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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

For the treatment of different forms of systemic sclerosis (SSc), drugs play a predominant role. Depending on disease activity as well as type and severity of cutaneous, vascular and internal organ manifestations, different systemic (antiinflammatory, immunosuppressive, antifibrotic) or organ-specific therapies are used. The scientific basis of most treatment modalities is insufficient and incomplete. There is sufficient evidence for an antiinflammatory and antiproliferative efficacy of glucocorticosteroids, methotrexate, cyclophosphamide and cyclosporine A in the treatment of diffuse cutaneous systemic sclerosis. Vasoactive therapies play an important role in treating Raynaud's phenomenon (nifedipine or other dihydropyridines, prostaglandin analogs, losartan, prazosine), and arterial (ACE blockers, AT-1 antagonists) or pulmonary (epoprostenol) hypertension. Cyclophosphamide is effective in fibrosing alveolitis, prokinetic substances (metoclopramid, domperidone) in gastroesophageal dysmotility or octreotide in intestinal pseudoobstruction. Physical therapies (e.g., massage) are poorly studied. In particular cases, surgical measures (e.g., removal of calcifications) are necessary.
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PMID:[Evidence-based therapy of systemic sclerosis]. 1182 42

Lung transplantation has become an accepted treatment modality for end stage lung disease including emphysema, fibrosing alveolitis, cystic fibrosis, pulmonary hypertension and bronchiectasis. Despite the use of potent immunosuppressive drugs, acute rejection occurs frequently, especially in the first few weeks and months after transplantation. Bacterial, viral and fungal infections frequently occur in lung transplant recipients. Rapid diagnosis and adequate treatment of infections is needed. The side effects with the use of long term immunosuppressive agents includes renal toxicity, hypertension, neurotoxicity, hyperlipidemia, leucopoenia, hyperglycaemia, weight gain, osteoporosis and malignancy. However, obliterative bronchiolitis (OB) which is regarded as a chronic rejection process remains the dominant cause of morbidity and mortality in the long-term survivors of lung transplantation. This article focuses on the postoperative and long term management of lung transplant recipients.
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PMID:Lung transplantation: management and complications. 1184 31

Clinical, functional, immunological and x-ray examinations were performed in 32 patients with interstitial pulmonary diseases (14 with idiopathic fibrosing alveolitis, 6 with exogenic allergic alveolitis and 12 with diffuse affection of the connective tissue). The diagnosis was verified using high resolution computed tomography. Lesser circulation was studied with doppler-echocardiography. In 9 patients the diagnosis was verified by thoracoscopic biopsy of the lung. Control group consisted of 16 healthy subjects. The highest pressure in the trunk of the pulmonary artery (31 +/- 4.3 mmHg) was registered in patients with idiopathic fibrosing alveolitis. The severity of pulmonary arterial hypertension correlated with that of respiratory disorders, clinical symptoms and changes in the lungs. Blood pressure in the pulmonary artery trunk tended to elevation with progression of alterations in the lungs registered by computed tomography.
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PMID:[Pulmonary hypertension in interstitial diseases of the lungs]. 1213 97

Beyond medical therapy, different interventional and surgical approaches exist for treatment of pulmonary arterial hypertension (PAH). Atrial septostomy has been applied in patients with lack of response to medical therapy in the absence of other surgical treatment options. With growing experience, procedure-related death rates have been reduced to 5.4%, and the most suitable patient group has been identified among patients with a mean right atrial pressure between 10 and 20 mm Hg. Pulmonary endarterectomy is the accepted form of treatment for patients with chronic thromboembolic pulmonary hypertension. Establishing the diagnosis and the classification of the type of lesions by pulmonary angiography is crucial for optimal patient selection. Perioperative mortality rates have been reduced to <10% in experienced centers, and the hemodynamic improvement is dramatic and sustained. Lung and heart-lung transplantation remains the procedure of choice for patients unsuitable for other treatment modalities. Timing of the procedure is difficult because waiting times vary between centers and usually are in a high range. Early referral of patients unresponsive to other treatment forms is therefore of importance to avoid transplantation of patients with established significant comorbidity. The survival rate during the first five years after transplantation for PAH is intermediate among the lung diseases, lower than chronic obstructive pulmonary disease but higher than idiopathic pulmonary fibrosis.
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PMID:Interventional and surgical modalities of treatment for pulmonary arterial hypertension. 1519 82

Systemic sclerosis (scleroderma) is considered as the most severe connective tissue disease. It is characterized by an abnormal immune activation, a vasculopathy and a fibrosis of the skin and of multiple internal organs. Numerous progress in the understanding of the pathogenesis with identification of key molecules have permit to introduce novel treatments that improve the management of some aspects of the disease. ACE inhibitors are effective in resolving renal crisis. Cyclophosphamide is useful for treatment of fibrosing alveolitis. Prostaglandins, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors permit to improve the treatment of the vascular complications (digital ulcerations, pulmonary arterial hypertension) of scleroderma.
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PMID:[Pathogenic mechanisms in systemic sclerosis and their therapeutical consequences. Part 2: treatment]. 1671 Nov 51

The prognosis of systemic sclerosis depends chiefly on the extent of the skin lesions, which correlates with the severity of the cardiovascular, pulmonary, and renal manifestations. An erythrocyte sedimentation rate greater than 15-25 mm/h or a hemoglobin level lower than 12.5-11 g/dl is associated with a 2.5- to 3-fold increase in mortality. Anticentromere antibodies are associated with delayed pulmonary hypertension, anti-topoisomerase I antibodies (Scl 70) with interstitial lung disease, and anti-RNA polymerase III antibodies with renovascular hypertension. The risk of death is directly related to the autoantibody pattern. For instance, in a study of 1432 cases from the Pittsburgh Scleroderma Databank, 10-year survival among patients with limited cutaneous disease was 88% in the group with anti-U1-RNP, 75% in the group with anticentromere antibodies, 72% in the group with anti-PmScl, and 65% in the group with anti-Th/To. Ten-year survival in patients with diffuse cutaneous disease was 64% with anti-topoisomerase antibodies, 61% with anti-U3-RNP, and 75% with anti-RNA polymerase III. Several prognostic markers are also available for predicting the risk of organ involvement. For instance, serum levels of KL-6, surfactant proteins SP-A and SP-D, the collagen peptide PIIINP, and homocysteine are associated with the risk of fibrosing alveolitis. Serum levels of CD40L and NP-ProBNP, circulating endothelial cells, and serum anticardiolipin titers correlate with the risk of arterial hypertension. Serum VCAM1 and markers for oxidative stress such as carboxyl terminus residues predict the risk of vascular disease. Other serum markers for organ involvement are under study, although their predictive performance remains to be evaluated.
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PMID:Prognostic markers for systemic sclerosis. 1679 48

Systemic sclerosis (SSc) is a generalised connective tissue disease of unknown origin, which clinically shows by skin thickening and sclerosis of different extent (scleroderma) and by typical involvement of visceral organs. At the same time fibrotic and sclerotic changes occur in the blood vesel walls. SSc usually involves females at young and middle age. Myalgias, arthralgias and arthritis are nonspecific, tendon friction rubs in fingers are more typical for this diagnosis. Gastrointestinal involvement starts early in the oropharyngeal part, esophagus and proceeds into the distal parts. Fibrotic changes lead to slow transit dysmotility and pseudoobstruction and/or dilation of the bowels. The main symptoms are dysphagia, pyrosis, malabsorption and constipation. SSc produces two major patterns of abnormality within the lungs a fibrosing alveolitis or a primary pulmonary vascular disease. More frequently an insterstitial process develops which can be followed by pulmonary arterial hypertension. Cardiac involvement can also have different forms. Myocardial fibrosis usually appears at first in the conduction system by arrhythmias and various conduction blocks while pericarditis is mostly asymptomatic. Renal manifestation of SSc is observed in 8-10% patients. The most severe form--scleroderma renal crisis is characterised by the new onset of accelerated hypertension and rapidly progressive oliguric renal failure. No therapies have been proven to modify the course of SSc. Some of the drugs can affect only the skin changes. Majority of the currently applied agents have only a symptomatic effect.
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PMID:[New trends in diagnosis and treatment of systemic sclerosis]. 1696 13

Scleroderma, also known as progressive systemic sclerosis (SSc), is a multisystem autoimmune disorder characterized by inflammation and fibrosis involving the skin as well as internal organs such as the vasculature, esophagus, and the respiratory tract. Pulmonary involvement consists most often of interstitial fibrosis and pulmonary vascular disease leading to pulmonary arterial hypertension (PAH). Bronchiectasis is an uncommon pulmonary manifestation of systemic sclerosis. Pulmonary hemorrhage with acute renal failure and diffuse alveolar hemorrhage in the absence of a history of renal involvement or penicillamine intake have rarely been reported in patients with systemic sclerosis.On high resolution CT, evidence of interstitial disease is seen in approximately 90% of patients, the main findings being a fine reticular pattern involving the subpleural regions of the lower lobe. Other common findings include ground-glass opacities, honeycombing, and parenchymal micronodules. The most distinctive pulmonary histologic findings in patients with scleroderma are the vascular changes found in PAH in the absence of significant interstitial fibrosis.There is no strong evidence that any drug alters the course of the two main types of lung disease in systemic sclerosis. This apparent failure of therapy may reflect the fact that pulmonary involvement is usually identified at an established or late stage. It has been suggested that, for fibrosing alveolitis, corticosteroids are most effective if given in combination with cyclophosphamide. In some patients with SSc, PAH has been considered as a major cause of morbidity and mortality. Centrally infused prostacyclin (epoprostenol) and its subcutaneously infused analog treprostinil improve hemodynamics, as well as the quality of life and survival in these patients. Iloprost has also shown a positive effect on PAH in SSc patients. More recently, bosentan, an endothelin receptor antagonist, has proved effective in controlling PAH after 6 months' treatment. Sildenafil has been used as a selective pulmonary vasodilator in SSc patients with isolated PAH. This drug decreased mean pulmonary artery pressure and pulmonary vascular resistance, and increased cardiac output, with much improvement of the physical condition of the patients. Lung transplant can be considered as a last option.Clinicians must be aware of the possibility of lung disease in patients with SSc so that it can be treated as early as possible.
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PMID:Pulmonary involvement in systemic sclerosis. 1715 72

Lung transplantation (LT) is an appropriate therapeutic option for patients with severe, fibrocystic pulmonary sarcoidosis refractory to medical therapy. Survival rates following LT for sarcoidosis are generally comparable to other indications. Timing of transplantation for patients with sarcoidosis is challenging because mortality rates are high (27 to 53%) among sarcoid patients awaiting LT. Deciding when to refer patients for LT is difficult because models predicting mortality have not been validated. Importantly, algorithms or parameters predicting mortality in idiopathic pulmonary fibrosis and other interstitial pneumonias may not apply to sarcoidosis. Pulmonary function tests do not correlate well with mortality risk in patients with sarcoidosis. However, retrospective studies have shown that the presence of pulmonary arterial hypertension in sarcoidosis is an ominous sign and warrants referral for LT. This article reviews indications and contraindications to LT among patients with sarcoidosis, examines risk factors for mortality, and discusses optimal timing of referral for LT. Early referral of patients allows for timely evaluation of patients for possible listing. If the risks specific to transplantation in sarcoidosis are considered and carefully evaluated, outcomes are reasonable and match those of other diagnoses. Recurrent sarcoidosis in the lung allografts can occur but does not affect survival or risk for complications.
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PMID:Lung transplantation in sarcoidosis. 1733 Jan 98

It was hypothesised that, apart from right ventricular (RV) dysfunction, patients with idiopathic pulmonary fibrosis (IPF) also exhibit left ventricular (LV) impairment, which may affect disease progression and prognosis. The aim of the present study was to evaluate LV performance in a cohort of IPF patients using conventional and tissue Doppler ECG. IPF patients exhibiting mild-to-moderate pulmonary arterial hypertension (mean age 65+/-9 yrs; n = 22) and healthy individuals (mean age 61+/-6 yrs; n = 22) were studied. Conventional and tissue Doppler ECG were used for the evaluation of RV and LV systolic and diastolic function. In addition to the expected impairment in RV function, all patients showed a characteristic reversal of LV diastolic filling to late diastole compared with controls (early diastolic peak filling velocity (E)/late diastolic peak filling velocity 0.7+/-0.2 versus 1.5+/-0.1, respectively). Patients with IPF also exhibited lower peak myocardial velocities in early diastole (E(m); 5.7+/-1.1 versus 10.3+/-1.6 cm x s(-1), respectively), higher in late diastole (A(m); 8.9+/-1.3 versus 5.5+/-0.8 cm x s(-1), respectively), lower E(m)/A(m) ratio (0.6+/-0.1 versus 1.9+/-0.5, respectively) and higher E/E(m) ratio (10.8+/-3 versus 6+/-0.6, respectively), all indicative of LV diastolic dysfunction. Moreover, LV propagation velocity was significantly lower in IPF patients (46+/-13 versus 83+/-21 cm x s(-1), respectively). Physicians should be aware that patients with idiopathic pulmonary fibrosis exhibit early impairment of left ventricular diastolic function.
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PMID:Left ventricular diastolic dysfunction in idiopathic pulmonary fibrosis: a tissue Doppler echocardiographic [corrected] study. 1925 11


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