Hungarian Immunology

[Molecular and cellular basis of fibrosis: recent insights to the pathomechanism of scleroderma from animal models and fibroblast studies]

LAKOS Gabriella, SHINSUKE Takagawa, JOHN Varga

DECEMBER 20, 2002

Hungarian Immunology - 2002;1(04)

[Scleroderma is a chronic, progressive connective tissue disorder featuring inflammation, fibrosis, vascular injury, and immunologic abnormalities. Fibrosis, a hallmark of the disease, is characterized by excessive synthesis and deposition of extracellular matrix components, mainly type I collagen in affected tissue. The key target organs are the skin, lungs, kidneys, gastrointestinal tract and heart. The pathogenesis of fibrosis remains poorly understood, and effective treatments are lacking. While unifying concept to explain the pathogenesis of fibrosis has not yet emerged, multiple alterations result in the development of pathological tissue fibrosis have been recently identified. Transforming growth factor-β, a potent profibrotic cytokine plays a key role in the process. There is growing knowledge on identifying the cytokine and growth factor mediators of fibrosis, characterizing their interactions, and in delineating the cellular and molecular signaling pathways that are activated by these mediators. This review summarizes recent results obtained from fibroblast studies, animal models, and gene expression experiments. A major goal of investigations into the pathomechanism of fibrosis is identifying new therapeutic targets for scleroderma.]

COMMENTS

0 comments

Further articles in this publication

Hungarian Immunology

[3rd International Congress of Autoimmunity - the congress report]

SZEKANECZ Zoltán

Hungarian Immunology

[IN MEMORIAM]

CZIRJÁK László

Hungarian Immunology

[On the role of aging in etiology of autoimmunity]

SEMSEI Imre, ZEHER Margit, BAKÓ Gyula

[Several types of diseases, among others autoimmune illnesses, could be coupled with the general processes of aging. The two-edged sword of the immune defense is directed once against environmental attacks and on the other side against the self. However, one has to make a difference between normal (physiological) clearance and autoimmune diseases, although both sides of autoimmunity are influenced by the general processes of senescence. Aging of the thymus seems to be one of the key elements of the etiology of autoimmunity, although other cell types and their aging also play a substantial role in this process. The spontaneous genetic instability, the acquired genetic mutations due to aging and the age-related alterations of the information level of the body together may be important elements of the patomechanism of both the physiological autoimmunity and the autoimmune diseases. Nevertheless, physiological autoimmunity seems to be directed mostly by natural factors (such as aging and apoptosis) but primary autoimmune diseases may be caused by genetic instability that is enhanced by aging as well.]

Hungarian Immunology

[The new Department of Immunology and Rheumatology in Pécs]

CZIRJÁK László

Hungarian Immunology

[History of immunology in Hungary Part II]

KARASSZON Dénes, CSABA Béla

All articles in the issue

Related contents

Lege Artis Medicinae

[PRIMARY TUBULOINTERSTITIAL NEPHRITIS]

FERENCZI Sándor

[Primary tubulointerstitial nephritis is characterised by an inflammatory infiltrate of tubulointerstitial space. The infiltrate consists of T and B lymphocytes, monocytes, macrophages, neutrophyl and eosinophyl granulocytes in varying degree. It is associated with interstitial oedema and different level of tubular damage. The disease exists in acute and chronic form. The main causes of this condition are: drugs, infection, systemic diseases, malignancy and in some cases the disease is idiopathic. The pathogenesis in most cases is immune-mediated. The secondary form of tubulointerstitial nephritis can occur in primary glomerular and vascular disease and is characterised by tubulointerstitial fibrosis and tubulus atrophy. The morphological alterations are major determinants of the progression of chronic renal disease. In both forms of tubulointerstitial nephritis the development of renal insufficiency is often observed.]

Lege Artis Medicinae

[PATHOLOGICAL FEATURES OF SYSTEMIC SCLEROSIS]

VARJÚ Cecília, KUMÁNOVICS Gábor, CZIRJÁK László

[Systemic sclerosis is characterized by fibrosis and subsequent atrophy of the skin and several internal organs as well as by generalized obliterative vasculopathy. The ethiology of systemic sclerosis is not quite clear yet, but the role of certain environmental factors, genetic properties and microchimaerism has been proven. Vasculopathy is a key feature that includes both functional changes (Raynaud's phenomenon) and morphological alterations (lesion of the endothel). The triggering event is the activation of endothelial cells. This is followed by an autoimmune inflammatory process causing vascular lesion, which will eventually lead to progressive pathologic fibrosis with increased deposition of collagen and intercellular matrix proteins. Normal tissues of vital internal organs will gradually loose structure, become atrophic and irreversibly damaged. In the treatment of systemic sclerosis the most significant achievements of the past decade have been made in the therapy and prevention of scleroderma renal crisis, pulmonary arterial hypertension and other vascular complications, resulting in higher survival rates and better quality of life. In pulmonary fibrosis the beneficial effect of cyclophosphamide therapy has been proven. Today, research focuses on new therapeutic approaches based on the recently clarified molecular pathological processes, as well as on laboratory and clinical markers that predict the activity of the disease or the efficiency of therapy. The aim of the present paper is to review current knowledge on the pathology of systemic sclerosis and provide help in the diagnosis, therapy and follow-up of the disease.]

Hypertension and nephrology

[About the care of patients with hyperuricaemia and gout]

[This consensus document is intended to provide guidance for the effective and efficient treatment of asymptomatic individuals with high uric acid levels and gout patients.]