Malignant Arterial Hypertension Revealing Focal Segmental Hyalinosis

Abstract

Malignant arterial hypertension (MAH) is a hypertensive emergency associated with grade III or IV retinopathy. Focal segmental hyalinosis (FSH), of late onset, is one of its rare entities, with an incidence of 7 per million, responsible for 15-20% of nephrotic syndromes in adults. We report a case of segmental and focal hyalinosis in a 27-year-old patient with no previous history of malignant hypertension, who consulted us for neurosensory signs and ocular redness. The clinical examination showed a hypertensive peak at 240/150mm Hg with myocardial hypertrophy confirmed by electrocardiogram and transthoracic echo. A fundus examination completed by an optical coherence tomography revealed a KIRKENDAL stage III retinopathy associated with an occlusion of a branch of the central retinal vein. An impure nephrotic syndrome associated with renal failure was observed. A biopsy of a fragment of renal parenchyma supplemented by pathology was performed in favour of FH with chronic tubulointerstitial disease. The patient was managed urgently with nicardipine 3-5mg/hr by electric syringe within 24 hours, followed by a triad of antihypertensive drugs consisting of amlodipine, indapamide and Ramipril at full dose combined with prednisone 60mg/day for 4 months with a 6 month taper. The evolution was marked by a normalisation of the renal function, disappearance of the proteinuria and even a normalisation of his eye fundus after one year. FSH constitutes a group of heterogeneous pathologies with multiple causes, of which malignant hypertension is a secondary one, requiring not only symptomatic but also etiological management.