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PARACENTRAL ACUTE MIDDLE MACULOPATHY ASSOCIATED WITH EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS
Antony C Boynes1, Thomas J Gin, Trevor Gin
1Department of Ophthalmology, Royal Victorian Eye and Ear Hospital, 32 Gisborne St, East Melbourne, VIC, Australia.
Purpose:
To present a case of paracentral acute middle maculopathy (PAMM) associated with transient loss of vision from eosinophilic granulomatosis with polyangiitis (EGPA)-associated ischemic vasculitis.
Methods:
Case report and review of the literature.
Results:
A 52-year-old man presented with recurrent episodes of amaurosis of the right eye. A diagnosis of EGPA was established with the combination of late-onset asthma, peripheral eosinophilia, patchy parenchymal consolidation, and diffuse pansinusitis. Fundus examination displayed cotton wool spots in the right eye. Optical coherence tomography (OCT) of the macula showed features of paracentral focal inner nuclear layer/outer plexiform layer hyperreflectivity consistent with PAMM. He was treated with corticosteroids and cyclophosphamide. At 2 months following the treatment, his EGPA was in remission; the patient reported no recurrence of visual symptoms. Dilated fundoscopy revealed resolution of the cotton wool spots. A repeat OCT of the macula showed resolution of PAMM.
Conclusion:
This case supports sublethal hypoxia of the middle retinal layers as the underlying mechanism for PAMM. Ophthalmologists should be cognizant to look for PAMM on OCT in patients presenting with transient visual loss and be aware of EGPA-associated ischemic vasculitis as a rare cause of amaurosis.
Insights
This case highlights paracentral acute middle maculopathy (PAMM) in a patient with eosinophilic granulomatosis with polyangiitis (EGPA)-associated ischemic vasculitis. Prompt treatment led to resolution of both conditions and visual recovery.
Area of Science:
- Ophthalmology
- Rheumatology
- Vasculitis
Background:
- Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis.
- Ocular manifestations of EGPA can include ischemic events affecting the retina.
- Paracentral acute middle maculopathy (PAMM) is characterized by focal damage to the inner retinal layers.
Observation:
- A 52-year-old man with EGPA experienced recurrent transient vision loss (amaurosis).
- Fundus examination revealed cotton wool spots, and OCT showed PAMM.
- EGPA diagnosis was confirmed by asthma, eosinophilia, and systemic findings.
Findings:
- Corticosteroids and cyclophosphamide effectively treated the EGPA and resolved the ocular symptoms.
- Optical coherence tomography (OCT) demonstrated resolution of PAMM after treatment.
- The case supports sublethal hypoxia of retinal layers as the mechanism for PAMM.
Implications:
- Ophthalmologists should consider PAMM on OCT in patients with transient vision loss.
- EGPA-associated ischemic vasculitis is a rare but important cause of amaurosis.
- Early diagnosis and treatment of EGPA can prevent severe visual impairment.
