Atypical central retinal artery occlusion: an uncommon cause of retinopathy and visual loss in dermatomyositis

Abstract

Several ophthalmic manifestations in patients with dermatomyositis have been described but involvement of the retina is rare. A 39-year-old man diagnosed with dermatomysitis had developed sudden painless visual loss in the left eye. He was non-diabetic and non-hypertensive. He was on treatment with systemic prednisolone (50 mg/day) and methotrexate (7.5 mg/day) for dermatomyositis. His visual acuity was finger counting at 2 feet in the left eye and 6/6 in the right eye. Left fundus showed slight blurring of the optic disc margins, large peripapillary flame-shaped haemorrhages and extensive area of opacification suggestive of central retinal artery occlusion (CRAO) with sparing of the area supplied by the cilioretinal artery. The exact pathomechanism of CRAO in dermatomyositis remains poorly elucidated. The infarctive events following endothelial damage and platelet thrombi occurring in arterioles and capillaries of retina, optic disc and choriocapillaries causing ischemia/infarction have been suggested as possible reasons. However, central artery occlusion from high intraocular pressure from marked (heliotrope) orbital edema appears more plausible in our case. The degree of retinal damage is variable when CRAO lasts between 105 and 240 min and irreversible profound visual loss may occur from its occlusion lasting for more than 240 min. A prompt ophthalmological opinion/management is recommended to limit its progression.

Perimysial microarteriopathy in dermatomyositis with anti‐nuclear matrix protein‐2 antibodies

“…Multisystem vasculopathy in DM comprises myocardial infarction, ischaemic stroke , renal infarction , retinopathy , digital infarcts and microinfarcts in the muscles , indicating the involvement of vessels larger than capillaries. The significantly higher frequency of microinfarcts in edematous (80%) than in non‐edematous (34%) adult DM suggests a prominent role of ischaemia and vasculopathy in the underlying edema .…”

Optical coherence tomography angiography in Purtscher-like retinopathy associated with dermatomyositis: a case report

“…DM has also been associated with other retinal pathology, such as central retinal artery occlusion [10], central retinal vein occlusion [11], and optic neuropathy [12]. In one report of 43 patients with polymyositis and DM, only six (14%) had retinopathy [13].…”

Observation on therapeutic efficacy of rt-PA intravenous thrombolysis combined with compound anisodine injection on central retinal artery occlusion

“…In recent years, through clinical research covering treatment of acute cerebral infarction, myocardial infarction and acute retinal central artery occlusion in China and other countries ( 24 ), it was verified that rt-PA intravenous thrombolysis could significantly improve blood supply to acute ischemic cerebral and cardiac tissues and narrow the range of infarction so that it could reduce the disability rate and death rate caused by acute cardiac and cerebral infarction. Findings showed that rt-PA intravenous thrombolysis could improve patients' visual acuity by at least 3 lines within 24 h since the attacks of symptoms, and effectively improve as well as store their intraocular perfusion and visual function ( 27 ). In the present study, 12 cases of CRAO patients having attacks within 24 h were treated by intravenous medicine administration with rt-PA (0.9 mg/kg, the highest dosage being 90 mg).…”