Clinical medicine (London, England)
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We present a rare case of fibromuscular dysplasia (FMD) manifesting in the mid segment of right renal artery, which led to the development of refractory hypertension. The patient received balloon angioplasty to a severe lesion on the middle of right renal artery and subsequently had normalisation of blood pressures. ⋯ The patient showed renal artery stenosis (RAS) with distal tumour-like dilatation, and multiple tortuosity and stenosis in carotid artery and coronary artery. At follow-up 2 months later, her blood pressures had normalised.
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Despite its recognition as an 'ANCA-associated vasculitis' (AAV), eosinophilic granulomatosis with polyangiitis (EGPA) is ANCA negative in up to 60% of cases. Herein, we report the case of a young man with a clinical syndrome highly suggestive of EGPA but with repeated negative ANCA serology, ultimately presenting with cardiac arrest before recognition of the primary systemic vasculitis, whereupon he received successful induction therapy with high dose glucocorticoids and cyclophosphamide. The case illustrates the importance of awareness of ANCA negative AAV among general physicians in order to minimise morbidity and mortality.