhoea, constipation, obstructed defecation, faecal incontinence, bowel habits, social activities, and quality of life was sent to 96 consecutive patients with systemic sclerosis. RESULTS: Among 83 respondents (86%) 16% did not have a normal desire to defecate, 18% regularly needed digital stimulation or evacuation of the rectum, and 38% had faecal incontinence. Most patients (79%) had episodes of diarrhoea, and 38% had this once or more each month. Overall, 20% reported that colorectal dysfunction caused some or a major restriction of social activities or the quality of life. CONCLUSIONS: Colorectal dysfunction is very common among patients with systemic sclerosis, often restricting social activities and the quality of life. Therefore, further studies of colorectal pathophysiology in patients with systemic sclerosis are needed.

Anal Canal↗
PubMed2006

Effect of linear polarized light irradiation near the stellate ganglion in skin blood flow of fingers in patients with progressive systemic sclerosis.

OBJECTIVE: The purpose of this study is to evaluate the effect of linear polarized light irradiation near the stellate ganglion area on cutaneous blood flow in fingers of patients with progressive systemic sclerosis. BACKGROUND DATA: Sympathetic overactivity is known to be present in patients with progressive systemic sclerosis. Recently introduced linear polarized light irradiation is designed to simulate noninvasive stellate ganglion block to decrease sympathetic output. METHODS: Five patients with progressive systemic sclerosis and three normal healthy controls were studied. Linear polarized light (Super Lizer) was irradiated near the stellate ganglion on the right side of the neck at 358 J/cm(2) for 10 min. Then, laser Doppler flowmetry, laser Doppler imager, and capillary microscopy were used to measure the cutaneous blood flow of the right fourth finger for 30 min. RESULTS: No significant alternations of the skin blood flow between normal controls and patients with progressive systemic sclerosis after linear polarized light irradiation were detected. The effect of linear polarized light on the microcirculation of patients with progressive systemic sclerosis was minimal and transient. CONCLUSION: The effect of linear polarized light in treating patients with progressive systemic sclerosis may not result from the improvement of skin blood flow. Therefore, the use of linear polarized light in those patients to increase cutaneous blood flow should not be overemphasized.

Adult↗
PubMed2000

Pathophysiological mechanisms of oropharyngeal dysphagia in amyotrophic lateral sclerosis.

We investigated the pathophysiological mechanisms of dysphagia in amyotrophic lateral sclerosis. Forty-three patients with sporadic amyotrophic lateral sclerosis were examined by clinical and electrophysiological methods that objectively measured the oropharyngeal phase of voluntarily initiated swallowing, and these results were compared with those obtained from 50 age-matched control subjects. Laryngeal movements were detected by a piezoelectric sensor and EMG of submental muscles, and needle EMG of the cricopharyngeal muscle of the upper oesophageal sphincter of both the amyotrophic lateral sclerosis and control groups was recorded during swallowing. Amyotrophic lateral sclerosis patients with dysphagia displayed the following abnormal findings. (i) Submental muscle activity of the laryngeal elevators, which produce reflex upward deflection of the larynx during wet swallowing, was significantly prolonged whereas the laryngeal relocation time of the swallowing reflex remained within normal limits. (ii) The cricopharyngeal sphincter muscle EMG demonstrated severe abnormalities during voluntarily initiated swallows. The opening of the sphincter was delayed and/or the closure occurred prematurely, the total duration of opening was shortened and, at times, unexpected motor unit bursts appeared during this period. (iii) During voluntarily initiated swallows there was significant lack of co-ordination between the laryngeal elevator muscles and the cricopharyngeal sphincter muscle. These results point to two pathophysiological mechanisms that operate to cause dysphagia in amyotrophic lateral sclerosis patients. (i) The triggering of the swallowing reflex for the voluntarily initiated swallow is delayed and eventually abolished, whereas the spontaneous reflexive swallows are preserved until the preterminal stage of amyotrophic lateral sclerosis. (ii) The cricopharyngeal sphincter muscle of the upper oesophageal sphincter becomes hyper-reflexic and hypertonic. As a result, the laryngeal protective system and the bolus transport system of deglutition lose their co-ordination during voluntarily initiated swallowing. We conclude that these pathophysiological changes are related mainly to the progressive degeneration of the excitatory and inhibitory corticobulbar pyramidal fibres.

Adult↗
PubMed2001

Increased urinary pyridinoline cross-link compounds of collagen in patients with systemic sclerosis and Raynaud's phenomenon.

OBJECTIVE: To study concentration changes in collagen degradation markers in patients with diffuse and limited cutaneous systemic sclerosis and patients with scleroderma-related diseases. METHODS: Pyridinoline cross-link compounds were analysed in urine samples using high-performance liquid chromatography. Samples were analysed for pyridinoline (Pyr), deoxypyridinoline (Dpyr) and soft-tissue pyridinoline (stPyr) in patients with diffuse cutaneous systemic sclerosis (dcSSc, n=23) and limited cutaneous systemic sclerosis (lcSSc, n=48) and in patients with scleroderma-related diseases such as primary Raynaud's phenomenon (pRP, n=16) and secondary Raynaud's phenomenon (sRP, n=14). Healthy controls (n=18) and patients with post-menopausal osteoporosis (OP, n=35) were also investigated. RESULTS: Urinary Pyr, Dpyr and stPyr concentrations were significantly higher in patients with Raynaud's phenomenon and systemic sclerosis than in healthy controls. The highest concentrations (two to three times greater than in healthy controls) were found in patients with dcSSc. The stPyr concentration was significantly higher in patients with dcSSc than in those with lcSSc, sRP and pRP. No significant difference in stPyr concentration was found between the healthy controls and the OP group, suggesting that stPyr is derived from soft tissues rather than bone. The extent and severity of skin involvement, measured as a skin score, significantly correlated with the concentrations of stPyr and Pyr, whereas no such correlation was found for Dpyr. CONCLUSIONS: Increased urinary concentrations of piridinoline cross-links reflect alterations in collagen turnover in both Raynaud's phenomenon and systemic sclerosis. The close correlation between stPyr concentration and the extent of skin involvement in systemic sclerosis suggests that this parameter may be useful in monitoring ongoing fibrosis in this disease.

Amino Acids↗
PubMed2005

Microchimerism and systemic sclerosis.

PURPOSE OF REVIEW: Although 6 years have elapsed since the initial report describing the presence of microchimeric cells in affected tissues from patients with systemic sclerosis, a mechanism by which these cells might contribute to the pathogenesis of the disease is still unknown. This article reviews the published literature related to the possible role of microchimeric cells in the pathogenesis of systemic sclerosis. RECENT FINDINGS: Numerous studies have reported the identification or quantification of microchimeric cells in the peripheral blood or tissues from systemic sclerosis patients; however, only one study to date has investigated their function. Recent investigations have demonstrated microchimeric cells in the clinically uninvolved tissues from patients with systemic sclerosis, suggesting that these cells are present in early disease. However, after the identification of microchimeric cells in blood and tissues of patients with systemic sclerosis, these cells have been found in organs affected by nonautoimmune conditions. These cells are also commonly detected in the peripheral blood of healthy people. SUMMARY: These observations have raised questions about whether microchimeric cells are responsible for the pathologic events in systemic sclerosis or are merely remnants of a pregnancy remote in time, and it has been suggested that they might also have beneficial effects for the host.

Adult↗
PubMed2006

The X chromosome and systemic sclerosis.

PURPOSE OF REVIEW: Similar to the majority of autoimmune rheumatic diseases, systemic sclerosis is characterized by a striking female predominance superimposed on a predisposing genetic background. At least two genetic mechanisms have been proposed that play a role in susceptibility to systemic sclerosis; firstly the maintenance of immune tolerance via genes on the X chromosomes and, secondly, fetal microchimerism. Based on these lines of evidence, experimental efforts have been most recently dedicated to investigating the role of X chromosome abnormalities (i.e. monosomy rates and inactivation patterns) in autoimmunity. We will review herein the most recent data on the role of the X chromosome in systemic sclerosis onset and discuss the potential implications. RECENT FINDINGS: Women with systemic sclerosis manifest an enhanced rate of X monosomic cells in peripheral blood compared with healthy age-matched women. Furthermore, a severely skewed X chromosome inactivation pattern is found in women with systemic sclerosis. SUMMARY: These observations, reproduced in other female-predominant autoimmune diseases, strongly support the role of the X chromosome in conferring susceptibility to tolerance breakdown and open novel scenarios to emphasize the unknown etiopathogenesis of systemic sclerosis. The implications of these findings will be discussed.

Autoimmunity↗
PubMed2004

Histopathological changes of cervical tissue in women with systemic sclerosis.

Systemic sclerosis is a connective tissue disease that can affect almost any organ of the body. The clinical aspects of systemic sclerosis on the reproductive system have been studied in large series, and an increased rate of cesarean section has been reported. For this reason, in the present study the histopathological features of cervical specimens of hysterectomyzed women with systemic sclerosis were evaluated. An increased frequency of vascular and stromal abnormalities in cervical specimens of women with systemic sclerosis were observed. Vascular medial hypertrophy, intimal thickening, and fibrosis were more often encountered in the cervical specimens of the patients with systemic sclerosis. Some of the histopathological features also showed correlation with the clinical profile of the disease. The patients with vascular medial hypertrophy in their cervical specimens were older, had a higher Rodnan score, and had longer duration of the disease. In contrast to vascular medial hypertrophy, periadventitial edema was found in the cervical specimens of the patients who were younger, had a lower Rodnan score, and had shorter duration of the disease. It was concluded that the problems that are seen in common obstetric and gynecological practices in patients with systemic sclerosis may be explained by these tissue abnormalities.

Adult↗
PubMed1993

Progressive systemic sclerosis sine scleroderma in a child presenting as nocturnal seizures and Raynaud's phenomenon.

Progressive systemic sclerosis sine scleroderma, as well as neurological manifestations of progressive systemic sclerosis are rare in adult-onset cases. Neither have been reported in children with progressive systemic sclerosis, either separately or together. We describe a six-year-old girl with nocturnal seizures and Raynaud's phenomenon of three years' duration. She died of cardiopulmonary sclerosis without ever fitting the required criteria of systemic sclerosis. Nailfold capillaroscopy revealed the specific "scleroderma-pattern" and provided the only clue for a diagnosis of progressive systemic sclerosis, confirmed eventually by skin biopsy.

Child↗
PubMed2005

Increased plasma soluble CD40 ligand concentrations in systemic sclerosis and association with pulmonary arterial hypertension and digital ulcers.

BACKGROUND: Increased expression of CD40L has been reported on activated CD4+ T lymphocytes in systemic sclerosis. CD40L can be expressed in soluble form (sCD40L). OBJECTIVE: To compare sCD40L concentrations in patients with systemic sclerosis and healthy controls. METHODS: Quantitative sandwich ELISA was used to measure plasma sCD40L in systemic sclerosis (n = 50) and matched healthy controls (n = 20). Patients with systemic sclerosis had limited cutaneous disease (29), digital ulcers (14), pulmonary arterial hypertension (PAH) (10), pulmonary fibrosis on CT (23), positive anti-Scl70 (14), and anti-centromere antibodies (10). Calcium channel blockers were discontinued 72 hours before measurements. RESULTS: Median (range) sCD40L concentration (pg/ml) was higher in systemic sclerosis than in controls (495 (10 to 7720) v 79 (50 to 118); p = 0.003), in limited cutaneous disease v diffuse disease (620 (20 to 7720) v 250 (10 to 2690); p = 0.005), in patients with digital ulcers v those without (1430 (36 to 7720) v 370 (10 to 2320); p = 0.002), and in those with PAH v those without (995 (15 to 3850) v 400 (10 to 7720); p = 0.048). sCD40L correlated with pulmonary arterial pressure estimated by Doppler echocardiography (r = 0.41; p = 0.005). CONCLUSIONS: The soluble form of CD40L is increased in plasma in systemic sclerosis and may be associated with vascular complications of the disease.

Adult↗
PubMed1999

Systemic sclerosis revealing T-cell lymphoma.

We describe a case of systemic sclerosis (SSc) occurring together with malignant lymphoma. A 43-year-old man, who had noticed sclerodactyly 1 month before consultation, was admitted for progressive skin sclerosis on his forearms and chest. SSc was diagnosed. Immediately after admission, skin sclerosis rapidly extended to the neck and trunk, and subcutaneous tumors developed on the neck, chest and back. Skin sclerosis was prominent at the sites where subcutaneous tumors were present. The tumors were diagnosed as non-Hodgkin's lymphoma of T-cell phenotype derived from soft tissue. Following 4 cycles of chemotherapy, he had complete remission and the skin sclerosis remarkably improved. It is possible that cytokines produced by T-cell lymphoma cells were responsible for the development of skin sclerosis in this case.

Adult↗
PubMed1996

Systemic sclerosis in German uranium miners under special consideration of autoantibody subsets and HLA class II alleles.

Systemic sclerosis (scleroderma) is a connective tissue disease with a wide range of clinical manifestations, with high or low degrees of skin and internal organ involvement together with different antinuclear antibody (ANA) specificities. Several studies provide evidence that males, who are rarely affected by systemic sclerosis, have an increased risk when working in mines. Therefore we reinvestigated 21 male subjects and 6 cases of deceased male patients who had been engaged in East German uranium mines and had shown evidence of this disease in medical examinations. Dermatological investigations, evaluation of chest X-rays and autoantibody estimation were performed. PCR-sequence-specific oligonucleotide typing was used to study the genetic association of HLA-D alleles with autoantibodies typical for scleroderma in these uranium miners suffering from systemic sclerosis and in patients with idiopathic systemic sclerosis. The determined HLA phenotype frequencies and the following statistical analysis (Fisher's exact test (2-sided)) revealed that in comparison with randomly selected controls, alleles DRB1*0300 (DR3) and DQB1*0201 (DQ2) were distinctly increased in the group of affected uranium miners, especially in those with anti-Scl-70 positivity. In contrast, we did not observe significant differences between affected and unaffected miners. Comparing anti-Scl-70-positive affected uranium miners with anti-Scl-70-positive idiopathic systemic sclerosis cases. DRB1*0300 as well as DQB1*0201 were also significantly enhanced in the former group. ACA-positive systemic sclerosis miners had significantly elevated frequencies in DRB1*0100 (DR1) and DRB1*0800 (DR8) only in comparison with unaffected miners and unexposed controls. Our genetic and immunological data lead to the assumption that a different set of HLA-D alleles in combination with exogenous factors is involved in the induction of anti-Scl-70 antibodies in uranium miners that might influence their susceptibility to the disease, whereas the same occupational exposure seems to have no influence on the induction of ACA antibodies.

Adult↗
PubMed1989

Diaphragmatic excursion after pleural sclerosis.

Chemical sclerosis of the pleural space is used to prevent recurrence of spontaneous pneumothorax. To test whether sclerosis restricts diaphragmatic excursion, we measured diaphragmatic excursion by ultrasonography in subjects with unilateral pleural sclerosis and compared it with diaphragmatic excursions in normal subjects, in subjects with cystic fibrosis (a diffuse bilateral lung disease), and in those who underwent surgical procedures that obliterate the pleural space. In five subjects with unilateral chemical sclerosis, diaphragmatic excursion was significantly less on the sclerosed side than on the contralateral side (10.7 +/- 1.3 vs 17.3 +/- 1.7 mm, mean +/- SEM; p less than .01). Compared with those of normal subjects, the side-to-side differences in excursion were increased by pulmonary disease (p less than .03) and additionally by unilateral sclerosis (p less than .015). There was no significant difference between diaphragmatic excursions on left and right sides of subjects without history of pleural disease. These data suggest that chemical pleural sclerosis causes a measurable reduction in diaphragmatic excursion on the affected side. The physiologic significance of this effect is not known.

Adolescent↗
PubMedDate not supplied

[Chorea as the presenting form of progressive systemic sclerosis].

We present an exceptional case of a patient with hemichorea of ischemic origin as a form of presentation of progressive systemic sclerosis. The neurologic complications, both central and peripheral, of this condition are considered infrequent and may be due to coexisting or iatrogenic factors, or may be a direct complication of progressive systemic sclerosis. There have been occasional reports of cerebrovascular disease in progressive systemic sclerosis, and this apparent rarity may be related to structural and morphological variations in brain arteries. Recently, there have been reports of primary cerebrovascular changes and vascular calcifications in progressive systemic sclerosis. There are no reported cases associated with movement disorders. The patient is a 66-year-old ex-smoker, and no other vascular risk factors, with chronic Raynaud's phenomenon affecting both hands, and polyarthralgias. Three months previously he began to complain of choreic movements affecting the right-sided limbs without any other neurological manifestations. The abnormal movements responded to haloperidol. There were skin changes suggestive of progressive systemic sclerosis, and capillaroscopy showed abundant avascular zones with dilated capillary loops. Anticentromere antibody titer was elevated to 1/1280. Brain arteriography was normal, and the brain SPECT showed bilateral hypoperfusion bilaterally, predominantly on the left side. Brain MR imaging confirmed an infarct in the left caudate and adjacent internal capsule. The study of this case supports the hypothesis of a direct primary cerebrovascular damage in progressive systemic sclerosis.

Aged↗
PubMed2003

The pattern of esophageal manometry in progressive systemic sclerosis.

OBJECTIVE: The purpose of this paper is to study the esophageal motility pattern and the frequency of acid reflux in patients diagnosed to have progressive systemic sclerosis and compare the results to that of normal controls. METHODS: All consecutive patients diagnosed to have progressive systemic sclerosis between 1417-1419 (Hijra year) at King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia were included (Group I). History of heartburn, dysphagia and regurgitation was reviewed. Drugs that might suppress acid or alter motility were discontinued 2 weeks before inclusion. The results were compared to that of 21 symptomatic controls of similar age and sexes that were seen at the same period (Group II). Esophageal manometry and upper gastrointestinal endoscopy were performed in all patients. Ambulatory 24 hour-pH monitoring was carried out in 6 patients of group 1 and 20 patients of group II. RESULTS: Thirteen progressive systemic sclerosis patients (12 females) mean age was 38.7 years and 21 (19 females) mean age was 34.8 years were included. The symptom scores, lower esophageal sphincter pressure, esophageal contractions amplitude were significantly worse in patients compared to control, dysphagia was mostly due to aperistalsis. All progressive systemic sclerosis patients showed the typical esophageal manometry pattern of lower esophageal sphincter pressure and diminished amplitude with aperistalsis. Gastroesophageal reflux was detected in 83% of patients with progressive systemic sclerosis. Moreover, all 24-hour pH monitoring variables were significantly worse in group I. CONCLUSION: Patients with progressive systemic sclerosis usually present with heartburn, dysphagia and regurgitation. Esophageal manometry typically shows lower pressure and aperistalsis. Gastroesophageal reflux is frequent.

Adult↗
PubMed1992

Left ventricular diastolic function in systemic sclerosis: assessment by radionuclide angiography.

The aim of this study was to assess left ventricular function in subjects with systemic sclerosis. Twenty-four women with systemic sclerosis (mean age 48 +/- 11 yr) and 14 age- and sex-matched normal subjects were studied by radionuclide angiography performed at rest with a temporal resolution of 20 msec/frame. Left ventricular volume curves were generated and indices of systolic and diastolic function were computed. Left ventricular diastolic asynchrony was evaluated by dividing the left ventricle into five regions and then computing the time-to-peak filling rate for each region. After excluding the valvular region, the coefficient of variation of this index was obtained. The isovolumic relaxation period was prolonged in systemic sclerosis patients in comparison to normal subjects (127 +/- 39 msec versus 87 +/- 44 msec, p less than 0.05). Moreover, 38% of the systemic sclerosis patients had a subnormal peak filling rate. Left ventricular diastolic asynchrony was increased in the systemic sclerosis group, as expressed by a higher coefficient of variation of the regional time to peak filling rate (27.9% +/- 11.5% versus 14.5% +/- 8.6%, p less than 0.05). Our results indicate an impaired relaxation and an increased diastolic asynchrony in patients with systemic sclerosis.

Female↗
PubMedDate not supplied

Calcium influx into red blood cells: the effect of sera from patients with systemic sclerosis.

The rheology of red blood cells in patients with systemic sclerosis is abnormal. To investigate this further we have examined the effect of sera from patients with systemic sclerosis on the handling of calcium ions by the erythrocyte membrane. Normal erythrocytes were filled with a photoprotein (aequorin) which emits light on contact with calcium. These photoprotein loaded normal erythrocytes were then incubated overnight with serum from: normal subjects (n = 20), from patients with systemic sclerosis (n = 27) or from patients with primary Raynaud's disease (n = 10). There was no significant difference in basal calcium leakage, as measured by the amount of light produced following the addition of triton X-100. Induced calcium influx, as measured by the amount of light produced following the addition of ionophore A23817, was significantly greater in the photoprotein loaded erythrocytes incubated overnight with serum from patients with systemic sclerosis compared to those incubated with serum from normal subjects (p less than 0.02) or patients with primary Raynaud's disease (p less than 0.01). This modulation of Ca2+ handling in erythrocytes by a serum factor from patients with systemic sclerosis could account for the alterations in erythrocyte function, such as red cell deformability, observed in systemic sclerosis.

Adult↗
PubMed1990

Endoscopic sclerosis in the treatment of bleeding peptic ulcers with a visible vessel.

We have assessed the efficacy of endoscopic sclerosis in the routine clinical management of patients with bleeding ulcers, in whom a visible vessel was identified at endoscopy. Over a period of 14 months, a visible vessel at the base of an ulcer was identified in 53 patients. Three patients could not undergo sclerosis, one because of torrential bleeding and two because of ulcer location. Fifty patients, 18 with a bleeding vessel and 32 with a nonbleeding vessel, were subjected to endoscopic sclerosis with adrenaline plus polidocanol. Permanent hemostasis was achieved in 45 patients (90%), 31 (62%) cases with one session and 14 (28%) cases with two sessions of sclerosis. Four patients had persistent rebleeding in spite of two sessions of sclerosis, and one patient had a massive rebleeding after the first session; all underwent emergency surgery. We believe that endoscopic sclerosis has a primary role in the treatment of ulcer bleeding, particularly in those patients in whom a visible vessel is identified at endoscopy.

Aged↗
PubMed1989

[Clinical pathology of the glomerulus--from phenomenon to entity. Focal sclerosis].

Three main types of global glomerular sclerosis (glomerular obsolescence) can be differentiated by light microscopy, immunohistochemistry and electron microscopy: the compressive type is characterized by an obliteration of the urinary space by type I collagen, the matrix type by a massive accumulation of mesangial matrix (sclerosis), and the insudative type is accompanied by marked subendothelial deposition of hyalin. Thus, different mechanisms may lead to glomerular obsolescence (scarring) even if mixed forms are frequently encountered. Segmental glomerular sclerosis represents a non-specific lesion observed in a variety of conditions. An increase in mesangial matrix and subendothelial deposition of hyalin material are the predominant histological features. The presence of segmental glomerular sclerosis, however, is of particular significance when occurring in the setting of an idiopathic nephrotic syndrome. The majority of patients (90%) are steroid-resistant and prone to develop renal failure. A rapidly progressive course is frequently observed when segmental lesions are superimposed to diffuse mesangial proliferation. The relationship between minimal change nephrotic syndrome and focal segmental glomerular sclerosis is still controversial. It is uncertain whether they represent two distinct, unrelated entities or whether they belong to the spectrum of a disease with focal segmental glomerular sclerosis being the more ominous end of the spectrum. The pathogenesis of segmental glomerulosclerosis is discussed.

Glomerulonephritis↗