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At least 1,171 records · Page 65Linked to original sources
PubMed2000

Macroangiopathy of the upper extremities in progressive systemic sclerosis.

BACKGROUND: Raynaud's phenomenon is a hallmark of progressive systemic sclerosis (SSc). While nailfold capillary changes are well known, morphological changes in larger arteries have less been focused on. OBJECTIVE AND METHODS: In the study presented we examined how often in digital subtraction angiography organic changes of the arteries of the fingers, hands and forearms were observed, whether they showed a typical picture like the changes in nailfold capillaries and whether they correlated with cutaneous sclerosis, Raynaud's phenomenon and serological findings. 29 patients were examined, 14 with acroscleroderma and 15 with proximal ascending sclerosis. RESULTS: In 27 of those 29 patients a stenosis of the arteries of the upper extremity was found. The frequency of arterial occlusions decreased from the fingers (26 patients) to the forearms (9 patients with occlusion of the ulnar artery, none with occlusion of the radial artery). The arteries of 47% of patients with proximal ascending sclerosis showed no reactivity towards the alpha-sympatholyticum tolazoline hydrochloride due to severe organic changes while in patients with acroscleroderma only 14% of patients did not respond to tolazoline hydrochloride. The severity of Raynaud's phenomenon did not correlate with the severity of the angiographic findings. In patients with stenoses refractory to tolazoline hydrochloride and in those with occlusions Scl-70 autoantibodies were more frequently positive than in other patients with SSc (44% compared to 23%). CONCLUSION: As in SSc the severity of the organic arterial changes is in close correlation with the extent of the cutaneous sclerosis and with the serological findings the arterial system should gain much more importance in the diagnosis and therapy of SSc.

Aged↗
PubMed2000

[An autopsy case of systemic sclerosis with small cell carcinoma of the lung].

We report an autopsy of a 69-year-old woman who had systemic sclerosis with small cell carcinoma of the lung. She had interstitial pneumonia associated with systemic sclerosis and was admitted to our hospital because of acute on chronic respiratory failure due to a respiratory tract infection. Although she was treated with antibiotics, artificial respiration, and so on, she died 54 days after admission of exacerbation of respiratory failure. Histopathological examination at autopsy revealed intermediate cell type small cell carcinoma of the lung and usual interstitial pneumonia. It is generally said that the most common type of lung cancer associated with systemic sclerosis is adenocarcinoma or alveolar cell carcinoma, and that small cell carcinoma is rare. No autopsy case of systemic sclerosis with small cell carcinoma of the lung has ever been reported in Japan. Small cell carcinoma of the lung is more responsive to chemotherapy and radiotherapy than other histological types of lung cancer. Patients with small cell lung cancer are generally treated with chemotherapy alone or a combination of chemotherapy and radiotherapy. It is important to remember that the lung cancers that may be complicated with systemic sclerosis include not only adenocarcinoma and alveolar cell carcinoma but also small cell carcinoma of the lung, because the histological type may dictate the treatment.

Aged↗
PubMedDate not supplied

Progressive systemic sclerosis (scleroderma), carcinoma breast and valvular heart disease: an unusual combination.

Systemic sclerosis is a multi system disorder characterised by fibrosis of skin and internal organs. There are reports of relation between cancer and polymyositis/dermatomyositis, but no overall association with systemic sclerosis. Reports of the coexistence of cancer and systemic sclerosis, however, emphasise a close temporal relation in their occurrence. Cardiac involvement in the form of myocardial fibrosis and pericarditis occurs frequently in systemic sclerosis, while valvular involvement has been reported only sporadically. We report a patient, admitted for adenocarcinoma of left breast who was found to have features of systemic sclerosis, pulmonary hypertension, gangrene of toes, and stenotic mitral valve disease. The possible mechanisms of the coincidence of the three disorders are discussed.

Breast Neoplasms↗
PubMedDate not supplied

[Systemic sclerosis (scleroderma) without scleroderma].

There are two approaches toward dealing with systemic sclerosis (scleroderma) presenting with no skin involvement. According to one of these the condition is to be treated as atypical systemic sclerosis (scleroderma). With the other approach it does not make much difference whether systemic sclerosis presents with skin involvement or not. In the study made a group of systemic sclerosis patients presenting with no skin involvement was examined vs patients with circumscribed skin involvement. No evidence has been found for systemic sclerosis (scleroderma) sine scleroderma to be regarded as running an atypical course.

Adolescent↗
PubMed2002

[Treatment of bladder neck obstruction (sclerosis): personal experience and literature review].

The goal of this article is a description of bladder neck obstruction (sclerosis) treatment results at the Department of Urology at Kaunas University of Medicine Hospital and the review of current literature on this subject. Sixty seven patients treated for bladder neck obstruction (sclerosis) in the period of 1995-2000 years were analyzed retrospectively. On admission to the hospital, 31 patient had undergone open prostatectomy for benign prostatic hyperplasy, 14 had undergone thransurethral prostate resection and 22 had no previous prostatic surgery. The usual diagnostic procedure for bladder neck obstruction (sclerosis) in Kaunas University of Medicine Hospital is retrograde uretherocystography. According to current literature the videourodynamics is the most effective diagnostic procedure, however, still not available at Kaunas University of Medicine Hospital. The transurethral bladder neck incision or/and resection is effectively used for bladder neck obstruction (sclerosis) treatment at Kaunas University of Medicine Hospital. The near postoperative results are good for 73.4% of patients. This method is the most widely used for bladder neck obstruction (sclerosis) treatment around the world.

Adult↗
PubMed1998

Amyotrophic lateral sclerosis and glutamate.

Amyotrophic lateral sclerosis is a progressive fatal disorder devastating the spinal cord and brain in humans. Excitotoxicity has been suggested to be involved in the pathogenesis of amyotrophic lateral sclerosis. This hypothesis has driven a wealth of basic research and stimulated development of neuroprotective therapies for chronic neurodegenerative disorders. As a result of these efforts, riluzole, an antiglutamatergic drug, has been established in the therapy of amyotrophic lateral sclerosis. A transgenic mouse showing features of amyotrophic lateral sclerosis has been subsequently engineered enabling studies of the disease in vivo. However, despite considerable progress, the etiology of amyotrophic lateral sclerosis remains obscure and the disturbances in excitatory neurotransmission should by no means be regarded as exclusive to the pathogenesis of the disease.

Journal Article↗
PubMed1992

Genetic and environmental factors in systemic sclerosis.

The occurrence of autoantibodies in patients with systemic sclerosis has suggested a role for immune dysregulation in this disease. Recent genetic studies have concentrated on the major histocompatibility complex-encoded antigens and found an association of particular HLA-DQ alleles with anticentromere antibodies. Although the role of major histocompatibility complex antigens and autoantibodies in the pathogenesis of systemic sclerosis remains unclear, determination of major histocompatibility complex alleles may have clinical value in identifying patients who are at increased risk for development of pulmonary fibrosis or rapidly advancing skin disease. A variety of environmental factors have been associated with systemic sclerosis-like skin diseases, including silica, vinyl chloride, paraffin, adulterated L-tryptophan, and "toxic" rapeseed oil. It has been suggested that silicone used during breast augmentation may be a risk factor for development of systemic sclerosis, but ascertainment bias in case reporting makes interpretation of these studies difficult. The heterogeneity of clinical features, major histocompatibility complex status, and autoantibody profiles in systemic sclerosis suggest that this disorder may actually be a group of distinct disorders, each of which has its own characteristic genetic and environmental predisposing risk factors.

Environment↗
PubMed1992

Treatment of systemic sclerosis with extracorporeal photochemotherapy. Results of a multicenter trial.

BACKGROUND AND DESIGN: In a pilot study of extracorporeal photochemotherapy, two patients with systemic sclerosis who received this therapy experienced significant clinical improvement. These results prompted the development of a multicenter trial to examine the benefit of extracorporeal photochemotherapy in the treatment of systemic sclerosis. Seventy-nine patients with systemic sclerosis of recent onset (mean symptom duration, 1.83 years) and progressive skin involvement during the preceding 6 months entered a randomized, parallel-group, single-blinded clinical trial comparing extracorporeal photochemotherapy treatments given on 2 consecutive days monthly with treatment with D-penicillamine at a maximum dose of 750 mg/d. Blinded clinical examiners evaluated skin severity score (thickness), percent surface area involvement, oral aperture, and hand closure. Serial skin biopsies and pulmonary function studies were also performed. RESULTS: Following 6 months of treatment, significant improvement in skin severity score occurred in 21 (68%) of 31 patients receiving photochemotherapy and in eight (32%) of 25 receiving D-penicillamine treatment, while significant worsening occurred in three (10%) of 31 receiving photochemotherapy and in eight (32%) of 25 receiving penicillamine treatment, thus indicating a significantly higher response rate for individuals who received photochemotherapy (P = .02). At both the 6- and 10-month evaluation points, the mean skin severity score, mean percent skin involvement, and mean oral aperture measurements were significantly improved from baseline among those who received photochemotherapy. Mean right and left hand closure measurements had also improved significantly by 10 months of therapy. By comparison, among the patients treated with D-penicillamine, none of the parameters of cutaneous disease had improved significantly after 6 months of therapy, although for those individuals in whom treatment was continued, the mean skin severity score and mean percent skin involvement had improved by 10 months. Skin biopsy studies revealed a correlation between clinical improvement and decreased thickness of the dermal layer. Adverse effects of extracorporeal photochemotherapy were minimal and did not require discontinuation of treatment in any of the patients receiving this therapy; six patients permanently discontinued the use of D-penicillamine treatment due to adverse effects. CONCLUSIONS: For patients with systemic sclerosis of recent onset, extracorporeal photochemotherapy is a well-tolerated treatment that may partially reverse the process that results in cutaneous sclerosis.

Adult↗
PubMedDate not supplied

The anti-platelet effect of nifedipine in patients with systemic sclerosis.

The enhanced platelet reactivity and impaired thrombolysis in patients with systemic sclerosis may contribute to the microvascular insufficiency seen in this disease. Anti-platelet therapy has therefore been suggested for the treatment of Raynaud's phenomenon secondary to systemic sclerosis. Using a novel technique, the Haemostatometer, haemostasis (shear-induced) and thrombolysis (dislodgement of the haemostatic plug) were assessed initially in 15 patients with systemic sclerosis before and 90 minutes after a single oral dose of nifedipine (10 mg), and then at 4-week intervals for 16 weeks in 10 patients on long-term nifedipine (10-20 mg tid). Ninety minutes after a single oral dose of nifedipine in 15 patients with systemic sclerosis, haemostasis was significantly prolonged from 140 +/- 12 sec to 178 +/- 21 sec (mean +/- SE, p less than 0.005). The time until spontaneous thrombolysis occurred was significantly shortened following nifedipine, from an abnormal time of 57.8 +/- 2.8 min to a more normal value of 34.0 +/- 5.1 min (mean +/- SE, p less than 0.01). This improvement in haemostasis and thrombolysis was maintained through 16 weeks on longterm nifedipine treatment. These findings suggest that nifedipine may reduce the risk of developing multiple thrombo-emboli in patients with systemic sclerosis.

Administration, Oral↗
PubMed1992

Capillary abnormalities, Raynaud's phenomenon, and systemic sclerosis in patients with localized scleroderma.

OBJECTIVES AND DESIGN: In vivo capillaroscopic examination was performed on patients with localized scleroderma to determine whether nailfold capillary abnormalities seen in systemic scleroderma (systemic sclerosis) were also present in the localized form. Twenty-seven patients (24 women, three men) were examined by this technique. RESULTS: Only two patients exhibited scleroderma-type nailfold capillary abnormalities similar to those seen in systemic sclerosis. Both patients also suffered from Raynaud's phenomenon and showed evidence of coexisting systemic sclerosis, one on first examination, the other 1.5 years later. Our results are compared with earlier studies reporting such rare coexistence of the two forms of scleroderma. Earlier capillaroscopic work in this disorder is also reviewed. CONCLUSIONS: These results suggest that the presence, in a patient with localized scleroderma, of nailfold capillary abnormalities similar to those seen in systemic sclerosis should alert the physician to a possible association with systemic sclerosis.

Adolescent↗
PubMed2005

[Capillaroscopy in the diagnosis of systemic sclerosis].

Capillaroscopy is a safe, simple, non-invasive, low-cost analysis of the nailfold circulation. Vascular lesions play a significant role in the pathogenesis of systemic sclerosis. It has been shown that a characteristic microangiopathy, so-called "scleroderma pattern capillary abnormalities", --are present in the majority of patients with systemic sclerosis and already appear in the early stage of the disease. Due to this fact, capillarosopy is considered a practical tool for diagnosing and monitoring patients with systemic sclerosis. Capillaroscopy can help in the selection of patients that need more aggressive therapeutic interventions. Recently, the presence of typical capillaroscopic abnormalities has been suggested as one of the minor criteria for the early diagnosis and classification of systemic sclerosis. The present study presents the role of capillaroscopy in the management of patients with systemic sclerosis.

Diagnosis, Differential↗
PubMed2005

Endothelin receptor antagonism--new perspectives in the treatment of systemic sclerosis.

Endothelin-1 is a naturally occurring polypeptide which possesses a broad range of activities including vasospastic, proinflammatory and profibrotic properties. Systemic sclerosis is a multisystem connective tissue disease characterized by vascular damage, inflammatory infiltrates and progressive fibrosis of the skin and internal organs. The results of the recent studies indicate that endothelin-1 may be a key element of the pathogenesis of systemic sclerosis. Accordingly, new class of drugs, endothelin receptor antagonists have been introduced for treatment of patients with systemic sclerosis. This article reviews the role of endothelin-1 in the pathogenesis of systemic sclerosis and the implications of endothelin receptor antagonism in the treatment of systemic sclerosis.

Endothelin Receptor Antagonists↗
PubMed2005

[An up date on pulmonary hypertension related to systemic sclerosis].

Systemic sclerosis (scleroderma) is a chronic, progressive connective tissue disease characterized by typical skin changes and internal organ involvement. Arterial pulmonary hypertension is found in approximately 10-15% of patients with systemic sclerosis, and is considered as one of the most fatal complications of the disease. When pulmonary hypertension secondary to scleroderma related interstitial lung disease, left-sided cardiac insufficiency and thrombo-embolic disease is also taken into account, the frequency of pulmonary hypertension in systemic sclerosis may be as high as.50%. Recent investigations brought new information considering the pathogenesis of pulmoanry hypertension and allowed new therapeutic approches to be introduced. Early diagnosis of pulmonary hypertension is a key issue in clinical practice, but it is a challange in patients with systemic disease such as systemic sclerosis. Current review presents up to date information on the pathogenesis, early diagnosis and treatment of pulmonary hypertension related to systemic sclerosis.

Humans↗
PubMedDate not supplied

Lack of association of the PTPN22 gene polymorphism R620W with systemic sclerosis.

OBJECTIVE: It has recently been reported that some autoimmune diseases seem to be associated with a functional polymorphism in PTPN22, a gene which encodes a phosphatase known to be important in T-cell signaling. The aim of our study was to check for the prevalence of the PTPN22 R620W polymorphism in patients with systemic sclerosis. METHODS: DNA samples from 54 systemic sclerosis patients and 55 healthy controls were obtained from peripheral blood and genotyping was performed by means of a restriction fragment length polymorphism analysis of PCR products (RFLP-PCR). RESULTS: Allele frequency for the T allele was slightly higher in the patients group (0.074 versus 0.055). Eight out of the 54 systemic sclerosis patients (14.8 %) were heterozygous for this single nucleotide polymorphism whereas the CT genotype was found in 6 out of the 55 controls (10.9%). Nevertheless, the difference did not reach statistical significance (p = 0.542). Neither certain antibodies linked to systemic sclerosis (anti-centromere and anti-topoisomerase I antibodies) nor any particular clinical involvement were associated with the polymorphism. CONCLUSION: This particular single nucleotide polymorphism of PTPN22 does not seem to be associated with systemic sclerosis.

Antibodies, Antinuclear↗
PubMedDate not supplied

[Ligation and sclerosis technique combined with electrocoagulation resection of internal hemorrhoids].

We review 1331 patients who were treated by sclerosis and rubber band ligation, stepped binding, simultaneous binding and sclerosis with electrocoagulation resection as the main hemorrhoidal bundle treatment, from July 1977 through July 1990, was performed. The benefits on symptoms, complications, and final outcome were analyzed. Binding of only one hemorrhoidal bundle with sclerosis of the other two in the 74.9%, binding of two bundles with sclerosis of the other in the 13.7% and simultaneously binding of the three bundles in the 4.8% was performed. Binding, sclerosis and electrocoagulation resection of three bundles was also performed in the 6.45%.

Adolescent↗
PubMed1991

[Prophylactic sclerosis of esophageal varices. Results of a controlled prospective study].

BACKGROUND: Endoscopic sclerosis of esophageal varices is effective both to treat active hemorrhage and to prevent recurrences. By contrast, its usefulness for the prophylaxis of the first episode of hemorrhage is not well established, the available data being contradictory. METHODS: Overall 46 patients with cirrhosis and esophageal varices of type B or higher, without previous upper gastrointestinal hemorrhage, were randomized to be treated with prophylactic sclerosis or not (n = 22 and 24, respectively). Both groups were comparable in age, sex, etiology of cirrhosis (mainly alcoholic), Child's degree of hepatic failure, coagulation studies, laboratory data and endoscopic findings. RESULTS: In a mean follow up of 16 months, the patients treated with sclerosis had a higher frequency of hemorrhage from gastroesophageal varices (27%) than those in the control group (8%) (n.s.). However, this difference reached significance (p = 0.02) when only patients from Child's classes A and B were considered (31% in the sclerosis group vs 5% in the control group). The mortality rate was similar in both groups, with a probability of one year survival of 80 and 76% in the sclerosis and control groups, respectively. CONCLUSIONS: The results indicate that prophylactic sclerotherapy does not improve short-term survival or reduce the risk of a first hemorrhage due to gastroesophageal varices of type B or higher in hepatic cirrhosis. It may even facilitate the development of this severe complication in Child's A and B class patients.

Endoscopy, Digestive System↗
PubMed1986

Quantification of the nail fold capillary abnormalities in systemic sclerosis and Raynaud's syndrome.

Measurements were made of capillary luminal diameters and capillary numbers in four groups: 19 normal controls 10 patients with 'idiopathic' Raynaud's disease, 12 patients with Raynaud's phenomenon with an underlying connective tissue disease other than systemic sclerosis, and 18 patients with systemic sclerosis. There was a significant reduction of capillary numbers in all three patient groups compared with the normal controls. Both the afferent and efferent luminal diameters were also increased in each patient group. Patients with Raynaud's phenomenon, either on its own or associated with connective tissue disease, gave results intermediate between normal controls and patients with systemic sclerosis. There were no significant correlations between either the reduction in capillary numbers or increase in luminal diameter with disease severity or duration in systemic sclerosis. It is unlikely that capillary microscopy will provide useful prognostic information for an individual patient with systemic sclerosis; its predictive value in Raynaud's disease must await the outcome of long term follow-up studies.

Adult↗
PubMed1985

Subcutaneous sclerosis with fasciitis and eosinophilia after phytonadione injections.

We treated a case of subcutaneous sclerosis after phytonadione injections (Texier's disease) that was noteworthy for two associated features: sclerosis with inflammatory infiltrate in the fascia, and blood eosinophilia. A peculiar type of cutaneous sclerosis after phytonadione injections was described in 1972. The sclerosis in that case was first confined to the buttocks, progressively extended to both thighs, and slowly resolved. In the 38 reported cases, neither sclerosis of the fascia nor blood eosinophilia was observed.

Drug Eruptions↗