Severe ophthalmic involvement in granulomatosis with polyangiitis: a case series
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Yes
Purpose
To evaluate clinical characteristics, management and outcomes of patients with severe ophthalmic involvement associated with granulomatosis with polyangiitis (GPA) followed at a uveitis referral center
Methods
Retrospective chart review of clinical/laboratorial data and of multimodal imaging
Results
We identified 11 patients with severe ophthalmic manifestations associated with GPA, aged 28–61 years. Bilateral involvement was seen 64%. Ocular manifestations included necrotizing scleritis (55%), anterior scleritis (36%), posterior scleritis (9%), peripheral ulcerative keratitis (PUK, 27%), dacryocystitis, and panuveitis (27%). In 10 patients, suggestive ocular findings were the initial clue to the diagnosis of GPA. Complications included retinal detachment (4 eyes), optic nerve atrophy (3 eyes), and severe visual impairment (NPL in 3 eyes/2 patients). Systemic manifestations were prominent, with recurrent sinusitis (92%), otitis/mastoiditis (45%), pulmonary involvement (55%), renal disease (27%) and mononeuritis multiplex (9%). Laboratory results revealed c-ANCA/PR3 positivity in 10 patients. Aggressive treatment included methylprednisolone pulse, cyclophosphamide pulse, antimetabolites, cyclosporine, rituximab and prednisone (100%), leading to control of ocular inflammation and partial/complete remission in all cases. Visual impairment was associated with severe disease activity/delayed diagnosis
Conclusion
Ophthalmic examination is important in GPA, as ocular disease may be its first manifestation. Severe ocular findings often correlate with systemic disease activity, underscoring the importance of timely recognition and multidisciplinary aggressive management to improve outcomes and prevent irreversible visual loss
Conflict of interest
No
1
Last name
GUIMARãES SILVA
Initials of first name(s)
M
Department
Hospital São Geraldo/HC da Universidade Federal de Minas Gerais (UFMG)
City
Belo Horizonte
Country
Brazil
2
Last name
de Oliveira Machado Azevedo
Initials of first name(s)
D
Department
Hospital São Geraldo/HC da Universidade Federal de Minas Gerais (UFMG)
City
Belo Horizonte
Country
Brazil
3
Last name
Christina Higino Rocha
Initials of first name(s)
A
Department
Hospital São Geraldo/HC da Universidade Federal de Minas Gerais (UFMG),
City
Belo Horizonte
Country
Brazil
4
Last name
Augusto Tanure
Initials of first name(s)
L
Department
Hospital São Geraldo/HC da Universidade Federal de Minas Gerais (UFMG)
City
Belo Horizonte
Country
Brazil
5
Last name
Vitor Vasconcelos-Santos
Initials of first name(s)
D
Department
Hospital São Geraldo/HC da Universidade Federal de Minas Gerais UFMG/ Faculdade de Medicina da UFMG
City
Belo Horizonte
Country
Brazil
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