Dacryoadenitis: do not forget ANCA vasculitis

TL;DR

This paper highlights that dacryoadenitis can be a sign of ANCA vasculitis, a rare autoimmune disease, and emphasizes the need for proper screening.

Contribution

The paper presents a rare case of GPA revealed by dacryoadenitis without typical systemic manifestations.

Findings

Dacryoadenitis can be the initial manifestation of GPA without kidney or lung involvement.

Patients with dacryoadenitis may have a better prognosis compared to those with orbital masses.

Pachymeningitis is more common in GPA patients with eye manifestations and is associated with specific ANCA types.

Abstract

Objective: This paper aimed to describe another form of aggressive limited Granulomatosis with polyangiitis (GPA) revealed by dacryoadenitis.

Methods and results: We report an unusually limited GPA in a 48-year-old man presenting with bilateral proptosis. She had never presented kidney or pulmonary manifestations, but her disease was persistently active including oto-rhino-laryngological manifestations, dacryoadenitis, and neurological manifestations unresponsive to corticosteroids and immunosuppressors.

Discussion: Granulomatosis with polyangiitis (GPA) is an auto-immune inflammatory vasculitis. Involvement of lacrimal glands as the first presentation is uncommon. It is characterized by the development of granulomas. Patients with orbital mass without lacrimal gland involvement have a higher rate of systemic disease, a severe clinical course, and a higher rate of recurrences. A patient with dacryoadenitis seems to be with a good prognosis. Eye manifestations were significantly more common in patients with pachymeningitis. MPO-ANCA-positive pachymeningitis was more frequent in older female patients. PR3-ANCA-positive pachymeningitis had more severe neurological damage. Induction treatment consists of intravenous methylprednisolone (IV) associated with cyclophosphamide.

Conclusion: Faced with dacryoadenitis, it is important to screen for ANCA-associated vasculitis.

Abbreviations: GPA = Granulomatosis with polyangiitis, ANCA = Antineutrophil Cytoplasmic Antibodies

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