Watermelon stomach and systemic sclerosis: localization of digestive system involvement?
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Gastrointestinal Diseases↗
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[Studies on the results of anti-toxoplasma enzyme-linked immunosorbent assay and false positive reactions in patients with progressive systemic sclerosis, rheumatoid arthritis and systemic lupus erythematosus].
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Adult↗
[Systemic sclerosis and the gastrointestinal system].
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Diagnosis, Differential↗
Acute myocardial infarction in systemic sclerosis patients: a case series.
To characterize the clinical manifestations of patients with systemic sclerosis who develop a myocardial infarction (MI), a retrospective review of the medical records of all patients who were admitted to our institution between 1982 and 2002 and had the dual diagnosis of systemic sclerosis and an acute MI was done. From 1,009 systemic sclerosis hospital admissions, 11 (1.09%) were for an acute MI. Three of these patients had normal coronaries, and instead of wall motion abnormalities, left ventricular hypertrophy was the predominant finding of an echocardiography. The odds ratio of finding normal coronaries in systemic sclerosis vs the general population who develops an acute MI is 33.89 (14.08-81.39). Seven of our patients had an elevated creatinine level on presentation. Acute MI is an uncommon manifestation in systemic sclerosis patients. Normal coronaries are seen more commonly in these patients as compared to the general population, while vascular, gastrointestinal, and renal involvement is prevalent in these patients.
Coronary Artery Disease↗
Helicobacter pylori in patients with systemic sclerosis: detection with the 13C-urea breath test and eradication.
In patients with systemic sclerosis peristaltic abnormalities may delay gastric emptying, giving rise to bacterial overgrowth, including possibly Helicobacter pylori (HP). Infection with Helicobacter is an important risk factor for esophageal and gastric diseases, including esophagitis, gastritis and gastric cancer. The purpose of this prospective study was to assess gastric HP infection in patients with systemic sclerosis. In 12 patients with systemic sclerosis the newly introduced breath test with 13C-labelled urea was used for indirect detection of gastric urease activity due to HP infection. Five out of 12 patients gave Helicobacter-positive results (42%); 7 patients were negative for Helicobacter colonization (58%). Thus, the risk for gastric diseases caused by HP infection is enhanced in patients with systemic sclerosis compared with white healthy, asymptomatic persons examined in other studies. Helicobacter-positive patients were treated with 2 x 20 mg omeprazole and 4 x 500 mg amoxicillin over 14 days. Afterwards the 13C-urea breath test was repeated and showed negative results for Helicobacter in all systemic sclerosis patients treated. Dual therapy with omeprazole and amoxicillin therapy effectively eradicated HP. The 13C-urea breath test did not cause any side-effects and is therefore considered to be a non-invasive, non-toxic and safe method for the diagnosis and therapeutic control of Helicobacter-status.
Adult↗
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↗
Giant cell arteritis associated with progressive systemic sclerosis.
The clinical and autopsy findings in a patient with both systemic sclerosis (scleroderma) and giant cell arteritis are presented. Giant cell arteritis and systemic sclerosis are autoimmune diseases affecting the elderly that may be associated with similar clinical symptoms of facial pain and arthralgias. Their coexistence is virtually unknown in the medical literature but is of great clinical importance. Systemic sclerosis is not treated with the steroid dosage necessary to prevent the morbid results of giant cell arteritis. An elevated sedimentation rate and/or an abnormal temporal artery examination in an elderly patient with systemic sclerosis should alert the clinician to the possible coexistence of giant cell arteritis.
Aged↗
Immunohistochemical findings in the renal vascular lesions of progressive systemic sclerosis.
Four selected patients with progressive systemic sclerosis showed deposition of immunoglobulins and complement in diseased renal arteries and arterioles. Although three patients had hypertension, in two of these malignant, the third patient did not have hypertension over a four year period. These findings suggest that immune complexes may be involved in the pathogenesis of some cases of progressive systemic sclerosis, the primary target being the vascular system.
Adult↗
Do patients with systemic sclerosis have abnormal gallbladder function?
OBJECTIVE: To determine gallbladder motility in patients with systemic sclerosis. DESIGN: Case control study. SETTING: University hospital, out-patient department of rheumatology. PATIENTS: Ten patients with systemic sclerosis according to the criteria of the American Rheumatism Association with documented involvement of the gastrointestinal tract and 10 healthy controls matched for age, sex and body mass index. INTERVENTION: Cephalic vagal cholinergic simulation by modified sham feeding and hormonal stimulation by infusion of cholecystokinin. MEASUREMENTS: Gallbladder volume obtained by ultrasonography and determination of plasma cholecystokinin concentrations. RESULTS: Fasting gallbladder volumes were not significantly different between patients with systemic sclerosis and controls (19.6 +/- 1.9 cm3 and 23.3 +/- 2.9 cm3, respectively, mean plus or minus standard error of the mean). Neither were there significant differences in reduction of gallbladder volume in response to modified sham feeding (35 +/- 4% and 33 +/- 4%, respectively) nor during cholecystokinin infusion (56 +/- 4% and 60 +/- 6%, respectively). The increase in plasma cholecystokinin levels during infusion was not different in the two groups. CONCLUSION: Gallbladder motility in patients with systemic sclerosis is preserved in response to both cholinergic and hormonal stimulation, even when other gastrointestinal motor disturbances are present. These results suggest that patients with systemic sclerosis are not at increased risk for cholelithiasis because of gallbladder dysmotility.
Case-Control Studies↗
[Scintigraphic diagnosis of esophageal involvement in systemic sclerosis (scleroderma)].
Motor function disturbances (MFD) of the esophagus occur in the majority (90%) of patients with systemic sclerosis. We have tested the diagnostic value of esophagus scintigraphy in 20 patients with systemic sclerosis (12 CREST and 8 with diffuse form) and 18 controls (11 normals and 7 with pyrosis and/or regurgitation), in order to establish sensitivity, specificity, effects of body posture, and use of a liquid or solid meal in the evaluation of MFD of the esophagus. The patients drank 4 ml of fruit juice marked with 500 microCi of 99mTc-sulfur-colloid and afterwards 10 g of an equally marked and scrambled egg. Pictures were made with a Gamma-Camera until 180 s. Radioactivity was plotted against time and separately analysed for the upper, middle and distal esophagus. 65% of the systemic sclerosis patients had dysphagia and 70% had abnormal barium meal transit in the esophagus. The scintigrams were altered in all the patients with systemic sclerosis (p less than 0.001). Sensitivity using fluids was 95%, and specificity was 89%; with solid food 100% and 50%, respectively. Tests done in the upright position showed a better clearance of the esophagus in patients with systemic sclerosis and control patients, with loss of sensitivity. The qualitative analysis yielded little benefit to the reported results. The quantitative analysis under use of 2 indices (total transit time and clearance rate) permitted a clear distinction among patients and control individuals with and without upper digestive symptoms in the first 15 s of the test. The esophagus scintigraphy is a simple, quantitative and very sensitive method for the diagnosis of MFD of the esophagus in patients with systemic sclerosis.
Adult↗
Autocrine overexpression of CTGF maintains fibrosis: RDA analysis of fibrosis genes in systemic sclerosis.
We have used representational difference analysis (RDA) to identify up-regulated genes in skin fibroblasts from fibrotic lesions obtained from patients with systemic sclerosis (scleroderma). RDA of cDNA libraries derived from fibroblasts from involved and uninvolved skin detected several differentially expressed genes. One such gene consistently up-regulated in scleroderma cells coded for human connective tissue growth factor (CTGF). Other studies described here show that the CTGF protein is readily detected in cultures of systemic sclerosis fibroblasts but was not detected in comparable normal cells. High levels of CTGF are also evident in biological fluids from patients with systemic sclerosis. TGFbeta stimulates CTGF production in both normal and systemic sclerosis fibroblasts with the latter found to be higher producers. Moreover, an analysis of constitutive and TGFbeta-induced CTGF gene activation showed altered and elevated transcriptional responses in systemic sclerosis cells compared with controls. CTGF stimulated a two- to threefold increase in proalpha1(I) collagen and fibronectin synthesis by both dermal and lung fibroblasts in culture and promoted significant matrix remodeling of fibroblast-populated three-dimensional collagen lattices. A direct relation between the overexpression of CTGF and elevated collagen synthesis was suggested by the observation that transfection of a CMV-CTGF cDNA construct and protein expression in fibroblasts increased the transcription of a Col 1alpha2 promoter-reporter construct to levels seen in systemic sclerosis fibroblasts. Using Col 1alpha2 promoter deletion constructs the CTGF responsive element was localized to the first 379 bp upstream of the transcriptional start site. These data indicate that there is an overexpression of CTGF in the systemic sclerosis cells, probably due to increased gene transcription, and suggest that the dysregulation of CTGF production is an important factor in fibroblast activation and the excessive deposition of collagen in systemic sclerosis.
3T3 Cells↗
Orofacial radiological manifestations of systemic sclerosis.
OBJECTIVE: To ascertain the orofacial radiological manifestations of systemic sclerosis. METHODS: Twenty-one patients with known systemic sclerosis underwent panoramic, and where appropriate periapical, radiography parotid sialography and a labial gland biopsy. RESULTS: The prevalence of periodontal ligament space widening was 33%, bone resorption 9.5% and abnormal parotid gland sialographic findings 43%. Root resorption affected 4 (33%) of the 12 dentate patients. CONCLUSIONS: Orofacial radiological manifestations occur commonly in systemic sclerosis. Root resorption has not previously been reported.
Adult↗
Bronchial hyperreactivity in systemic sclerosis patients: influence of associated Sjögren's syndrome.
OBJECTIVE: To determine the frequency and relative risk of bronchial hyperreactivity to methacholine in systemic sclerosis patients with or without associated Sjögren's syndrome. METHODS: A prospective study of 56 patients with systemic sclerosis (42 with the diffuse and 14 with the limited variant; 24 with associated Sjögren's syndrome), 57 with primary Sjögren's syndrome, and 61 healthy controls. RESULTS: Bronchial hyperreactivity (BH) was present in 6.5% of the healthy controls, 25% of the systemic sclerosis patients without associated Sjögren's syndrome, 42.2% of those with primary Sjögren's syndrome, and in 50% of those with systemic sclerosis with associated Sjögren's syndrome. The presence of BH did not correlate with age, disease duration, chest radiograph abnormalities, respiratory, and immunological data. The subgroup of subjects with the limited variant of systemic sclerosis more frequently had associated BH than did those with the diffuse variant of the disease; coexisting Sjögren's syndrome further increased this frequency. CONCLUSIONS: In agreement with previous studies, we have confirmed the high prevalence of bronchial hyperreactivity in primary Sjögren's syndrome; systemic sclerosis likewise appears to be associated with an increased frequency of bronchial hyperreactivity compared with healthy control subjects. There is evidence also that the coexistence of Sjögren's syndrome and systemic sclerosis further increases the frequency and the calculated relative risk of developing bronchial hyperreactivity.
Bronchi↗
In vitro cytokine modulation of intercellular adhesion molecule-1 expression on systemic sclerosis dermal fibroblasts.
Our objective was to study the regulation of intercellular adhesion molecule-1 (ICAM-1) expression by cytokines on cultured fibroblasts obtained from systemic sclerosis and normal skin. ICAM-1 expression on dermal fibroblasts obtained from diffuse systemic sclerosis patients with early disease (< or = 2 years) and normal dermal fibroblasts incubated with and without cytokines was measured by radioimmunoassay and flow cytometry. Systemic sclerosis dermal fibroblasts expressed lower basal levels of ICAM-1 than did normal dermal fibroblasts. Both the normal and systemic sclerosis dermal fibroblasts increased their cell surface expression of ICAM-1 in response to interleukin-1 beta (IL-1 beta), tumor necrosis factor-alpha (TNF-alpha), and interferon-gamma (IFN-gamma) in a dose-dependent fashion. Systemic sclerosis dermal fibroblasts appeared to be hyperresponsive to IL-1 beta, TNF-alpha, and IFN-gamma. ICAM-1 expression in response to cytokine stimulation increased to a greater degree on systemic sclerosis compared to normal dermal fibroblasts. The enhanced ICAM-1 expression may play a role in the retention of leukocytes involved in systemic sclerosis skin lesions.
Adult↗
[Cerebral vascular pathology in systemic sclerosis].
Thirty patients with systemic sclerosis (SS), aged 20-55 years, illness duration 1-10 years, were observed. Vasospastic syndrome of different expression predominated in the clinical picture of the disease. Among neurological signs, prevalent were peripheral nervous system lesion--isolated (20% of the cases) or in combination with chronic insufficiency of brain circulation (80%). Vascular pathology was distinctly determined by ophthalmoscopy: angiopathy was found in 42% of the patients, angiospasm--in 25%, angiosclerosis--in 17%. The following types of disorders characterized brain blood flow: distonic (42%), dyscirculatory (33%), normotonic (17%), hypotonic (8%). Besides, 83% of the patients had hemispheric asymmetry of blood flow and difficulties with venous outflow. In some cases, the signs of inner, external and combined hydrocephalia were determined by CT and MRI; more than a half of the patients had dilatation or deformation of brain ventricular system with intracranial hypertension. The data obtained suggest that extra- and intracranial blood flow pathology in SS may, to a large extent, determine both organic brain lesion development in general and the genesis of many symptoms (headaches, vestibular disorders, etc).
Adult↗
Systemic sclerosis after augmentation mammoplasty with silicone implants.
We describe four women who presented with systemic sclerosis several years after cosmetic augmentation mammoplasty with silicone-gel implants. The interval between implantation mammoplasty and the onset of systemic sclerosis ranged from 6 to 15 years. All patients fulfilled the criteria established by the American Rheumatism Association for systemic sclerosis and had Raynaud phenomenon, arthralgia, and evidence of pulmonary or gastrointestinal involvement. Enlargement of lymph nodes draining the prostheses was noted in two patients. Antinuclear antibodies were detected in three patients and showed speckled or nucleolar patterns. Removal of the prostheses in two cases did not result in improvement of systemic sclerosis. Evidence of silicone leakage from the implants included the following. The observation by light microscopy of refractile particles in tissues distant from the prostheses, the observation by electron microscopy of electron-dense structures consistent with silica, and the definitive identification of the element silicon by energy-dispersive analysis in these electron-dense structures. A marked, chronic inflammatory infiltrate containing lymphocytes, "foamy" histiocytes, and larger numbers of multi-nucleated giant cells with vacuoles and asteroid bodies was found at the same sites. Our demonstration that silicone escapes from elastomer-silicone-gel breast implants and appears to be closely associated with a chronic inflammatory reaction suggests that silicone plays a role in the development of certain cases of systemic sclerosis.
Adult↗
Anti-RNA polymerases and other autoantibody specificities in systemic sclerosis.
Sera from 735 patients with systemic sclerosis were classified according to antinuclear antibody (ANA) pattern as follows: centromere (25%), homogeneous (26%), fine speckled (21%), fine speckled with nucleolar (14%), coarse speckled (7%), nucleolar only (3%) and cytoplasmic only (3%). Immunoprecipitations using 35S-labelled HeLa cell antigen extract were performed using sera from 374 of these patients to detect the systemic sclerosis-specific antibodies to RNA polymerases I and III. The sera were selected to represent each ANA group, but focused on those giving fine speckled nucleoplasmic staining (with or without nucleolar staining) where all 86 sera positive for these antibodies were concentrated. Immunoprecipitates from a further 93 sera from patients with ANA-positive autoimmune diseases other than systemic sclerosis did not precipitate RNA polymerases. In addition, all sera were tested for antibodies to the extractable nuclear antigens topoisomerase I, nRNP, Ro, La and PM-Scl. Sera positive for antibodies to these antigens gave clear correlations with ANA patterns but, of the examples tested, none contained antibodies precipitating RNA polymerase I or III. Thus, sera containing antibodies to RNA polymerases I and III were exclusive of both anticentromere and anti-topoisomerase I, and formed a major serological subgroup (11.7%). Clinically, 77% were patients with diffuse cutaneous disease reflected by higher skin scores and a significantly higher incidence of renal involvement (33%) than patients with antibodies to topoisomerase I (3%).
Adult↗
Cold-induced coronary Raynaud's phenomenon in patients with systemic sclerosis.
OBJECTIVE: Cardiac involvement with myocardial-band necrosis is common in systemic sclerosis. One possible explanation is that an underlying vasomotor abnormality accounts for these histologic findings. To shed light on this issue we investigated the existence of "myocardial Raynaud's phenomenon" in such patients. METHODS: We examined 25 patients with systemic sclerosis and 14 patients with systemic lupus erythematosus or rheumatoid arthritis, using cold pressor and dipyridamole-thallium-201 scintigraphy. RESULTS: Twenty-three patients with systemic sclerosis and 13 patients with lupus erythematosus or rheumatoid arthritis had normal perfusion during dipyridamole imaging. Seven scleroderma patients with normal dipyridamole test presented cold-induced transient myocardial ischemia, while none of the control patients had cold-induced ischemia (p = 0.034). All patients with cold-induced ischemic defects presented long-standing Raynaud's phenomenon (> 5 years); of the 14 patients with long-standing Raynaud's phenomenon 7 presented ischemic thallium-201 defects; of the remaining 9 patients with Raynaud's phenomenon of short duration (< 5 years) none presented cold-induced ischemia (p = 0.019). CONCLUSION: Patients with systemic sclerosis and long-standing Raynaud's phenomenon, even in the presence of normal myocardial perfusion during pharmacological vasodilation with dipyridamole, may present cold-induced myocardial ischemia, a functional Raynaud's phenomenon of the heart.
Adult↗