A case of secondary focal segmental glomerulosclerosis associated with malignant hypertension

Abstract

Focal segmental glomerulosclerosis (FSGS) is associated with various clinicopathological conditions, including hypertension. We report here a case of secondary FSGS associated with malignant hypertension. A 33-yearold man with a 1-month history of visual impairment and headache visited the Department of Ophthalmology at our hospital and was found to have hypertensive retinopathy and severe hypertension (230/160 mmHg). He was referred to our department based on suspected renal dysfunction. His blood pressure on admission was 250/130 mmHg. Physical examination and laboratory tests revealed hypertensive cardiac dysfunction, focal brain edema, renal dysfunction (serum creatinine, Cr 7.07 mg/dl, blood urea nitrogen, BUN 49.9 mg/dl), massive proteinuria (10.7 g/day), and thrombotic microangiopathy. Funduscopy showed exudate, hemorrhage, and papilledema. The cause of secondary hypertension could not be identified. He was treated for primary malignant hypertension, but required hemodialysis 3 days after admission due to anuria. Treatment with antihypertensive agents resulted in the gradual recovery of renal function, although heavy proteinuria continued with nephrotic syndrome. Renal biopsy performed 1 month after admission showed features of malignant nephrosclerosis with secondary FSGS. Hemodialysis was discontinued following further improvement in renal function and the most recent laboratory tests showed proteinuria 1.8 g/day and persistent renal dysfunction (BUN 36.5 mg/dl, Cr 3.14 mg/ dl). Malignant hypertension may cause various injuries, including glomerular endothelial and epithelial cell injuries in glomerular hypertension and hyperfiltration, increase of the renin-angiotensin-aldosterone system, and endothelialepithelial interaction, resulting in the development of secondary FSGS and heavy proteinuria.

Spectrum of Complement-Mediated Thrombotic Microangiopathies: Pathogenetic Insights Identifying Novel Treatment Approaches

“…234 The concomitant finding of FSGS and TMA has been reported in four patients. In two patients, this was associated with malignant hypertension, 71,235 and in the other two cases TMA was felt to be druginduced 236,237 Two case reports describe the association of TMA and MN. 167,168 While a disease-enhancing role for complement has been generally accepted, the recent discovery of MN-specific autoantibodies to phospholipase A2 receptor (PLA2R), introduce a specific CP initiated role for the complement system in MN pathogenesis.…”

Collapsing glomerulopathy is common in the setting of thrombotic microangiopathy of the native kidney

“…[10][11][12] In native kidneys, TMA-CG has only been anecdotally described in case reports or small series in conjunction with calcineurin inhibitors-related arteriolopathy, 13 sickle-cell disease, 14 antivascular endothelial growth factor (anti-VEGF) therapy-associated TMA, 15 mixed connective tissue disease-associated TMA, 16 or malignant hypertension. 17 The question of the prevalence and significance of CG changes in the setting of histological TMA has never been addressed in an unselected fashion, using native renal biopsy specimens. The purpose of our study was to explore the prevalence of CG in TMA, its clinicopathologic features, and the outcome of TMA-CG in native kidneys.…”

Nephrotic syndrome with focal segmental glomerular lesions unclassified by Columbia classification; Pathology and clinical implication

“…Podocyte is major source of vascular endothelial growth factor (VEGF), and anti-VEGF antibody, anti-VEGF ligand, and receptor tyrosine kinase inhibitors similarly caused heavy proteinuria with TMA and FSGS lesions [ 30 , 31 ]. Several reports described the overlap between disorders with endothelial damage and histological FSGS: Preeclampsia [ 32 ], TMA induced by malignant hypertension [ 33 ] or reno-vascular hypertension [ 34 ]. A retrospective study showed that FSGS, particularly COL, was commonly (62.3%) observed among cases with TMA [ 35 ].…”