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Updated: Jun 21, 2025

An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis

Published on: February 8, 2019

Dacryoadenitis: do not forget ANCA vasculitis.

Abir Derbel¹, Raida Ben Salah¹, Rihem Boukhzar¹

¹Department of Internal Medicine, Hedi Chaker Hospital, Sfax, Tunisia.

Romanian Journal of Ophthalmology

|July 15, 2024

Summary

Granulomatosis with polyangiitis (GPA) can present unusually with dacryoadenitis, even without kidney or lung issues. Early screening for ANCA-associated vasculitis is crucial when dacryoadenitis is observed.

Keywords:

ANCA vasculitis dacryoadenitis pachymeningitis

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Area of Science:

Rheumatology
Ophthalmology
Neurology

Background:

Granulomatosis with polyangiitis (GPA) is a rare autoimmune vasculitis.
GPA typically affects the respiratory tract and kidneys.
Ocular manifestations, particularly dacryoadenitis, are less common presenting features.

Observation:

A 48-year-old male presented with bilateral proptosis and active oto-rhino-laryngological, dacryoadenitis, and neurological symptoms.
The patient had no history of kidney or pulmonary involvement.
Disease activity persisted despite corticosteroid and immunosuppressor treatment.

Findings:

This case highlights an aggressive, limited form of GPA primarily manifesting as dacryoadenitis.
Lacrimal gland involvement as an initial GPA presentation is infrequent.
Patients with dacryoadenitis as a primary symptom may have a better prognosis compared to those with orbital masses without lacrimal gland involvement.

Implications:

Dacryoadenitis warrants screening for ANCA-associated vasculitis.
Prompt diagnosis and treatment are essential for managing GPA, even in atypical presentations.
Understanding GPA's varied manifestations aids in early detection and management, potentially improving patient outcomes.