Hepatobiliary Complications of Polyarteritis Nodosa

Abstract

Although polyarteritis nodosa (PAN) may result in thrombosis or aneurysm formation in any organ in the body, hepatobiliary complications are unusual. We reviewed seven cases that demonstrated the diagnostic difficulties and therapeutic options available in the management of hepatobiliary PAN. No consistent sign that indicated the severity of hepatobiliary PAN could be identified. In cases of thrombotic PAN, acalculus cholecystitis usually could be diagnosed preoperatively. Early tissue diagnosis and aggressive intervention are required for appropriate patient treatment. If the diagnosis is unclear, a preoperative muscle or skin biopsy specimen is often helpful in establishing a tissue diagnosis of PAN, even if no obvious pathologic condition is evident. Patients who undergo celiotomy for acalculus cholecystitis or peritoneal signs of an unclear origin should have tissue specimens (gallbladder wall, liver, or omentum) submitted for pathologic study. Angiography may be diagnostic preoperatively or when results of biopsies are equivocal. In addition, early angiography can define the extent of visceral involvement and permit control by embolization of hemorrhage secondary to aneurysm rupture. Awareness of the possibilities of thrombotic, ischemic, or bleeding complications from PAN allows more aggressive and rapid management of abdominal complaints, especially in patients who are receiving immunosuppressant therapy.

Single-organ gallbladder vasculitis: Characterization and distinction from systemic vasculitis involving the gallbladder. An analysis of 57 patients

“…Mean duration of GB-related (right-upper quadrant or abdominal) symptoms was 9.5 weeks (median, 1 wk; range, 0-60 wk) prior to diagnosis. Lithiasic cholecystitis and/or chronic cholelithiasis was the clinical presentation in 44% of patients, 3,4,11,14,17,19,31,34,38,40,46,48,50,51,61 45.7% presented with acalculous cholecystitis, 1,3,5,12,16,18,20,22,23,28,32,36 -38, 42-48,51,58-60 3.4% with bile duct obstruction, 10,15 and 6.7% did not exhibit abdominal symptoms; GV was discovered because of abnormal GB findings in imaging studies, 9,38 exploratory laparotomy (current study Patient 5), or necropsy. 56 GB-related manifestations were the only expression of GV in 20 (33%) patients.…”

“…9,10,12,37,46,54 GB-SOV was found in 20 (33%) patients and GB-SV in 41 (67%). Forms of SV that affected the GB were PAN (n ¼ 10), 4,12,22,32,38,45,46,51,54 HBV-associated vasculitis (n ¼ 8), 10,11,17,38,46,56,60 cryoglobulinemic (essential or HCVassociated) vasculitis (n ¼ 6), 9,14,19,36,50 MPA (n ¼ 4), 5,31,38,40 EGPA (Churg-Strauss) (n ¼ 4), 20,43,48,61 IgA vasculitis (Henoch-Schönlein) (n ¼ 2), 23,28 giant cell arteritis (n ¼ 1), 47 and vasculitis associated with autoimmune diseases (n ¼ 6) 11,16,18,42,58 (see Table 4). Systemic diseases with associated vasculitis included RA (n ¼ 3), 11,16,18 SLE (n ¼ 2), 42,58 and systemic sclerosis (n ¼ 1).…”

“…10,12,46 Among GB-SV patients, 23 (59%) had a biopsy performed in regions other than the GB [9][10][11]14,19,23,32,36,38,40,42,43,45,46,48,50,51,54,60,61 and 82% of them confirmed vasculitis. SV (including GV) was diagnosed at necropsy in 1 patient.…”

“…Among GB-SOV patients, only 3 received glucocorticoids for 6 to 16 weeks, 15,37 whereas all GB-SV patients were treated with glucocorticoids (p ¼ 0.001),; 4,5,9,10,12,14, 16-18,20,22,23,28,31,32,36,38,40,42,43,46 -48,54,58,60,61 54% of patients with SV also received cytotoxic agents 5,12,14,16,17,22,23,31,36,40,42,46,48,58,60 (see Table 2). Among GB-SV patients with adequate information, 17/20 patients were receiving glucocorticoid therapy at the end of …”

Cholangitis as an Initial Manifestation of Polyarteritis Nodosa

“…Immune complex deposition is considered to be associated with infections, such as hepatitis B, although the exact etiology remains unclear (1)(2)(3).…”