Tags

Type your tag names separated by a space and hit enter

Scleroderma renal crisis accompanied by new-onset pulmonary arterial hypertension: an acute systemic endothelial injury? Case report and literature.
Inflamm Allergy Drug Targets. 2010 Sep; 9(4):313-8.IA

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.

Authors+Show Affiliations

Department of Medicine, AHEPA University Hospital, Aristotle University of Thessaloniki, St. Kiriakidi 1, 54636, Thessaloniki, Greece. dimitroul@hotmail.comNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

20969543

Citation

Dimitroulas, Theodoros, et al. "Scleroderma Renal Crisis Accompanied By New-onset Pulmonary Arterial Hypertension: an Acute Systemic Endothelial Injury? Case Report and Literature." Inflammation & Allergy Drug Targets, vol. 9, no. 4, 2010, pp. 313-8.
Dimitroulas T, Sarafidis P, Roma V, et al. Scleroderma renal crisis accompanied by new-onset pulmonary arterial hypertension: an acute systemic endothelial injury? Case report and literature. Inflamm Allergy Drug Targets. 2010;9(4):313-8.
Dimitroulas, T., Sarafidis, P., Roma, V., Karagiannopoulou, G., Kapoulas, S., Dimitroula, H., Settas, L., & Lasaridis, A. (2010). Scleroderma renal crisis accompanied by new-onset pulmonary arterial hypertension: an acute systemic endothelial injury? Case report and literature. Inflammation & Allergy Drug Targets, 9(4), 313-8.
Dimitroulas T, et al. Scleroderma Renal Crisis Accompanied By New-onset Pulmonary Arterial Hypertension: an Acute Systemic Endothelial Injury? Case Report and Literature. Inflamm Allergy Drug Targets. 2010;9(4):313-8. PubMed PMID: 20969543.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Scleroderma renal crisis accompanied by new-onset pulmonary arterial hypertension: an acute systemic endothelial injury? Case report and literature. AU - Dimitroulas,Theodoros, AU - Sarafidis,Pantelis, AU - Roma,Vasiliki, AU - Karagiannopoulou,Georgia, AU - Kapoulas,Stergios, AU - Dimitroula,Hariklia, AU - Settas,Lucas, AU - Lasaridis,Anastasios, PY - 2010/08/16/received PY - 2010/08/31/accepted PY - 2010/10/26/entrez PY - 2010/10/26/pubmed PY - 2011/2/18/medline SP - 313 EP - 8 JF - Inflammation & allergy drug targets JO - Inflamm Allergy Drug Targets VL - 9 IS - 4 N2 - 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. SN - 2212-4055 UR - https://www.unboundmedicine.com/medline/citation/20969543/Scleroderma_renal_crisis_accompanied_by_new_onset_pulmonary_arterial_hypertension:_an_acute_systemic_endothelial_injury_Case_report_and_literature_ L2 - http://www.eurekaselect.com/87017/article DB - PRIME DP - Unbound Medicine ER -