Scleroderma renal crisis accompanied by new-onset pulmonary arterial hypertension: an acute systemic endothelial injury? Case report and literature.
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.
Department of Medicine, AHEPA University Hospital, Aristotle University of Thessaloniki, St. Kiriakidi 1, 54636, Thessaloniki, Greece. dimitroul@hotmail.com
Theodoros
T
Dimitroulas
Pantelis
P
Sarafidis
Vasiliki
V
Roma
Georgia
G
Karagiannopoulou
Stergios
S
Kapoulas
Hariklia
H
Dimitroula
Lucas
L
Settas
Anastasios
A
Lasaridis
D058186:Acute Kidney Injury / Q000209:etiology* / Q000628:therapy; D000328:Adult; D019332:Endothelin-1 / Q000378:metabolism; D004730:Endothelium, Vascular / Q000473:pathology; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D017582:Renal Replacement Therapy / Q000379:methods; D012595:Scleroderma, Systemic / Q000150:complications* / Q000503:physiopathology
D019332:Endothelin-1