Histopathological Features Of Orbital Biopsies Associated With The Clinical Diagnosis Of Wegener’s Granulomatosis

Abstract

Purpose: : Wegener’s granulomatosis (WG) is a presumed autoimmune disease characterised by granulomatous inflammation, small vessel vasculitis and necrotising glomerulonephritis. Limited WG primarily involves the head and neck region, including the orbit, and is often difficult to diagnose, owing to the lack of classical features of WG and sensitivity of anti-neutrophil cytoplasm antibodies (ANCA). The purpose of this study was to examine orbital biopsy tissue and determine factors predictive of a clinical diagnosis of WG.

Methods: : A retrospective study of orbital biopsies with a diagnosis of OID was undertaken using the Institute of Ophthalmology Pathology Department database. 243 biopsies were identified over a period of 15 years. Ethics approval is obtained from the Moorfield & Whittington Research Ethics Committee (REC ref. no. 09/H0721/75, LIGS 1023)

Results: : Of the 234 patients, 43 (18%) were WG and 191 (82%) were non WG. The mean age was 50 years in both groups and both showed a female preponderance (F:M=3:2). The majority of biopsies were obtained from orbital masses (45%). Histology showed a range of acute and chronic inflammatory pictures in both WG and non-WG groups. The presence of neutrophils (p=<0.001), eosinophils (p<0.001), vasculitis (p=<0.001), histiocytes (p=0.04) and nuclear dust (p=0.03) were significantly associated with a clinical diagnosis of WG. In a multivariate analysis, only neutrophils (OR=3.6,p=0.01), histiocytes (OR=2.9,p=0.02) and vasculitis (OR=2.6, p=0.02) were independently associated with the clinical diagnosis of WG. Interestingly, and in contrast to previous reports, eosinophils and necrosis were not significantly associated with a clinical diagnosis of WG.

Conclusions: : Some histopathological features and infiltrating cell types present in orbital biopsies are significantly associated with the clinical diagnosis of WG. These features may be helpful in the establishing the diagnosis of limited WG, particularly in the absence of raised serum ANCA.

Keywords: pathology: human • orbit • inflammation