Vascular Changes in Bleomycin-Induced Scleroderma PDF Print E-mail
Tuesday, 24 January 2012 12:25
Toshiyuki Yamamoto and Ichiro Katayama
Department of Dermatology, Fukushima Medical University, Hikarigaoka 1, Fukushima 960-1295, Japan
Department of Dermatology, Osaka University, Yamadaoka 2-2, Suita, Osaka 565-0871, Japan
Received 6 June 2011; Revised 17 August 2011; Accepted 17 August 2011

Systemic sclerosis (SSc) is a connective tissue disease which shows fibrosis of the skin and various internal organs. Although the pathogenesis of SSc has not been fully elucidated yet, it is characterized by vascular injury, immunological abnormalities, and excessive accumulation of extracellular matrix (ECM) proteins in the skin and various internal organs. In particular, systemic vasculopathy plays a fundamental role in SSc and is associated with various altered vascular dysfunctions in the lung, kidney, heart, and skin.

Clinically, Raynaud’s phenomenon, digital ulcers, and abnormal nailfold capillaries are seen in association with peripheral vasculopathy. Raynaud’s phenomenon is caused by vasospasm, commonly seen in patients prior to the onset of sclerodactyly. Endothelial cells have been reported to play an important role in the initial inflammatory as well as subsequent fibrotic process. Histological analysis of the initial stage of scleroderma reveals perivascular infiltrates of mononuclear cells in the dermis, which is associated with increased collagen synthesis in the surrounding fibroblasts. T-cell interaction with vascular endothelial cells may lead to the subsequent cellular immune reaction, which may induce further vascular injury and tissue fibrosis. A number of studies have demonstrated the crucial role of several fibrogenic cytokines released from immunocytes in initiating the sequence of events leading to fibrosis.

Animal models are useful in providing clues for understanding various human diseases and for exploring new treatments. Although animal models which reproduce all the aspects of SSc are not currently available, bleomycin-induced scleroderma mouse exhibits definite dermal sclerosis mimicking human scleroderma. Inthismodel, features such as definite dermal sclerosis with dermal thickening, pulmonary fibrosis, and the presence of autoantibody in the sera are induced; however, vascular alteration in this model has not been remarked. In this paper, insights into the vascular pathogenesis in bleomycin-induced murine model are discussed.

Continue reading the full review paper, by downloading it from the link provided below.

 
More articles :

» Autoimmunity Versus Nutrition

Our immune system protects us from various diseases. However, at times it goes into an overdrive and attacks the body itself, leading to tissue destruction and degenerative diseases referred to as autoimmune diseases. Autoimmune disorders include...

» Frequency of Sexual Dysfunction In Women With Rheumatic Diseases

Sexuality is part of human life and of quality of life, accounting for individual well-being. It relates not only to sexual intercourse itself, but also to a whole spectrum that ranges from self-image and self-valuing to relationship with the...

» We Need Your Help!

Today, the Scleroderma Care Foundation embarks upon a mission of vital importance, and we are counting on your help. One of our dearest patients and friend, , is on the verge of Renal or and we are seeking out a qualified kidney donor. Currently,...

» Autoimmune Disease Overlap Syndromes

Because of their variable features, overlapping autoimmune connective tissue, thyroid and liver disorders can be difficult to diagnose.It’s not unusual for several years to go by before overlapping autoimmune conditions are properly diagnosed. One...

» Long-Term Outcomes of Scleroderma Renal Crisis

Virginia D. Steen, MD, and Thomas A. Medsger Jr., MDPublished: October 17th, 2000

» Actelion Pharmaceuticals Announces Commercial Availability Of Epoprostenol For Injection For The Treatment Of Pulmonary Arterial Hypertension

Actelion Pharmaceuticals US, Inc. today  announced the commercial availability of Epoprostenol for Injection, an improved formulation of epoprostenol that is stable at room temperature, for the treatment of primary pulmonary hypertension and...