Ocular Manifestations of ANCA-associated Vasculitis

Inflammation can involve several ocular structures, including the sclera, retina, and uvea, and cause vascular changes in these tissues. Although retinal vasculitis is the most common finding associated with uveitis involving the posterior segment, other vascular abnormalities may be seen in the retina. These include capillary nonperfusion and ischemia, vascular occlusions, preretinal neovascularization, microaneurysms and macroaneurysms, and telangiectasia. Moreover, vasoproliferative tumors and subsequent coat-like response can develop secondary to uveitis. Fluorescein angiography is ideal for the investigation of retinal vascular leakage and neovascularization, while optical coherence tomography angiography can provide depth resolved images from the superficial and deep capillary plexus and can demonstrate vascular remodeling. Choroidal vascular abnormalities primarily develop in the choriocapillaris or in the choroidal stroma and can appear as flow void in optical coherence tomography angiography and filling defect and vascular leakage in indocyanine green angiography. Extensive choriocapillaris nonperfusion in the presence of choroidal inflammation can increase the risk of choroidal neovascular membrane development. Iris vascular changes may manifest as dilation of vessels in stroma due to inflammation or rubeosis that is usually from ischemia in retinal periphery secondary to chronic inflammation. More severe forms of scleral inflammation, such as necrotizing scleritis, are associated with vascular occlusion in the deep episcleral plexus, which can lead to necrosis of sclera layer and uveal exposure.

To analyse the ocular manifestations of patients with GPA, their treatment and outcome.

Retrospective cohort study performed at the National Referral Center for Vasculitis, Cochin Hospital, Paris (France), from January 2005 to December 2015. Among 308 patients with a new diagnosis of GPA in accordance with the American College of Rheumatology classification criteria and/or revised Chapel Hill nomenclature definitions, we identified those with ocular involvement and a subsequent follow up in our center.

The prevalence of ocular involvement in our GPA series was 38.6%; 63 patients were analysed with a median follow-up of 50.5 months (IQR: 17.8–82.8). Scleritis (18 patients, 28.6%) and episcleritis (18 patients, 28.6%) were the most common ophthalmologic manifestations, followed by orbital disease (13 patients, 20.6%). Bilateral involvement and visual acuity loss was seen in 29.1% and 16.7% of patients, respectively. Ocular involvement was the first GPA manifestation in 9 patients (14.3%), concomitant with systemic manifestation in 36 (57.1%), and occurred only during follow-up in 18 (28.6%). The indication for GPA treatment was suggested by ocular involvement in 12 patients (19.0%), by systemic features in 40 (63.5%) and by both ocular and systemic involvement in 11 (17.5%). Remission of ocular involvement was achieved in 51 patients (80.9%). In the remaining 12 (19.1%), symptoms persisted or even worsened, finally leading to rituximab (RTX) therapy in 8 of them (66.7%). Altogether, when used as first line or for refractory disease, ocular remission was achieved in 11 of the 12 cases (91.7%) treated with RTX versus 34 of the 47 cases (72.3%) treated with CYC (P = .260). Eye disease relapsed in 14 patients (22.2%). RTX allowed achievement of remission in 8 of them (57.1%). In the remaining six, other immunosuppressive drugs were used.

Scleritis and episcleritis are the most common ocular manifestations in GPA, most of the time associated with other systemic manifestations. In >40% of cases, ocular manifestations were refractory to initial treatment or recurrent and led to RTX prescription, which appeared to be useful in these situations.

Mujer de 43 años, consulta por aumento de volumen orbitario superolateral izquierdo, doloroso, eritematoso de 2 meses de evolución. Se realiza biopsia escisional revelando vasculitis con poliangeítis de glándula lagrimal. El estudio sistémico no reveló compromiso de otros órganos.

El compromiso orbitario ocurre hasta en el 60% de los pacientes con granulomatosis con poliangeítis. La afección de la glándula lagrimal es rara, y frecuentemente unilateral. Las pruebas serológicas generalmente son negativas, tanto en etapas iniciales, como en las formas localizadas de la enfermedad.

A 43 year-old woman consulted due to 2 months of swelling on the superolateral side of the left orbit, with pain and erythema. An excisional biopsy was performed that revealed vasculitis with polyangiitis of the lacrimal gland. A systemic study showed that no other system was compromised.

Orbital involvement occurs in up to 60% of patients with granulomatosis with polyangiitis. The involvement of the lacrimal gland is rare and often unilateral. Serological tests are generally negative, both in initial stages, as in localized forms of the disease.

La granulomatosis con poliangeítis (Wegener) es considerada como una enfermedad granulomatosa sistémica, no infecciosa, caracterizada histológicamente por una vasculitis necrosante de pequeño vaso. El tracto respiratorio superior e inferior son los más frecuentemente afectados, en asociación a manifestaciones renales. Sin embargo, también se describe el compromiso aislado de un solo órgano, como es el caso del globo ocular y la órbita. Presentamos el caso de una mujer cuya principal manifestación consistió en un proceso inflamatorio de tejido periorbitario y proptosis del globo ocular izquierdo. La paciente fue valorada en manejo conjunto con el servicio de plástica ocular, otorrinolaringología y finalmente se derivó a reumatología para inicio de terapia inmunosupresora.

Se realizó una revisión de la literatura, en búsqueda de reportes de caso y series de caso que permitieran comparar las presentaciones clínicas y desenlaces más frecuentes.

Granulomatosis with polyangiitis (Wegener) is considered as a systemic, non-infectious granulomatous disease, histologically noted for its small vessel necrotising vasculitis. The upper and lower respiratory tracts are the most frequently affected, in association with renal manifestations. However, the isolated involvement of a single organ, such as the eyeball and the orbit is also described. The case is presented of a woman whose main manifestation was an inflammatory process of periorbital tissue and proptosis of the left eyeball. The patient was evaluated jointly with eye plastic surgery, and the ear, nose and throat, and rheumatology departments.

A literature review was carried out, looking for case reports and case series that allowed comparisons to made between the clinical manifestations and the most frequent outcomes.