Single-Organ Gallbladder Vasculitis

Abstract

Systemic vasculitis (SV) involving abdominal structures usually has a poor prognosis. Gallbladder vasculitis (GV) has been reported as part of SV (GB-SV) and focal single-organ vasculitis (GB-SOV). We analyzed clinical and histologic characteristics of patients with GV to identify features that differentiate GB-SOV from the systemic forms of GV. To identify affected patients with GV we used pathology databases from our institution and an English-language PubMed search. Clinical manifestations, laboratory and histologic features, treatment administered, and outcomes were recorded. Patients were divided in 2 groups, GB-SOV and GB-SV. As in previous studies of single-organ vasculitis, GB-SOV was only considered to be a sustainable diagnosis if disease beyond the gallbladder was not apparent after a follow-up period of at least 6 months. Sixty-one well-characterized patients with GV were included (6 from our institution). There was no significant sex bias (32 female patients, 29 male). Median age was 52 years (range, 18–94 yr). GB-SOV was found in 20 (33%) and GB-SV in 41 (67%) patients. No differences were observed in age, sex frequency, or duration of gallbladder symptoms between groups. Past episodes of recurrent right-upper quadrant or abdominal pain and lithiasic cholecystitis were more frequent in GB-SOV patients, whereas acalculous cholecystitis occurred more often in GB-SV. In GB-SV, gallbladder-related symptoms occurred more often concomitantly with or after the systemic features, but they sometimes appeared before SV was fully developed (13.5%). Constitutional and musculoskeletal symptoms were reported only in GB-SV patients. Compared to GB-SOV, GB-SV patients presented more often with fever (62.5% vs 20%; p = 0.003) and exhibited higher erythrocyte sedimentation rate levels (80 ± 28 vs 37 ± 25 mm/h, respectively; p = 0.006). All GB-SV patients required glucocorticoids and 50% of them also received cytotoxic agents. Mortality in GB-SV was higher than in GB-SOV (35.5% vs 10%; p = 0.05). Nongranulomatous inflammation with fibrinoid necrosis of medium-sized vessels occurred equally in both groups (>90%). Forms of SV affecting the gallbladder included polyarteritis nodosa (n = 10), hepatitis B virus-associated vasculitis (n = 8), cryoglobulinemic (essential or hepatitis C virus-associated) vasculitis (n = 6), vasculitis associated with autoimmune diseases (n = 6), microscopic polyangiitis (n = 4), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (n = 4), IgA vasculitis (Henoch-Schönlein) (n = 2), and giant cell arteritis (n = 1).GV is uncommon. Its histology most often consists of a nongranulomatous necrotizing vasculitis affecting medium-sized vessels. GB-SOV is usually discovered after routine cholecystectomy performed because of the presence of local symptoms, gallstone-associated cholecystitis, and contrary to GB-SV, GB-SOV is usually not associated with systemic symptoms. Acute phase reactants and surrogate markers of autoimmunity are usually normal or negative in GB-SOV. GB-SOV does n...

Association between Hashimoto’s thyroiditis and cholelithiasis: a retrospective cohort study in Taiwan

“… 36–38 Finally, in addition to hypothyroidism, HT-associated vasculitis or oxidative stress could predispose to the development of cholelithiasis. 39 40 The use of thyroxine does not seem to ameliorate the increased incidence of cholelithiasis for HT since patients with HT with (aHR=1.94, 95% CI 1.51 to 2.49) and without (aHR=1.95, 95% CI 1.53 to 2.49) thyroxine treatment were similarly associated with a higher risk of cholelithiasis than those in the non-HT cohort and their aHRs were comparable ( table 4 ). These findings support that the association between HT and cholelithiasis might not simply be relevant to the thyroid function.…”

Eosinophilic granulomatosis polyangiitis associated with acute acalculous cholecystitis

“…2 Systemic vasculitides, including EGPA, polyarteritis nodosa, immunoglobulin A vasculitis, cryoglobulinaemic vasculitis and giant cell arteritis may predispose individuals to AAC. 3 Organ hypoperfusion is implicated as a pathogenetic contributor to AAC. 4 Laboratory tests in patients with AAC show non-specific results and include elevated serum bilirubin, alkaline phosphatase and aminotransferases.…”

Gallbladder Vasculitis: An Unexpected and Puzzling Finding

“…Gallbladder vasculitis is an unusual condition. 1,2 It may present either as single-organ vasculitis or as part of systemic vasculitis (mainly polyarteritis nodosa and hepatitis B virus-associated vasculitis). 1,2 Diagnosis of gallbladder single-organ vasculitis is made when vasculitis is limited to gallbladder in the absence of systemic disease over a minimum follow-up interval of 6 months.…”

“…1,2 It may present either as single-organ vasculitis or as part of systemic vasculitis (mainly polyarteritis nodosa and hepatitis B virus-associated vasculitis). 1,2 Diagnosis of gallbladder single-organ vasculitis is made when vasculitis is limited to gallbladder in the absence of systemic disease over a minimum follow-up interval of 6 months. 1 However, we did not make a definite diagnosis of gallbladder single-organ vasculitis in our patient because persistent eosinophilia after cholecystectomy is an unusual finding for such a condition.…”

“…1,2 Diagnosis of gallbladder single-organ vasculitis is made when vasculitis is limited to gallbladder in the absence of systemic disease over a minimum follow-up interval of 6 months. 1 However, we did not make a definite diagnosis of gallbladder single-organ vasculitis in our patient because persistent eosinophilia after cholecystectomy is an unusual finding for such a condition. Although vasculitic involvement of gallbladder appears similarly as lithiasic or acalculous cholecystitis, the last one is more prevalent in patients suffering from systemic vasculitis.…”