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Abstract: FR-PO613

Glomerular Microangiopathy in a Patient With Takayasu Arteritis

Session Information

Category: Glomerular Diseases

  • 1302 Glomerular Diseases: Immunology and Inflammation

Authors

  • Fujii, Makiko, Kurume Daigaku Igakubu Daigakuin Igaku Kenkyuka, Kurume, Fukuoka, Japan
  • Taguchi, Kensei, Kurume Daigaku Igakubu Daigakuin Igaku Kenkyuka, Kurume, Fukuoka, Japan
  • Fukami, Kei, Kurume Daigaku Igakubu Daigakuin Igaku Kenkyuka, Kurume, Fukuoka, Japan
  • Ito, Sakuya, Kurume Daigaku Igakubu Daigakuin Igaku Kenkyuka, Kurume, Fukuoka, Japan
Introduction

Takayasu arteritis (TA) is a large vessel inflammation that predominantly involves aorta and its main arteries. TA-caused kidney injury is known to be mainly due to renal artery stenosis. However, glomerulonephritis has been histologically identified in some TA cases.

Case Description

A 69-year-old female presented with leg edema and refractory hypertension with nephrotic range proteinuria (17.0 g/gCr). She exhibited >10 mmHg blood pressure discrepancy between left and right upper limbs and severe aortic regurgitation. Lab test showed high level of erythrocyte sedimentation rate. Contrast-enhanced CT identified wall thickening of the left subclavian artery, which led to the diagnosis of TA. Renal biopsy demonstrated that double basement membrane, enlarged subendothelial space, and mesangiolysis with no immunoglobulin deposition. Although azilsartan and nifedipine had been prescribed, systolic blood pressure (sBP) was still >160 mmHg. Thus, while oral prednisolone was started to suppress TA-induced inflammation, azilsartan was replaced with sacubitril valsartan, an angiotensin receptor-neprilysin inhibitor (ARNI), for treating refractory hypertension. After a few weeks, sBP was decreased to 130 mmHg with sacubitril valsartan 200mg QD and proteinuria was dramatically improved to 0.5 g/gCr.

Discussion

TA commonly involves the renal artery, leading to stenosis and subsequent ischemic nephropathy. However, no stenosis and occlusion were observed in renal artery of this case. Small vessel inflammation in the kidney such as vessel wall necrosis and immune cells infiltration was not found. Serum levels of pro-inflammatory cytokines including VEGF and IL-6 were within the normal range. The results indicate that TA-caused hypertension, but not vessel inflammation induced by TA, plays a pathological role for development of glomerular microangiopathy. A previous study showed that 70% of TA patients presenting with glomerular lesion has severe hypertension and intimal thickening in renal arterioles was observed more frequently in TA patients presenting with glomerular lesion when compared to those without glomerulonephritis, which supports our hypothesis. In the present case, ARNI significantly reduced sBP which might directly contribute to the improvement of proteinuria. ARNI can be potent to control hypertension more effectively than conventional hypertensive agents in TA patients.