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PubMed1990

[Systemic scleroderma (apropos of a case with dominant pulmonary manifestations)].

A case of systemic sclerodermy mainly affecting the lungs is presented. For a long time the diagnostic was incorrect. The case is all the more interesting since the patient had worked in iron mines and unproved pneumoconiosis had been diagnosed. Treatment basically with corticotherapy and oxygenotherapy, with D-penicillamine added, produced a satisfactory outcome to the attack and definite improvements in the cutaneous lesions.

Humans↗
PubMed2000

[Microchimera. New thought process for pathogenesis of systemic scleroderma].

Persistent cellular microchimerism might play a role in the pathogenesis of systemic sclerosis (SSc). Microchimerism results from the traffic of fetal cells through the placenta during pregnancy into the maternal circulation and their survival due to HLA class II compatibility. In female SSc patients the presence of fetal CD3-positive T-cells in the maternal circulation and of fetal cells in the affected skin tissue has been identified through y chromosome specific DNA sequences. The persistent microchimerism might cause SSc in certain patients by initiating a fetal anti-maternal Graft-versus-host-like response.

CD3 Complex↗
PubMedDate not supplied

[Systemic scleroderma and primary bronchopulmonary adenocarcinoma. A new case].

The authors report a case of systemic sclerosis associated with primary lung adenocarcinoma. This association has previously been reported in patients with old scleroderma complicated by extensive fibrosis of the lungs allegedly regarded as a precancerous lesion. The case reported here is of interest owing to the absence of pulmonary fibrosis.

Adenocarcinoma↗
PubMed1994

Digital pressure responses to cooling in patients with suspected early vs definite scleroderma (systemic sclerosis) vs primary Raynaud's phenomenon.

OBJECTIVE: To compare digital vascular responses to local finger cooling in 4 groups of subjects: patients with definite scleroderma (SD, systemic sclerosis) meeting ARA criteria (n = 16), patients with suspected early SD (n = 12), patients with primary Raynaud's phenomenon (RP) (n = 23) and a control group of 29 healthy subjects. METHODS: Their digital systolic pressures were measured at 30, 20, 15 and 10 degrees C finger cuff temperature, in a room kept at 18 degrees C. RESULTS: The results of the digital pressure measurements showed a clearcut difference between the SD, primary RP and control groups, but there was no difference between patients with definite SD and those with suspected early SD. CONCLUSION: Under our experimental conditions, digital pressure response to local cooling separates groups of patients with primary RP and those with secondary RP related to SD from each other and from controls. The similarity between digital pressure responses of patients with suspected early SD and those with definite SD, suggests an early organic involvement of the digital vasculature in SD.

Adult↗
PubMed1997

[Systemic scleroderma and cancer. Search for predictive factors of cancer in 123 patients with scleroderma].

Several studies have suggested an increased risk of cancer in patients with systemic sclerosis, but the potential risk factors for these cancers remain unknown. The aim of this study was to identify, among patients with systemic sclerosis, factors associated with the development of cancer, with attention to clinical (age, sex, cutaneous sclerosis), immunological (antinuclear antibodies, anticentromere antibodies, anti-Scl-70 antibodies) and histological (pulmonary fibrosis) features. We retrospectively studied 123 patients with systemic sclerosis. The median follow-up period was 4 years. Fourteen cases of cancer (11.3%) were found (lung n = 3, breast n = 2, ovarian n = 2, skin n = 1, thyroid n = 1, rectum n = 1, uterine cervix n = 1, larynx n = 1, pancreas n = 1, myelodysplasia n = 1). The characteristics of systemic sclerosis were similar in patients with and patients without cancer. Yet, the three cases of lung cancers occurred in association with CREST syndromes and anticentromere antibodies.

Adolescent↗
PubMed1988

[Anorectal motility in systemic scleroderma].

We prospectively compared esophageal and rectal motility data from 7 patients with progressive systemic sclerosis (4 females, 3 males) to esophageal recordings in 22 and anorectal recordings in 9 healthy controls. All patients with sclerosis exhibited motility disturbances in the lower esophageal sphincter (LES): LES resting pressure, LES relaxation amplitude and duration, and the number of incomplete LES relaxations were significantly different compared to the controls. All patients had alterations of anorectal motility: resting pressure, maximal squeeze pressure, and sphincter relaxation amplitude following balloon distension of the rectum were significantly decreased as compared to the control subjects. We conclude that esophageal and anorectal manometry are comparable in their sensitivity to differentiate between patients with systemic sclerosis and normal subjects.

Anal Canal↗
PubMed1992

[Immunogenetic studies of familial occurrence of progressive systemic scleroderma and circumscribed scleroderma].

Two different forms of scleroderma in one family are described: the mother suffers from systemic sclerosis and her daughter from linear morphoea. The observed HLA antigens indicate that systemic sclerosis and morphoea have various features in common. The immunogenetic data can be used to calculate the aetiological and preventive fractions, which together with environmental hazards and other risk factors describe the HLA-associated potential for provocation of scleroderma.

Adolescent↗
PubMed1999

[Involvement of the small intestine in systemic scleroderma].

INTRODUCTION: Though impairment of the gastrointestinal tract is commonly encountered in patients with systemic sclerosis, the most frequent abnormalities are esophageal and anorectal disorders. Involvement of the small intestine is also common, reaching a 40-80% prevalence. It often leads to life-threatening complications. CURRENT KNOWLEDGE AND KEY POINTS: The occurrence of small intestine impairment and its potential relationships with other organ impairment is still unknown. However, it rarely indicates the existence of the disease (10%) which remains asymptomatic for a long period. As clinical symptoms are non-specific and radiological tests (upper intestinal tract barium meal, gastrointestinal transit times of radiolabeled meal, computerized tomography scan) not sensitive enough to detect the symptoms, diagnosis of small intestine impairment is delayed, i.e., when severe complications such as malabsorption or pseudo-obstruction are present. The physiopathology of small intestine disorders is still unclear, leading to both collagenous fibrosis and atrophy of muscle fibers. As well, its treatment is difficult. FUTURE PROSPECTS AND PROJECTS: Knowledge of the mechanisms at the origin of small intestine impairment in the course of systemic sclerosis is important for the development of efficacious therapies. Manometry of the small intestine would be a useful tool to assess the various motor abnormalities that may occur in patients presenting with systemic sclerosis associated with either malabsorption or pseudo-obstruction. It would also provide a useful test in selecting patients whose treatment require somatostatin analogs.

CREST Syndrome↗
PubMed1997

[Effects of various prokinetic drugs on gastrointestinal transit times in patients with progressive systemic scleroderma].

The intestine is involved in about half of the cases with progressive-systemic sclerosis. Intestinal transit disturbances which are caused by neuropathy of the enteric nerve system occur frequently. However, upto-date only few studies which determined the effect of prokinetic drugs exist. Patients with intestinal involvement caused by progressive-systemic sclerosis were treated with the prokinetic drugs cisapride (20 mg, TID; n = 9), erythromycin (250 mg, TID; n = 7) and octreotide (50 micrograms s. c., at night time; n = 5) over a period of four weeks. At study entry and after each treatment period the transit times through the stomach, small and large intestine were evaluated by use of the metal-detector test. Gastric emptying was only accelerated by erythromycin (42 +/- 3 min vs. 54 +/- 6 min; p = 0.0422), whereas treatment with cisapride and octreotide did not result in significant changes (48 +/- 4 min; p = 0.3743 and 44 +/- 4 min; p = 0.1975; resp.). Small intestinal transit times were not altered significantly by cisapride (108 +/- 15 min vs. 108 +/- 9 min; p = 0.2733), crythromycin (92 +/- 8 min; p = 0.0707) or octreotide (106 +/- 12 min; p = 0.8927). Furthermore colonic transit was not fastened by none of the prokinetic agents (study entry: 68 +/- 12 h; cisapride: 88 +/- 12 h; p = 0.0569; erythromycin 77 +/- 14 h; p = 0.7349; octreotide 107 +/- 14 h; p = 0.8927). Four patients were withdrawn from the study because of diarrhea. Prokinetic drugs do not seem to have a major impact on intestinal transit times in patients with progressive-systemic sclerosis. The use of these drugs is limited because of frequent side effects.

Aged↗
PubMed1978

[Formation and destruction of the skin collagen structures in systemic scleroderma].

Histological, electron microscopic, and morphometric studies of skin biopsies from 70 patients with systemic sclerodermia showed an increase in biosynthetic processes both in the affected and nonaffected parts of the skin. Enhanced neofibrillogenesis was found only in areas of sclerodermic lesions. Abnormalities in newly formed collagen structures as well as splitting of newly formed collagen fibrillae into microfibrillae were observed. In unaffected skin areas two types of destruction of collagen fibrillae were observed: extracellular destruction and fibroplasia.

Adolescent↗
PubMed2004

[Characteristics of clinical symptoms and course of systemic scleroderma depending on sex and age of onset].

AIM: To define clinical features of systemic sclerosis (SS) in age and sex aspects. MATERIAL AND METHODS: The study covered 100 patients aged 15 to 83 years with SS (24 males and 76 females) lasting for 1-15 years (mean 6.2 +/- 4.1 years). Groups of females and males, with disease onset age under 50 years (32 years) and over 50 years were compared. RESULTS: Males had a prevalent diffuse clinical form of SS with advanced skin syndrome, primarily indurative alterations, marked disturbances of microcirculation, abnormal heart rhythm and conduction, interstitial pulmonary fibrosis with development of pulmonary hypertension. The patients with late SS onset are characterized by development of visceral pathology within the first 3 years of the disease. CONCLUSION: In making SS diagnosis and in the disease treatment it is necessary to consider the patients' sex and age, peculiarities of the debut, clinical picture, course and prognosis.

Adolescent↗
PubMed1988

[Motility disorders of the esophagus in progressive systemic scleroderma. Pathophysiology, diagnosis and therapy].

Gastrointestinal manifestations of collagen diseases are frequent. In progressive systemic sclerosis esophageal involvement is found in 60% of cases and is thus the main gastrointestinal complication. Atrophy of the smooth muscle and fibrotic degeneration of the distal esophagus result in progressive motility disorders which may cause severe reflux esophagitis with typical consequences, such as stenosis and strictures. Manometry and cinematography are basic diagnostic procedures. Esophagoscopy and long-term pH-monitoring are most useful for evaluating the degree of esophageal involvement. The severity of sclerodermatous motility disorders should be classified according to a modification of the Garrett scale, which is particularly recommended for determining the further prognosis and therapeutic approach. Esophageal involvement of grades I and II should be treated conservatively, whereas grade III is a clear indication for surgical therapy. The original Nissen type of fundoplication or distal gastric resection with Roux-en-Y anastonosis are the methods of choice.

Combined Modality Therapy↗
PubMed1993

Early undifferentiated connective tissue disease: III. Outcome and prognostic indicators in early scleroderma (systemic sclerosis).

OBJECTIVE: To characterize the course of early scleroderma and to delineate prognostic factors present within 1 year of disease onset that might identify patients at high risk. DESIGN: Inception cohort study. SETTING: Ten university-based rheumatology clinics participating in the Cooperative Systematic Studies of Rheumatic Diseases Program. PATIENTS: Forty-eight patients who had had scleroderma for less than 1 year. MEASUREMENTS: Fifteen patients with early scleroderma who died were compared with those still living during the initial study period (1982 to 1992). Kaplan-Meier survival estimation and Cox proportional hazards analysis were used to analyze baseline variables for their ability to predict survival duration. RESULTS: Eight of 15 deaths were due to cardiac or pulmonary system failure. The estimated 5-year survival rate was 68%. Baseline factors that were the most predictive of a poor outcome included the presence of abnormal cardiopulmonary signs and abnormal urine sediment (pyuria, hematuria). CONCLUSION: Evidence of early cardiopulmonary disease, renal disease, inflammation, or immune activation may identify a subset of patients with scleroderma who will experience rapidly progressive disease and early death.

Adult↗
PubMedDate not supplied

[Studies of natural killer cell cytotoxicity against human chondrocytes in patients with articular arthritic changes complicated by psoriasis and systemic scleroderma].

The natural killer (NK) activity against fetal chondrocytes was studied in patients with psoriasis and systemic sclerosis. It was shown that the cell responsible for the cytotoxic effect is a lymphocyte CD16+, CD2-. In patients with arthritic psoriasis NK activity against chondrocytes was significantly higher than in other psoriasis patients or in healthy controls. Preliminary studies show that the NK activity against chondrocytes is decreased in patients with systemic sclerosis.

Arthritis, Psoriatic↗
PubMed2001

[Pulmonary hypertension screening in systemic scleroderma: a cohort study of 67 patients].

PURPOSE: Pulmonary hypertension is a severe complication of systemic sclerosis and has emerged as a major cause of morbidity and mortality in this condition. Treatment is all the more efficient as pulmonary hypertension is early diagnosed. A good knowledge of the clinical, biological and functional features of pulmonary hypertension in systemic sclerosis is therefore necessary to suspect and to diagnose pulmonary hypertension as early as possible. METHODS: Sixty seven patients with systemic sclerosis were retrospectively studied. We compared clinical, immunological, functional (spirometry) and morphological (pulmonary fibrosis) features according to the presence (n = 25) and the characteristic of pulmonary hypertension (isolated or secondary) or the absence (n = 42) of pulmonary hypertension, assessed by Doppler echocardiography. RESULTS: CREST syndrome (calcinosis, Raynaud's phenomenon, oesophageal involvement, sclerodactyly and telangiectasia) was more frequent in patients with isolated pulmonary hypertension than in patients without PH (72.7% vs 28.5%, P < 0.05; odds-ratio [OR] = 6.6) and dyspnea was more severe (P < 0.001; OR = 11.4). The age at time of pulmonary hypertension diagnosis was higher in patients with secondary pulmonary hypertension than in patients with isolated from (median: 62.5 years (range: 32-35) vs 53 years (range: 37-85), P < 0.05). Patients with isolated pulmonary hypertension had anticardiolipin antibodies more frequently than patients without pulmonary hypertension (72.7% vs 35.7%, P < 0.05). Isolated reduction of diffusing capacity was preferentially observed among patients with isolated pulmonary hypertension than among those without pulmonary hypertension. A linear relation between systolic pulmonary artery pressure values and diffusing capacity values (r = 0.72, P < 0.01) was found. Isolated reduction of diffusing capacity was more frequent in patients with isolated pulmonary hypertension than in patients without pulmonary hypertension (63.6% vs 14.3%, P < 0.001; OR = 10.5). CONCLUSION: The severity of pulmonary hypertension in systemic sclerosis justifies a systematic screening by Doppler echocardiography and diffusing capacity measurement. Our results allow us to better define the characteristics of sclerodermic patients with isolated or secondary pulmonary hypertension. The search for pulmonary hypertension should be repeated with time and clinicians should be particularly vigilant in the case of a patient presenting these characteristics.

Adult↗
PubMed1998

[Iloprost administration over 21 days as an effective therapy in systemic scleroderma--case report and review of the literature].

A 57-years old female patient with systemic sclerosis underwent a prolonged intravenous therapy during 21 consecutive days with iloprost, a stable analogue of prostacyclin. Beginning with 0.5 ng/ kg/minute the dose was increased every 2 days up to 2 ng/kg/minute. At the end of follow up, Iloprost was shown to enhance the perfusion of the finger and to improve pulmonary-function tests including the diffusion-capacity. Furthermore, the Erythrocyte Sedimentation Rate and the C-reactive protein decreased. The case report shows the necessity of a controlled study of prolonged iloprost therapy.

Clinical Trials as Topic↗