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Allergic granulomatosis and angiitis, also known as Churg-Strauss syndrome (CSS) is predominantly a disease of adults.1 Ocular involvement is rare.2 We describe a case of CSS in a child that resulted in bilateral optic neuropathy with vasculitis and multiple branch retinal artery occlusions affecting the macula.
A 10 year old African-American girl developed sudden painless loss of vision in both eyes over 2 days. The child was an inpatient admitted for examination of a multisystem disorder, affecting her pulmonary, gastrointestinal, muscular, and renal systems. On examination the best corrected vision was hand movement in both eyes. Confrontation visual fields were full bilaterally. Pupils were normal, without an afferent papillary defect. Anterior segment examination was completely normal. Posterior segment examination revealed massive retinal opacities in the papillomacular bundle extending into the peripapillary region bilaterally (fig 1). There was pronounced macular oedema and sludging in the venous system. There were extensive scattered intraretinal haemorrhages bilaterally.
Fluorescein angiogram demonstrated bilateral blocking defect caused by retinal opacification and haemorrhages and non-perfusion in the macular region (fig 2). There was also pruning and non-perfusion of both small retinal capillaries and choroidal vessels. The retinal and arterioles venules demonstrated staining and leakage. Both optic discs stained with fluorescein.
The patient had patchy pneumonitis and asthma, hypertension, acute renal failure, elevated liver enzymes, and eosinophilia of 40%. Renal biopsy showed non-specific glomerular inflammation, but no eosinophilic infiltration or granulomata. A magnetic resonance image of the liver showed no parenchymal damage. An infectious examination was negative for tuberculosis, Lyme, cat scratch disease, syphilis, HIV, cytomegalovirus, Epstein-Barr virus, toxoplasmosis, or toxocariasis. An oncological examination was negative. ANA, anti-dsDNA, ANCA, complement levels C3, C4, and CH50 were all normal. The diagnosis of Churg-Strauss syndrome was made based on three of six criteria advanced by the American College of Rheumatology,3 which include asthma, hypereosinophilia of >10%, non-fixed pulmonary infiltrates.
The patient was started on high dose intravenous steroids (2 mg/kg/day) and cyclophosphamide. Intravitreal triamcinolone acetomide injection was perfomed in the right eye with improvement of macular oedema. However, the vision remained no better than 20/400 in both eyes at 1 month.
CSS is a systemic granulomatous necrotising vasculitis affecting small to medium-sized vessels. Takanashi et al2 classified ocular manifestations into two groups: pseudotumour-type and ischaemic vasculitis-type.4,5 Pure ischaemic vasculitis-type manifestations were associated with a sudden onset loss of vision, a quiet looking eye, lack of computed tomography (CT) abnormalities, and a positive ANCA.2 In contrast, pseudotumour-like manifestations involved a chronic onset, a red eye, orbital abnormalities on CT, and a negative ANCA.1 Takanashi et al advised that patients with positive ANCA CSS, who are visually asymptomatic, be carefully examined and treated prophylactically with high dose steroids in order to prevent potentially irreversible visual loss from the ischaemic-type ocular manifestations.2 The devastating loss of vision in this child with ANCA negative CSS suggests that all patients with CSS should be considered for prophylactic high dose corticosteroids, regardless of their ANCA status.
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