Eosinophilic granulomatosis with polyangiitis complicated by cholecystitis: a case report and review of the literature

Abstract

The objectives are to report an atypical case of eosinophilic granulomatosis with polyangiitis (EGPA) associated with cholecystitis and to review the main clinical features, therapy, and prognosis of it. We present a 49-year-old male with non-classic clinical manifestations of EGPA and EGPA-related cholecystitis. EGPA was diagnosed by histology of the gallbladder after cholecystectomy. In addition, 11 cases of EGPA-associated cholecystitis have been reported and were described in details in this article. Gallbladder involvement is very uncommon in EGPA. All cases reviewed showed multiple organs involved as well as obviously elevated eosinophilic granulocyte proportion with inflammatory index, although antineutrophil cytoplasmic antibody may be negative. All patients in this cohort that showed gallbladder involvement were eventually confirmed with EGPA by histology examination after cholecystectomy. The pathological change could be infiltration of inflammatory mononuclear cells of small- and medium-sized vessels. Of the cases, 91.7% responded well to steroid and immunosuppressant therapy. Gallbladder involvement is a very rare comorbid condition in EGPA. However, it is an important symptom or secondary condition to alert physicians the diagnosis of EGPA. Moreover, timely diagnosis and correct administration in the early stage of this disease could obviously improve the prognosis.

“Idiopathic Eosinophilic Vasculitis”: Another Side of Hypereosinophilic Syndrome? A Comprehensive Analysis of 117 Cases in Asthma-Free Patients

“…Considering that EoV was diagnosed in the absence of asthma in ANCAnegative HES patients, the CHCC2012 criteria for a diagnosis of EGPA were not met. If we consider our patients in whom blood eosinophilia and extravascular eosinophils were nearly constant features, and the other 1990 American College of Rheumatology (ACR) criteria for EGPA (neuropathy, pulmonary infiltrates and paranasal sinus abnormalities), 8 patients met the 1990 ACR criteria (21,28,67,79,83,88,90)(case #6). Given that granuloma was only reported in three cases of EoV with a systemic presentation, we argue that EoV is mostly a non-granulomatous vasculitis (in contrast to EGPA), and that these asthma-free patients meeting 4 other 1990 ACR criteria might represent exceptions to the CHCC2012 diagnostic classification of EGPA.…”

“…In 6 cases with systemic disease, unexpected histological features of EoV were found in organs that are not typical sites for vasculitic lesions, such as the gallbladder (n=2) (83,84), urethra (n=1) (24), fascia (58), and thymus (17). Unexpected findings were also found in cases of EoV limited to one organ, with one case of EoV associated with prostatitis (15), and one case of eosinophilic fasciitis (86).…”

“…Only one patient of our series (personal case #2) and one patient identified in the literature review had a history of childhood asthma (48). Only 7 patients had a history of chronic sinusitis and/or allergic rhinitis (16,24,58,78,79,83) (case #6). None of the patients in this series was reported as having developed asthma after the diagnosis of EoV.…”

“…Fifteen patients developed other typical clinical manifestations of HES, such as skin lesions (variously described as reddish plaques, erythema, swelling, erythematous papules, subcutaneous nodules, skin rashes, etc.) (48,69,83,84) (personal cases #9 and 10), eosinophilic fasciitis (52,69), arterial thrombosis (in the brain or the digestive tract) (34,46), liver nodules and/or dysfunction (35,57), spleen enlargement and/or infiltration by eosinophils (23,69,74), eosinophilic colitis (83) (case #9), eosinophilic meningoencephalitis (case #7), inflammatory white matter lesions (case #8), and eosinophilic myositis (personal case #8). Biopsies were collected in the great majority of these cases but no features of EoV were found.…”

Eosinophilic Granulomatosis with Polyangiitis Diagnosed by Gallbladder Tissue

“…In eosinophilic granulomatosis with polyangiitis (EGPA), the incidence of gastrointestinal involvement is reported to range from 17% to 59% [1]. Gallbladder involvement is a rare comorbid condition in EGPA [2]. We present an atypical case of EGPA diagnosed on the basis of histological findings of the gallbladder after cholecystectomy.…”