PURPOSE: To report a case series of two cases of granulomatosis with polyangiitis, previously known as Wegener granulomatosis, which developed macular necrosis, not previously associated with granulomatosis with polyangiitis, healed with fibrosis, despite aggressive immune-modulating therapy and good control of systemic disease.
METHODS: Case series of two cases with observation of treatment progress.
RESULTS: The results reported the progress of response to treatment in the two cases, which resulted in the final outcome of fibrosis in the macula region, despite being on aggressive immune-modulating therapy and good systemic control.
CONCLUSION: Granulomatosis with polyangiitis can be associated with macular necrosis leading to fibrosis, unresponsive to immune-modulating therapy.
Systemic lupus erythematosus (SLE) is a common multisystem disorder. However, retinal vasculitis as a primary manifestation of SLE is uncommon, accounting for only 4% of causes of retinal vasculitis. The postulated mechanism appeared to be vaso-occlusion of the retinal arterioles by thrombosis, with resultant ischaemia. Optic neuropathy in SLE is also rare, with a prevalence of 1%. This is a case report of a young lady who presented to us with retinal vasculitis as her initial presentation of SLE. Interestingly, the pathologic mechanism appeared to be inflammatory and not vaso-occlusive.