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

Author(s): Theodoros Dimitroulas, Pantelis Sarafidis, Vasiliki Roma, Georgia Karagiannopoulou, Stergios Kapoulas, Hariklia Dimitroula, Lucas Settas, Anastasios Lasaridis.

Journal Name: Inflammation & Allergy - Drug Targets (Discontinued)
(Formerly Current Drug Targets - Inflammation & Allergy)

Volume 9 , Issue 4 , 2010

Abstract:

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.

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

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Article Details

VOLUME: 9
ISSUE: 4
Year: 2010
Page: [313 - 318]
Pages: 6
DOI: 10.2174/187152810793358750
Price: $58

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