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Scleroderma Renal Crisis Accompanied by New-Onset Pulmonary Hypertension: An Acute Systemic Endothelial Injury? Case Report and Literature Review

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Systemic Sclerosis (SSc) is a multisystem connective tissue disease characterized by fibrosis of the skin and internal organs and extensive vasculopathy. We report a case of a 41 year-old white woman with a 10 year-history of limited scleroderma, who developed the rare combination of Scleroderma Renal Crisis (SRC) and Systemic Sclerosis related Pulmonary Arterial Hypertension (SScPAH) in the same time. Although the patient received the proposed antihypertensive treatment, the renal function did not recover, and she initiated on renal replacement therapy. SRC and SScPAH are two aspects of SSc vasculopathy characterized by endothelial dysfunction mediated by endothelin-1 and other vasoactive hormones. Further new studies with therapies directed towards the underlying mechanisms of SRC (i.e. endothelin-receptor antagonists), which are proven helpful in SScPAH, should take place to establish new therapeutic options and improve prognosis of these patients, for which our therapeutic armamentarium is currently poor.

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Keywords: Fundoscopic examination; Rheumatology; Scleroderma; Scleroderma Renal Crisis (SRC); Systemic Sclerosis (SSc); Systemic Sclerosis related Pulmonary Arterial Hypertension (SScPAH); Vasculopathy; angiotensin converting enzyme (ACE); anti-RNA polymerase; anti-topoisomerase-1; antihypertensive treatment; bosentan; cyclosporine therapy; echocardiography; endothelin-1; hemodialysis; mesangial cells; paracetamol; prophylaxis; pulmonary hypertension; renal crisis; sitaxsentan; thrombotic microangiopathy

Document Type: Research Article

Publication date: September 1, 2010

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  • Inflammation & Allergy - Drug Targets aims to cover all the latest and outstanding developments on the medicinal chemistry, pharmacology, molecular biology, genomics and biochemistry of contemporary molecular targets involved in inflammation and allergy e.g. disease specific proteins, receptors, enzymes, genes. Each issue of the journal contains a series of timely in-depth reviews written by leaders in the field covering a range of current topics on drug targets involved in inflammation and allergy. As the discovery, identification, characterization and validation of novel human drug targets for anti-inflammation and allergy drug discovery continues to grow, this journal has become essential reading for all pharmaceutical scientists involved in drug discovery and development.
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