Lege Artis Medicinae

[The Generous Patron: Marcell Nemes]

NAGY Zsuzsanna

OCTOBER 20, 2011

Lege Artis Medicinae - 2011;21(10)

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FERENCZI Andrea

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[Meditation on health]

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[Pulmonary arterial hypertension in systemic autoimmune diseases]

VÉGH Judit, ZEHER Margit

[Pulmonary arterial hypertension is a rare disease, but it occurs more often in systemic autoimmune diseases, where it represents one of the most severe, life-threatening complications. Its development is due to an immunoregulatory disorder characteristic to systemic diseases, persistent inflammation and the subsequent endothelial dysfunction, the presence of pathogenic autoantibodies, smooth muscle cell dysfunction and complex angiogenetic disorder. As a consequence of endothelial cell dysfunction, the balance between regulatory factors of vasoconstriction and vasodilation is disrupted. Intimal hyperplasia, endothelial cell proliferation, media hypertrophy and local thrombus formation can be observed and one of the main pathomorphological characteristic features, plexiform lesion develops, leading to obliterative vasculopathy. A more severe form of the disease develops in systemic sclerosis, which is explained by the main pathophysiological elements of scleroderma, namely immunoregulatory disorder, vasculopathy and fibroblast dysfunction. It is not easy to monitor the disease in these cases, because the deterioration can be caused by many other factors as well. Therefore, beseides the usual examinations, biomarkers and screening methods have a significant role. Treatment is not simple either, since no wellapplicable algorithms are available. In many disorders (systemic lupus erythematosus, mixed connective tissue disease, rheumatoid arthritis), effective immunosuppressive therapy started in time is crucial, whereas in case of systemic sclerosis, the principles of therapy applied for the idiopathic form should be followed.]

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[Recently, physicians and medical literature are more concerned about end-of-life care. A review is given of studies dealing with the rights of the dying patient, with advance directives and with possible treatments in the last days of life. A survey was done in author's department on medical therapy of the terminal period of 103 inpatients, died between 01. 10. 2001. and 31. 03. 2002. Comparing these data with those of American, Finnish etc. authors, the treatments seem to be more generous - probably because in Hungary it is not (yet?) usual to ”declare” endof- life care and to withdraw active therapy. However, indication of antibiotics seems to be more clinical (28.1%, vs. 42%-88% given by similar foreign data). Antibiotics are not palliative means, however, they may be administered in the last days, if the patient is suffering from a terminal infection. Indications and choice of antibiotics are suggested in these cases; medical and ethical problems discussed. Physicians can relieve the physical and mental distressing symptoms of the dying patient and ensure human dignity and peace of the last days.]

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