Optic Neuropathy Associated With Periostitis in Relapsing Polychondritis

Abstract

Optic neuropathy is an uncommon manifestation of relapsing polychondritis (RPC), a rare systemic disease affecting cartilaginous and proteoglycan-rich structures. The optic neuropathy has been attributed to ischemia, intrinsic inflammation of the optic nerve, or spread of inflammation to the nerve from adjacent intraconal orbital tissues. We report a case of recurrent corticosteroid-responsive optic neuropathy in which MRI did not show ocular, optic nerve, or intraconal orbital abnormalities but did show periosteal thickening and enhancement in the apical orbit and adjacent intracranial space consistent with periostitis. The periostitis, which is a manifestation of a systemic vasculitis or an autoimmune reaction to progenitors of cartilage, probably caused the optic neuropathy by compression or inflammation. It is important to recognize this mechanism of optic neuropathy as its imaging features may be a subtle yet critical clue to an underlying systemic condition that can be life-threatening if not properly managed.

Severe visual impairment and subclinical encephalitis preceding clinical signs of chondritis in relapsing polychondritis

“…Severe visual impairment is also rare in RP but can be due to ischemic optic neuropathy, optic neuritis, or spread of inflammation to the optic nerve from adjacent orbital tissues . In our case, the inflammation of posterior sclera had most likely spread to the optic nerve, causing severe papilledema and visual impairment.…”

“…1,2 Although scleritis is a common complication, severe visual impairment is also rare in RP. 3 We herein report a case of RP with severe visual impairment and asymptomatic brain inflammatory lesions preceding clinical signs of chondritis.…”

“…Central nervous system (CNS) involvement is rarely reported in RP, where neurological symptoms develop after clinically evident chondritis and systemic inflammation . Although scleritis is a common complication, severe visual impairment is also rare in RP …”

Isolated unilateral disk edema

“…175 Associated bilateral disk edema has been reported with the condition, 30,176,177 but no cases of UDE were discovered for this review. Thrombocytopenia Thrombocytopenia (low platelets) has been linked to bilateral disk edema 178 and disk edema in conjunction with other Neonatal onset multisystem inflammatory disease (NOMID) 294,295 Relapsing polychondritis 296 Systemic lupus erythematosus 297,298 Local vasculitides Optic disk edema with macular star (ODeMS) 299,301 Optic disk vasculitis (ODV) 302,304 Sarcoidosis (no optic nerve head granulomas) [9][10][11]311,312 Other biochemical alterations in cells Decreased nitric oxide concentration Hypercholesterolemia 242…”

Visual loss in relapsing polychondritis: rapid progression of orbital inflammation

“…Hirunwiwatkul et al reported that there had been 25 previous reported cases of optic neuropathy (ON) in RPC, 4 cases as ischaemic, 13 as inflammatory and 8 of indeterminate mechanism. They presumed that vision should be improved after anti‐inflammatory treatment in inflammatory ON and not in ischaemic ON.…”