Bilateral Subcapsular and Perinephric Hemorrhage as the Initial Presentation of Polyarteritis Nodosa

Abstract

Polyarteritis nodosa is a systemic vasculitis of medium and small-sized arteries associated with aneurysm formation. Aneurysms may be complicated by thrombosis, rupture and hemorrhage. Kidney involvement in polyarteritis nodosa can rarely be complicated by Wunderlich syndrome which is characterized by acute onset of spontaneous, nontraumatic subcapsular and perirenal hematomas. A 22-year-old woman with mental retardation due to childhood cytomegalovirus infection presented with flank pain, weight loss and fever, and was found to have bilateral subcapsular and perinephric hematomas. Spontaneous bilateral renal hemorrhage as the initial manifestation of polyarteritis nodosa is rare, and it can be associated with delays in diagnosis and treatment.

Polyarteriitis Nodosa with Real Arteries and Vein Involvement and Uroligical Manifestations: Case Report

“…The typical clinical presentation is a loin pain, significant Hb fall, hypotension and a peri-renal mass, which was also found in our patient. In some cases surgical evacuation of the hematoma or partial/complete nephrectomy is necessary [9][10][11].…”

“…His symptoms persisted; he lost 5 kilos of weight. Within next three months work-up found proteinuria 0.1 g/L and microhematuria 4-5 RBC/hpf, Plt count 448x10 9 /L, CRP 70 mg/L, and IgG antibodies against EBV and VCA-EBV. PCR test for EBV DNA was negative; RF, ACCP, anti-DNA, antinuclear and antiphospholipid antibodies, pANCA, cANCA -all within normal range; blood culture was negative.…”

Successful non-surgical treatment on bilateral spontaneous perirenal giant hematomas in rapidly progressive glomerulonephritis with MPO-ANCA positive

“…Seventeen of 20 patients with PN treated for spontaneous perirenal hematomas required angioembolization or nephrectomy, whereas the other three were cured following conservative treatment (Table 2) [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]. Some of these patients may actually have had MPA.…”

Spontaneous perinephric hemorrhage (Wunderlich syndrome) secondary to polyarteritis nodosa: Computed tomography and angiographic findings

“…The kidneys are affected in 80% cases with hypertension, proteinuria and ultimately renal functional deterioration being the most common manifestations. [234] Spontaneous perinephric hemorrhage is a rare, but potentially devastating complication of PAN. About 60 cases have been reported in the literature.…”

“…[2] It is secondary to the rupture of arterial aneurysms that usually involve the renal artery, and its segmental and interlobar divisions. [3] Nephrectomy is associated with a high mortality rate (50%) in the acute setting. [2] Angiography with selective embolization allows preservation of renal parenchyma and is now the treatment of choice in cases of active bleeding.…”