Pulmonalis artériás hypertonia szisztémás autoimmun kórképekben

Translated title of the contribution: Pulmonary arterial hypertension in systemic autoimmune diseases

Végh Judit, M. Zeher

Research output: Contribution to journalArticle

Abstract

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.

Original languageHungarian
Pages (from-to)601-606
Number of pages6
JournalLege Artis Medicinae
Volume21
Issue number10
Publication statusPublished - Oct 2011

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Pulmonary Hypertension
Autoimmune Diseases
Systemic Scleroderma
Endothelial Cells
Tunica Intima
Mixed Connective Tissue Disease
Immunosuppressive Agents
Rare Diseases
Vasoconstriction
Vasodilation
Systemic Lupus Erythematosus
Autoantibodies
Hypertrophy
Smooth Muscle Myocytes
Hyperplasia
Rheumatoid Arthritis
Thrombosis
Fibroblasts
Biomarkers
Cell Proliferation

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Pulmonalis artériás hypertonia szisztémás autoimmun kórképekben. / Judit, Végh; Zeher, M.

In: Lege Artis Medicinae, Vol. 21, No. 10, 10.2011, p. 601-606.

Research output: Contribution to journalArticle

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