Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The aim of this paper was to study the spectrum of juvenile scleroderma (JSSc) seen at a tertiary care referral center in Asia. Retrospective analysis of case records of patients with systemic sclerosis, having age of onset less than 16 years and seen at our hospital from 1988 to 2004, was done. Patients with linear scleroderma and morphea were excluded. There were 23 patients (19 girls, 4 boys) with median age of onset of 12 years (range 5-16 years). The median age at presentation was 17 years (range 10-34 years). The median time from first symptoms to presentation was 4 years (range 0.2-26 years). Among these, 14 had diffuse systemic sclerosis (DSSc), while 9 had limited scleroderma (LSSc). The clinical features seen at presentation in patients were: Raynaud's phenomenon in 19, digital ulcers in 14, loss of finger tip pulp in 12, reflux in 8, dysphagia in 7, arthritis in 8, digital gangrene in 2, and pulmonary artery hypertension in 1. Antinuclear antibody was positive in 15 out of 18 patients tested. Interstitial lung disease was seen in 15 patients, 6 of whom had diffuse disease. The median skin score was 22 (range 7-48) . One patient died of primary pulmonary hypertension within 1 year of onset of symptoms. At a mean follow-up of 34 months, 14 patients were stable or had improvement in skin score or dyspnea on exertion. DSSc and LSSc in childhood have a clinical presentation similar to adult patients, with cardiopulmonary involvement being the major predictor of outcome. The short-term prognosis of JSSc is good.
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PMID:Juvenile onset systemic sclerosis: a single center experience of 23 cases from Asia. 1718 Feb 99

Systemic sclerosis (scleroderma) is a chronic debilitating disease that is caused by the occurrence of fibrotic changes and vascular abnormalities at various levels such as: skin, lungs, kidneys or heart. Lung involvement in scleroderma is represented by scleroderma interstitial lung disease and by pulmonary arterial hypertension, and is one of the leading causes of mortality in this disease. Pulmonary arterial hypertension can be successfully treated with specific therapies such as sildenafil, bosentan or epoprostenol, whereas only cyclophosphamide has been shown to be effective for interstitial lung disease. The discussed study has shown that cyclophosphamide improved lung function, functional status and health-related quality of life, and reduced dyspnea in scleroderma patients. Most of these patients had acute alveolitis although higher incidences of treatment-related leukopenia and neutropenia were also detected.
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PMID:Cyclophosphamide for scleroderma interstitial lung disease. Tashkin DP, Elashoff R, Clements PJ et al.: Cyclophosphamide versus placebo in scleroderma lung disease. N. Engl. J. Med. (2006) 354(25):2655-2666. 1679 Jun 98

Kidney biopsy plays an important role in the diagnosis and management of several renal diseases. There is a general reluctance to perform kidney biopsy in elderly due to fear of complications. There is no prospective head to head trial comparing complications of percutaneous kidney biopsy in elderly versus young. This prospective study was undertaken to know the frequency and type of biopsy related complications in elderly. Biopsy was performed using a spring loaded automatic 16 G biopsy gun. Post-biopsy, patients were confined to bed rest for 24 h. A record of intraprocedural problems and post-procedural complications was kept. A total of 210 native kidney biopsies were done of which 26 were performed in elderly patients (61-78 years). Co-morbid conditions were present in 17 patients, some having more than one, hypertension (11), diabetes mellitus (5), chronic obstructive airway disease (6), interstitial lung disease (2) and coronary artery disease (2). Mean serum creatinine was 5.6 mg/dl (range 0.8-14.1 mg/dl). Pre-biopsy dialysis was given to 10 patients. Adequate tissue for histopathological diagnosis was seen in 24 out of 26 biopsies. In two elderly patients biopsy had to be abandoned though indicated due to inability to hold the breath because of underlying lung and cardiac disease. Clinico-pathologic discorrelation was seen in eight patients. Incidence of gross hematuria was more in elderly than in young (4/26 vs. 7/184 P<0.01). Hematuria subsided within 1-2 days in three, one had persistent hematuria for 1 week. Other complications viz. gross hematuria with need of blood transfusions or hemodynamic compromise (0/26 vs. 4/184), perinephric hematoma (0/26 vs. 1/184) and need of intervention (0/26 vs. 1/184) were not higher in the elderly. We conclude that the standard precautions kidney biopsy in elderly is a safe procedure.
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PMID:Safety of kidney biopsy in elderly: a prospective study. 1731 59

Few randomized controlled trials (RCT) have shown a demonstrable treatment effect in systemic sclerosis (SSc), making it difficult to evaluate outcome measures in this disease indication. Results from recent RCT, including those evaluating cyclophosphamide for SSc interstitial lung disease and endothelin receptor antagonists for pulmonary hypertension, have allowed analysis of certain organ-specific endpoints using the OMERACT filter. An earlier metaanalysis established that skin score, measures of Raynaud's, pulmonary function tests, blood pressure, pain, Health Assessment Questionnaire, and Medical Outcomes Survey Short-Form 36 are validated outcome measures in SSc. At OMERACT 8, data regarding validation of high-resolution computed tomography of the lungs, 6-minute walk test, and patient reported outcomes in SSc were presented. A Delphi exercise to develop consensus regarding a combined set of noninvasive measures for pulmonary arterial hypertension (PAH) is under way. Given the protean nature of this illness and its multiorgan system involvement, a composite responder index may be preferable. Another Delphi exercise is designed to develop consensus regarding a combined SSc response index to be validated in future RCT.
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PMID:Systemic sclerosis - continuing progress in developing clinical measures of response. 1747 86

The lungs are frequently involved in systemic sclerosis ('scleroderma'), a rare, disabling disease of unknown origin, characterised by skin thickening and Raynaud's phenomenon. The pathogenesis of scleroderma is complex, but signs and symptoms of excessive fibrosis, vasculopathy and inflammation are almost universally present. Dyspnoea in scleroderma patients can be due to chest wall tightening from skin thickening, pleural disease, cardiac involvement, myositis of intercostal muscles, or so-called scleroderma lung disease. Scleroderma lung disease encompasses vascular (pulmonary artery hypertension) or interstitial lung disease, or both. A comprehensive work-up is required to delineate the underlying cause of dyspnoea in a scleroderma patient, and to establish the contribution of each component to the symptoms. This should include a 6-minute walk test, pulmonary function testing, high-resolution thoracic CT scanning, ECG, echocardiography and, if pulmonary artery hypertension is suspected, right-heart catheterisation; bronchoalveolar lavage is optional. Lung disease in scleroderma contributes significantly to excess morbidity and early mortality, especially when diffusion capacity drops below 40% and/or forced vital capacity below 50%. However, recent clinical studies have unequivocally demonstrated that scleroderma lung disease is amenable to treatment with new vasodilatory drugs that target specific pathways involved in vasoconstriction, or with cyclophosphamide for interstitial lung disease. Uncontrolled studies have suggested that these therapies also have an impact on survival, but controlled studies with a long follow-up are needed to corroborate this point.
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PMID:Scleroderma lung: pathogenesis, evaluation and current therapy. 1748 44

Pulmonary hypertension frequently complicates interstitial lung disease, where it is associated with a high mortality. Patients with this dual diagnosis often fare worse than those with pulmonary arterial hypertension (PAH) alone and respond poorly to standard PAH therapy, often dying of right ventricular (RV) failure. We hypothesize that nitric oxide synthase (NOS) uncoupling is important in the pathogenesis of interstitial lung disease-associated pulmonary hypertension, and this process can be abrogated by phosphodiesterase type 5 (PDE5) inhibition to improve pulmonary vascular remodeling and right ventricular function. Intratracheal bleomycin (4 U/kg) or saline control was administered to C57/BL6 mice after anesthesia. After recovery, animals were fed a diet of sildenafil (100 mg.kg(-1).day(-1)) or vehicle for 2 wk when they underwent hemodynamic measurements, and tissues were harvested. Survival was reduced in animals treated with bleomycin compared with controls and was improved with sildenafil (100.0 vs. 73.7 vs. 84.2%, P < 0.05). RV/LV+S ratio was higher in bleomycin-alone mice with improvement in ratio when sildenafil was administered (33.00 +/- 0.01% vs. 20.98 +/- 0.01% P < 0.05). Histology showed less pulmonary vascular and RV fibrosis in the group cotreated with sildenafil. Bleomycin was associated with a marked increase in superoxide generation by DHE histological staining and luminol activity in both heart and lung. Treatment with sildenafil resulted in a concomitant reduction in superoxide levels in both heart and lung. These data demonstrate that PDE5 inhibition ameliorates RV hypertrophy and pulmonary fibrosis associated with intratracheal bleomycin in a manner that is associated with improved NOS coupling and a reduction in reactive oxygen species signaling.
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PMID:PDE5A inhibition attenuates bleomycin-induced pulmonary fibrosis and pulmonary hypertension through inhibition of ROS generation and RhoA/Rho kinase activation. 1796 19

Pulmonary hypertension (PH) is a serious form of pulmonary complication that occurs less frequently in lupus than in other connective tissue diseases like scleroderma; however, it is likely that it is under-recognized in lupus. The symptoms of PH in lupus are non-specific (dyspnea, fatigue, impaired exercise tolerance) and can also be caused by other factors such as pleural or pericardial effusions, interstitial lung disease and many more, making it possible to miss the diagnosis. There are several potential causes of PH in lupus including thromboembolic disease, pulmonary vasculitis, and hypoxia and fibrosis from interstitial lung disease. Endothelin-1 is elevated in lupus and may be associated with PAH. In some studies, pulmonary arterial hypertension (PAH) has been found to be a major cause of mortality in lupus patients. Echocardiograms are a screening tool, but may yield false positives, and a right heart catheterization must be performed to confirm PAH. Early identification is important and can alter the natural history of this dangerous complication of lupus. Treatment of PAH associated with lupus includes standard PAH treatment as well as immunosuppression.
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PMID:An update in pulmonary hypertension in systemic lupus erythematosus - do we need to know about it? 1907 76

Systemic sclerosis (SSc) is a relatively rare chronic connective tissue disease characterized by varying degrees of skin fibrosis and visceral organ involvement. Pulmonary compromise, including pulmonary arterial hypertension and interstitial lung disease, is currently the leading cause of death in patients with SSc. Digital ulcers are common complications which lead to substantial morbidity and functional limitation. Until recently, treatment options for these complications were quite limited. Endothelin-1 (ET-1) is a peptide that has a role in promoting both vascular injury and the fibrotic process in SSc. Bosentan is a dual endothelin receptor antagonist approved for the treatment of pulmonary arterial hypertension. In patients with pulmonary hypertension secondary to SSc, bosentan therapy prevents deterioration in exercise capacity and may improve survival. No beneficial effect was found in one study in patients with interstitial lung disease and SSc. Bosentan is able to reduce the number of new digital ulcers in patients with either a history of previous ulcers or an active ulcer, without expediting the healing of existing ulcers. Bosentan therapy is contraindicated in pregnancy and causes elevated liver transaminases in up to 14% of patients. Hence, monthly pregnancy tests should be performed and hepatic function should be monitored.
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PMID:Bosentan in systemic sclerosis. 1859 96

The lungs are involved in many inflammatory rheumatic diseases. We will focus on the most common clinical problems. In systemic sclerosis interstitial lung disease resulting in fibrosis and pulmonary arterial hypertension (PAH) resulting in right heart failure are the leading complications requiring close cooperation between specialists in rheumatology and pulmonology. In Sjogren's syndrome interstitial pneumonia, pleural and bronchial inflammation and rarely PAH will be the main pulmonary complications. Hypereosinophilic syndrome, eosinophilic pneumonia, and Churg-Strauss syndrome are conditions that show marked eosinophilia and can clinically be confused with asthma and allergic bronchopulmonary aspergillosis. These conditions mandate thorough investigation of the lungs including bronchoscopy and possibly open lung biopsy. Finally, patients for whom treatment with tumour necrosis factor blocking agents is planned should undergo interdisciplinary management to prevent tuberculosis activation or infection.
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PMID:[Interdisciplinary point of contact between rheumatology and pneumology]. 1870 64

Nitric oxide metabolites (NOx) in serum, and alveolar concentration of NO (CA(NO)), are markers of inflammation and alveolitis, respectively, in systemic sclerosis (SSc). We prospectively evaluated the usefulness of both NOx and CA(NO) to assess lung involvement and skin fibrosis in SSc. Serum NOx, and CA(NO) measured by two different methods, namely the two-compartment (2CM) and the "trumpet" models (TM), were concomitantly assessed in 65 patients with SSc and 17 healthy controls. Whilst serum NOx remained comparable between groups, CA(NO) were significantly higher in SSc patients (n=65, 6.7ppb; 4.8-9.7 and 5.9ppb; 3.9-8.9) as compared with controls (n=17, 3.0ppb; 2.0-3.8 and 1.8ppb; 1.1-2.9, p<0.001, p<0.001) using the 2CM and the TM, respectively). CA(NO) from SSc patients with interstitial lung disease (ILD) (n=26, 8.6ppb; 6.5-10.9 and 8.5ppb; 5.9-10.7) or pulmonary arterial hypertension (n=12, 7.3ppb; 6.5-10.4 and 6.9ppb; 5.4-9.9) were significantly higher as compared with patients without ILD (n=27, 4.9ppb; 3.8-6.5 and 4.7ppb; 2.8-5.7; p<0.001 and p<0.001) using the 2CM and the TM, respectively). CA(NO) assessed either by the 2CM or the trumpet model were directly related to the extent of ILD and inversely related to DLCO. There was no correlation between NOx and ILD, or DLCO. Neither CA(NO) nor NOx was correlated with skin fibrosis and no relationship was found between CA(NO) and NOx. Alveolar concentration of NO, but not serum NOx, closely correlates with the extent of ILD in patients with systemic sclerosis. Neither parameter, however, is related to skin fibrosis.
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PMID:Exhaled nitric oxide, but not serum nitrite and nitrate, is a marker of interstitial lung disease in systemic sclerosis. 1910 Aug 54


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