A "silent" course of normotensive scleroderma renal crisis: case report and review of the literature.
Scleroderma renal crisis (SRC) is a complication of systemic sclerosis characterized by the sudden onset of accelerated arterial hypertension, followed by progressive renal failure. Rarely, patients with SRC may be normotensive on presentation. These patients have poorer prognosis and higher mortality rates than those with hypertensive SRC. This is partly explained by the insidious course of normotensive SRC leading to delayed diagnosis and treatment. Normotensive patients also seem to be less responsive to current treatment modalities. Since available data on etiology, pathogenesis, and risk factors of the disease are inadequate, no effective therapy has been established to date. We report a patient with diffuse cutaneous scleroderma who developed SRC during his hospitalization. The patient remained normotensive and had an insidious course until oliguria and signs of hypervolemia occurred. Etiology, pathogenesis, risk factors, diagnosis, treatment modalities and prognosis of normotensive SRC are also discussed through previously published reports.
Akoglu
Hadim
H
Department of Nephrology, Ankara Numune Research and Education Hospital, Ankara Numune Eğitim ve Araştirma Hastanesi Nefroloji Kliniği, Samanpazari, 06100, Ankara, Turkey. drakomd@yahoo.com
Atilgan
Gokhan Kadir
GK
Ozturk
Ramazan
R
Yenigun
Ezgi Coskun
EC
Gonul
Ipek Isik
II
Odabas
Ali Riza
AR
D058186:Acute Kidney Injury / Q000175:diagnosis* / Q000209:etiology; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D001794:Blood Pressure; D017809:Fatal Outcome; D006801:Humans; D006977:Hypertension, Renal / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D007902:Length of Stay; D008297:Male; D008875:Middle Aged; D010351:Patient Discharge; D006435:Renal Dialysis; D045743:Scleroderma, Diffuse / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis*
D000806:Angiotensin-Converting Enzyme Inhibitors