Wunderlich Syndrome as an unusual presentation of Microscopic Polyangiitis: A Case report with Review of Literature
Authors
BrajaGopal Jana; Kaustav Bhowmick; Arghya Chattopadhyay ; Uddalok Das; Indranil Sen; Spandan Bhadury;
Keywords
Wunderlich syndrome, characterized by spontaneous perinephric hematoma with subcapsular extension has been scarcely reported in microscopic polyangiitis (MPA). We report the case of a 45-year-old woman, who presented with constitutional symptoms, left-eye episcleritis, and rapidly progressive glomerulonephritis. She developed sudden, severe left flank pain with hemoglobin drop two days after admission. Both computed tomography (CT) and non-contrast magnetic resonance imaging revealed large left-sided perinephric hematoma. CT angiography failed to demonstrate intrarenal aneurysms. A remarkable reduction in size of her perinephric hematoma was observed after three and a half months of treatment with glucocorticoids and intravenous cyclophosphamide (IV CYC) following the international guidelines. A literature review on renal vessel involvement in antineutrophil cytoplasmic antibody-associated vasculitis revealed 26 case reports and one case series with 20 cases of renal aneurysms. Eighteen cases in the case reports (69.2%) and nine in the case series (45%) ruptured their renal arteries. The majority (44.4%) were managed with IV CYC and high-dose glucocorticoids. Angioembolization, renal replacement therapy, and plasma exchange were used as adjuvant measures. Only three patients (16.7%) underwent nephrectomy, while the majority(63.6%) fully recovered.
BrajaGopal Jana
North Bengal Medical College and Hospital, Sushrutranagar, Darjeeling, West Bengal, India.
Kaustav Bhowmick
North Bengal Medical College and Hospital, Sushrutranagar, Darjeeling, West Bengal, India.
Arghya Chattopadhyay
North Bengal Medical College and Hospital, Sushrutranagar, Darjeeling, West Bengal, India.
Uddalok Das
North Bengal Medical College and Hospital, Sushrutranagar, Darjeeling, West Bengal, India.
Indranil Sen
North Bengal Medical College and Hospital, Sushrutranagar, Darjeeling, West Bengal, India.
Spandan Bhadury
North Bengal Medical College and Hospital, Sushrutranagar, Darjeeling, West Bengal, India.
North Bengal Medical College and Hospital, Sushrutranagar, Darjeeling, West Bengal, India.
Kaustav Bhowmick
North Bengal Medical College and Hospital, Sushrutranagar, Darjeeling, West Bengal, India.
Arghya Chattopadhyay
North Bengal Medical College and Hospital, Sushrutranagar, Darjeeling, West Bengal, India.
Uddalok Das
North Bengal Medical College and Hospital, Sushrutranagar, Darjeeling, West Bengal, India.
Indranil Sen
North Bengal Medical College and Hospital, Sushrutranagar, Darjeeling, West Bengal, India.
Spandan Bhadury
North Bengal Medical College and Hospital, Sushrutranagar, Darjeeling, West Bengal, India.