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Results for “Scleroderma, Diffuse”

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At least 631 records · Page 35Linked to original sources
PubMed2005

Prokinetic therapy: what can be measured by gastric scintigraphy?

OBJECTIVE: To evaluate whether gastric scintigraphy with quantitative analysis of gastric peristalsis may be a useful tool for documenting the effects of prokinetic therapy. METHODS: Gastric emptying was determined in eight patients with insulin dependent diabetes mellitus (IDDM) and nine patients with progressive systemic sclerosis (PSS) after ingestion of a semi-solid test meal. Fourier analysis of condensed images was used to evaluate contraction amplitudes of the entire stomach, as well as frequency and velocity of gastric contractions. Patients were studied twice, first under baseline conditions and then after 4 weeks of continuous oral prokinetic therapy with cisapride (10 mg t.i.d.). Findings were compared with data derived from 15 healthy controls. RESULTS: At baseline conditions IDDM and PSS patients exhibited reduced gastric peristalsis and delayed emptying compared to controls. After 4 weeks of prokinetic treatment, gastric contraction amplitudes increased significantly, especially in the gastric corpus and the proximal stomach, associated with a slight improvement of gastric emptying. Changes in frequency and velocity of gastric contractions during prokinetic treatment correlated with the improvement of gastric motility and emptying. CONCLUSIONS: Prokinetic therapy with cisapride stimulates gastric motility, and could be non-invasively monitored by scintigraphy. This radionuclide technique provides a number of relevant parameters of gastric function, such as gastric emptying, contraction amplitudes, frequency and velocity of gastric contractions, which give crucial insights into the mechanisms of action of prokinetic drugs.

Adult↗
PubMed2006

Esophageal disease in scleroderma.

Progressive systemic sclerosis (PSS) causes smooth muscle atrophy and fibrosis of the distal two-thirds of the esophagus. Motility studies show reduced-amplitude or absent peristaltic contractions in this region and normal or decreased lower esophageal sphincter pressure. Patients complain of dysphagia, heartburn, and regurgitation due to reflux and dysmotility. Complications include strictures found in 17% to 29% of patients and Barrett esophagus is 0% to 37%. Candida esophagitis is a complication of PSS not seen with non-PSS reflux. Esophageal disease correlates with pulmonary involvement but not with disease in the stomach or intestines. Whether reflux contributes to the pulmonary disease is an open question. Although manometry is the gold standard for diagnosis, cine-esophagram and scintography are only slightly less sensitive and should be considered for following the patients. Symptoms correlate poorly with evidence of esophagitis or abnormal 24-hour pH recordings. As a result, it is unclear which patients should receive acid-reducing or prokinetic medications and which medication to use. Aspiration precautions are important in those with severe esophageal dysmotility. This review of the literature highlights many areas of uncertainty in the diagnosis and treatment of esophageal disease in PSS that can be addressed in clinical studies.

Autonomic Nervous System↗
PubMed2005

The relaxin gene knockout mouse: a model of progressive scleroderma.

Relaxin is a peptide hormone with anti-fibrotic properties. To investigate the long-term effects of relaxin deficiency on the ageing skin, we compared structural changes in the skin of ageing relaxin-deficient (RLX-/-) and normal (RLX+/+) mice, by biochemical, histological, and magnetic resonance imaging analyses. Skin biopsies from RLX+/+ and RLX-/- mice were obtained at different ages and analyzed for changes in collagen expression and distribution. We demonstrated an age-related progression of dermal fibrosis and thickening in male and female RLX-/- mice, associated with marked increases in types I and III collagen. The increased collagen was observed primarily in the dermis of RLX-/- mice by 1 mo of age, and eventually superseded the hypodermal layer. Additionally, fibroblasts from the dermis of RLX-/- mice were shown to produce increased collagen in vitro. Recombinant human gene-2 (H2) relaxin treatment of RLX-/- mice resulted in the complete reversal of dermal fibrosis, when applied to the early onset of disease, but was ineffective when applied to more established stages of dermal scarring. These combined findings demonstrate that relaxin provides a means to regulate excessive collagen deposition in disease states characterized by dermal fibrosis and with our previously published work demonstrate the relaxin-null mouse as a model of progressive scleroderma.

Animals↗
PubMed2005

Dementia associated with scleromyxoedema reversed by high-dose intravenous immunoglobulin.

Scleromyxoedema is a rare skin disease, characterized by deposition of acid mucopolysaccharides in the dermis. Although the disease primarily affects the skin, cardiovascular, renal and rheumatological manifestations have been described. In addition to these noncutaneous manifestations, about 15% of patients have central neurological symptoms such as psychosis, convulsions and encephalopathy. Successful therapy is difficult but high-dose intravenous immunoglobulin (IVIg) has been reported to be a successful treatment. We describe a patient with scleromyxoedema who presented with novel central nervous system manifestations of chronic cognitive impairment and dementia (Folstein Mini Mental State test score 8/30), which improved within a week after treatment with high-dose IVIg, with full restoration (Folstein Mini Mental State test score 27/30) at 2 months.

Aged↗
PubMed2005

Multiple myeloma with scleroderma-like changes.

We present a case of a patient who presented concomitantly with generalized pruritus, brownish sclerodermatous plaques, sclerodactyly and a monoclonal band for IgG-kappa. The patient was diagnosed as having multiple myeloma by bone marrow examination. The rapidly progressive evolution with acute anuric renal failure, malignant hypertension and the skin sclerosis seem to be related to the neoplastic disorder. The scleroderma-like changes have to be differentiated from systemic scleroderma.

Abdomen↗
PubMed2006

Stiff skin syndrome.

Stiff skin syndrome is a rare disorder characterized by pronounced skin induration, mild hypertrichosis and limited joint mobility, predominantly on the buttocks and thighs. Many heterogeneous cases have been reported under the name of stiff skin syndrome. We present a case of stiff skin syndrome from China, the diagnosis based on the patient's typical clinical and histopathological features.

Child, Preschool↗
PubMed2005

Angioimmunoblastic T cell lymphoma with an unusual proliferation of Epstein-Barr virus-associated large B cells arising in a patient with progressive systemic sclerosis.

We report an unusual case of angioimmunoblastic T cell lymphoma arising in the setting of 5 years of immunosuppressive treatment for progressive systemic sclerosis. The lymph node lesion was accompanied by large blastic B cells with an association of Epstein-Barr virus. Southern blot study demonstrated the clonal rearrangement of T cell receptor beta-chain gene, but not of immunoglobulin heavy chain gene. Phenotypical examination of the lymph node also revealed the predominance of CD4+ T cells in addition to the proliferation of follicular dendritic cells, but no light chain restriction in large B cell components. In the clinical and laboratory aspects, neutrophilia (15.8 x 10(9)/l) and plasmacytosis (40%) in bone marrow were noted, which were considered to be closely related to elevated serum granulocyte colony-stimulating factor, interleukin (IL)-4 and IL-6. Based on the combined data described here, our preferred diagnosis was angioimmunoblastic T cell lymphoma with Epstein-Barr virus-associated B cell lymphoproliferative disorder, the pathogenesis of which was suggested to be closely associated with immunosuppressive treatment for progressive systemic sclerosis.

Aged↗
PubMed2006

[Does garlic influence rheologic properties and blood flow in progressive systemic sclerosis?].

BACKGROUND: According to traditional European naturopathy garlic is an agent that increases perfusion. In studies with healthy subjects and in-vitro research garlic has shown influences on erythrocyte and thrombocyte aggregation as well as on vasoregulation. However, data on its effects in clinical populations are still lacking. Garlic may be useful for systemic sclerosis which is characterised by impaired perfusion that often cannot sufficiently be influenced by standard treatment. We investigated if dried garlic powder can influence rheologic properties and vasomotor function in systemic sclerosis. SUBJECTS AND METHODS: During a randomised, double blind pilot study, 20 female inpatients with systemic sclerosis received a 7 day add-on therapy with either 900 mg dried garlic powder or placebo. Rheologic properties (erythrocyte aggregation, ADP-induced thromboycyte aggregation, plasma viscosity, fibrinogenous plasma level, blood sedimentation rate) were assessed initially as well as after 1 and 7 days of treatment. Vasomotor function was evaluated using near-infrared red photoplethysmography, a new diagnostic tool to assess microcirculation. Furthermore, acral skin temperature was measured. RESULTS: After 7 days, only the verum treatment had induced a significant reduction of ADP-induced thrombocyte aggregation and a decrease in erythrocyte aggregation. Results showed no significant effects on vasomotor function, but an immediate effect of garlic on acral skin temperature. DISCUSSION: According to the 'Qualitatenlehre' of traditional European naturopathy, garlic is classified as a 'heating agent'. Our results suggest that the improvement of rheologic properties could be a possible biological correlate for this. Although further research is required, we conclude garlic could be a rational add-on therapy in the 'Kaltekrankheit' ('cold disease') of systemic sclerosis.

Blood Viscosity↗
PubMed2005

Local hyperemia to heating is impaired in secondary Raynaud's phenomenon.

Accurate and sensitive measurement techniques are a key issue in the quantification of the microvascular and endothelial dysfunction in systemic sclerosis (SSc). Thermal hyperemia comprises two separate mechanisms: an initial peak that is axon reflex mediated; and a sustained plateau phase that is nitric oxide dependent. The main objective of our study was to test whether thermal hyperemia in patients with SSc differed from that in patients with primary Raynaud's phenomenon (RP) and healthy controls. In a first study, we enrolled 20 patients suffering from SSc, 20 patients with primary RP and 20 healthy volunteers. All subjects were in a fasting state. Post-occlusive hyperemia, 0.4 mg sublingual nitroglycerin challenge and thermal hyperemia were performed using laser Doppler flowmetry on the distal pad of the third left finger. In a second study, thermal hyperemia was performed in 10 patients with rheumatoid arthritis and 10 patients with primary RP. The thermal hyperemia was dramatically altered in terms of amplitude and kinetics in patients with SSc. Whereas 19 healthy volunteers and 18 patients with primary RP exhibited the classic response, including an initial peak within the first 10 minutes followed by a nadir and a second peak, this occurred only in four of the SSc patients (p < 0.0001). The 10 minutes thermal peak was 43.4 (23.2 to 63), 42.6 (31 to 80.7) and 27 (14.7 to 51.4) mV/mm Hg in the healthy volunteers, primary RP and SSc groups, respectively (p = 0.01), while the 44 degrees C thermal peak was 43.1 (21.3 to 62.1), 42.6 (31.6 to 74.3) and 25.4 (15 to 52.4) mV/mm Hg, respectively (p = 0.01). Thermal hyperemia was more sensitive and specific than post-occlusive hyperhemia for differentiating SSc from primary RP. In patients with rheumatoid arthritis, thermal hyperemia was also altered in terms of amplitude. Thermal hyperemia is dramatically altered in patients with secondary RP in comparison with subjects with primary RP. Further studies are required to determine the mechanisms of this altered response, and whether it may provide additional information in a clinical setting.

Adult↗
PubMed2006

Necrotizing fasciitis in rheumatic diseases.

Necrotizing fasciitis is an uncommon but life-threatening complication in immunocompromised hosts. We reported four patients with rheumatic diseases complicated by necrotizing fasciitis and reviewed 14 others from literature search. Most patients were on corticosteroid treatment. Septic shock, disseminated intravascular coagulopathy and acute renal deterioration were common giving rise to an overall mortality rate of 27.8%. Septic arthritis may also complicate the condition. Statistical analysis on the series showed the lack of major surgical debridement as the only risk factor associated with increased mortality (RR 7.5, 95% CI 2.1-27.3, P = 0.01). Timely debridement of necrotic tissue is important for reducing mortality.

Adult↗
PubMed2005

Mandibular resorption in progressive systemic sclerosis: a report of three cases.

Progressive systemic sclerosis is a generalized collagen disorder, which is characterized by fibrosis that involves skin, muscles and other organ systems like the gastrointestinal tract, lungs, heart and kidneys. Its oral manifestations include features like restricted mouth opening, widening of periodontal ligament space, pseudoankylosis, malocclusion and mandibular resorption. Mandibular resorption in systemic sclerosis is relatively uncommon and is reported only in 10% of cases. The purpose of reporting these three cases is to highlight the importance of screening all patients with advanced systemic sclerosis with panoramic radiographs. Panoramic radiographs are essential for early detection of resorption in the mandible to prevent possible consequences like pathological fractures, osteomyelitis and neuropathies.

Adult↗
PubMed1996

Effect of aging on the esophageal motor functions.

To clarify the changes of esophageal motility along with age, we performed esophageal manometry on 47 healthy volunteers, and compared the values of four groups under 49 years old (n = 11), 50 to 59 (n = 15), 60 to 69 (n = 11), and over 70 years old (n = 10). Resting lower esophageal sphincter (LES) pressure in the group over 70 years old showed the tendency of decrease, but not statistically significant. Nadir LES pressure on swallow-induced relaxation was not statistically different among 4 groups. On esophageal body testing, percentage of non-conduction sequence in the group 60 to 69 and over 70 years old was statistically higher compared with that of the group under 49 years old (p < 0.05) but spared in some elderly subjects. Percentage of simultaneous contractions was not statistically different among 4 groups. Peristaltic contraction amplitude in the group over 70 years old was significantly lower than that of the group under 49 years old both at the level of 5 cm above (p < 0.01) and 10 cm above LES (p < 0.05). We speculate that the influence of aging on esophageal motility is the reduced transmission sequence of peristalsis and contractility of esophageal body. This alteration along with age may differ from the pathological condition of scleroderma or diffuse esophageal spasm.

Adult↗
PubMed2004

Pleural involvement in systemic disorders.

The collagen vascular diseases are a heterogeneous group of immunologically-mediated inflammatory disorders. Frequently, these diseases affect organ systems outside the thorax as their primary manifestation, but may involve the pleura as a single presenting feature, as part of multisystem involvement, or as an isolated manifestation of a disease that is otherwise quiescent. In this article, we review the manifestations of respiratory disease caused by rheumatoid arthritis, systemic lupus erythematosus, scleroderma, polymyositis/dermatomyositis, mixed connective tissue disease, Sjogren's syndrome, Wegener's granulomatosis, and sarcoidosis.

Arthritis, Rheumatoid↗