The eye is a common site of granulomatosis with polyangiitis. A collaborative study

Abstract

Background Ocular manifestations of granulomatosis with polyangiitis (GPA) have been reported in a limited number of studies and with largely variable frequency. Here we report on the clinical, diagnostic, and therapeutic features of a cohort of 63 GPA patients, with particular regard to 22 of them with ophthalmic involvement (35%). Methods Clinical manifestations, results of immunological findings, histopathological pictures, imaging data, Birmingham Vasculitis Activity Score, therapeutic regimens, and outcomes were retrospectively analyzed. At diagnosis, in addition to a structured clinical assessment, all patients underwent a comprehensive ophthalmologic examination. Results The most frequently involved organs were kidneys, lungs, ear/nose/throat, and eyes. Ocular manifestations were bilateral in 32%. The three most commonly diagnosed ophthalmologic manifestations were scleritis (36%), retro-orbital pseudotumor or orbital mass (23%), and episcleritis (13%). Ocular and systemic involvement were simultaneously present at onset in 41% of the patients; systemic involvement was followed by ocular lesions in 36%; ocular inflammation was followed by systemic manifestations in 18%; and an orbital mass in the absence of systemic disease characterized 5%. Glucocorticoids plus cyclophosphamide and glucocorticoids plus rituximab were the combined therapies most frequently employed during remission induction and remission maintenance, respectively. Persistent ophthalmologic and extra-ocular remissions were achieved in 77 and 64% of the patients, respectively. One to three systemic relapses were diagnosed in 7 patients (31.8%). At the last follow-up, a visual outcome 20/40 or better in 31 (70%) of 44 eyes was determined. Conclusions The eye was involved in over one third of our patients with GPA. Increased awareness, early diagnosis, and multi-specialty collaboration are critical in achieving a favorable outcome of GPA.

Eyes on the Line: A Case of Ocular Granulomatosis With Polyangiitis

“…However, this is not true [ 10 ]. Most commonly, scleritis or conjunctivitis will be observed and is followed by retro-orbital pseudotumor, keratitis, compressive neuropathy, and retinal vasculitis, along with other manifestations of the disease [ 11 ].…”

Granulomatosis with polyangiitis, complicated by ocular adnexa lesion and vascular changes in retina and choroid (literature review with a clinical case)

“…В таблице 1 представлены данные по частоте различных офтальмологических осложнений ГПА по данным R. Dammacco и соавт. [11].…”

Changes in Lipids in Granulomatosis with Polyangiitis Relates to Glucocorticoids and History of Hypertension

“…Patients with GPA present nonspecific symptoms for weeks or months, such as fever, malaise, myalgias, arthralgias, and weight loss, without evidence of specific organ involvement [6]. Prodromal symptoms are followed by a specific organ involvement due to necrotizing granulomatous inflammation most frequently in the upper (sinusitis, crusting rhinitis, saddle nose deformity, otitis media, mastoiditis, hearing loss) and lower (lung nodules, pulmonary interstitial capillaritis, alveolar hemorrhage) respiratory tract, in small to medium vessels (systemic necrotizing vasculitis), and in the kidney (necrotizing glomerulonephritis) [7,8]. Although the life expectancy and control of symptoms of GPA patients have improved with immunosuppressive therapies [2], atherosclerosis has now emerged as a significant morbidity [9,10], and its progression may lead to the occurrence of atherosclerotic cardiovascular disease (ASCVD).…”