Nasal and Lacrimal Sac Histopathology in Patients With Granulomatous Polyangiitis Undergoing Lacrimal Drainage Surgery.

PURPOSE: To examine histological changes within nasal and lacrimal sac mucosa harvested from patients with granulomatous polyangiitis (GPA) who required lacrimal drainage surgery. As these patients were considered quiescent on clinical and serological grounds, particular attention was paid to the presence of active vasculitis.

DESIGN

Retrospective noncomparative case series.

PARTICIPANTS

Patients with GPA who had open lacrimal drainage surgery under the care of a single surgeon between 1991 and 2017. All patients had paired biopsies of both nasal and ipsilateral lacrimal sac mucosa.

METHODS

Biopsies of the nasal and lacrimal sac mucosa, taken from the operative site during either primary or revisional open dacryocystorhinostomy, were reported by experienced ophthalmic histopathologists. Outcome measures were the surgical appearance of the tissues and the proportion of each tissue showing histological evidence of active vasculitis, chronic inflammation with or without fibrosis, marked fibrosis without inflammation, or acute-on-chronic inflammatory changes.

RESULTS

Paired tissues were available from 47 patients (25 females; 53%) who had undergone 64 procedures (57 primary, 7 revisional). All patients had systemic disease, this being known prior to surgery in 43 patients (with 41/43 on long-established systemic immunosuppression) and suspected clinically after the initial lacrimal examination in 4/47. Most lacrimal sacs and nasal spaces showed a variety of abnormalities, this commonly being extensive scarring. Despite most patients being considered to have inactive systemic GPA, active vasculitis was evident in 23/64 (36%) lacrimal sac biopsies and 32/64 (50%) nasal mucosal biopsies-suggesting that serological markers may not be reliable guide to disease activity where disease is of limited volume (as, for example, with limited sino-nasal disease). Chronic lymphocytic inflammation was present in a further 35/64 (55%) sacs-associated with marked fibrosis in 5-and a further 25/64 (41%) nasal biopsies (with fibrosis in 2).

CONCLUSIONS

The lacrimal sac and nasal space are highly abnormal in most patients with GPA undergoing lacrimal drainage surgery. Despite having clinically and serologically inactive disease on established systemic immunotherapy, histological examination often shows unrecognized active vasculitis, which might indicate inadequate systemic immunosuppression for the underlying condition.Lacrimal sac and nasal mucosa from patients with granulomatous polyangiitis undergoing dacryocystorhinostomy usually show chronic lymphocytic inflammation with marked fibrosis, but up to half reveal active vasculitis despite long-established systemic immunosuppression and apparently "quiescent" disease.