Acute choroidal involvement in lupus nephritis: A case report and review of literature

Abstract

BACKGROUND Systemic lupus erythematosus (SLE), characterized by the production of autoantibodies and widespread deposition of immune complexes, predominantly affects women of childbearing age. More than one-third of SLE patients present ocular manifestations. Choroidal disease is currently not completely understood, and its precise differentiation from central serous chorioretinopathy is rarely achieved. To date, no more than 60 patients with choroidal involvement have been reported. CASE SUMMARY A 37-year-old Chinese woman experienced decreased visual acuity bilaterally, accompanied by increasing periorbital swelling and severe conjunctival chemosis. Decreased breath sounds in both bases were detected via auscultation, as well as pitting edema in both ankles. SLE and lupus nephritis were diagnosed based on serositis, renal disorder, leukopenia and positive anti-Smith and anti-nuclear antibodies. Lupus choroidopathy was diagnosed based on ocular presentation and imaging. The patient was treated with systemic corticosteroids, spironolactone, hydroxychloroquine (HCQ), mycophenolate mofetil (MMF), and intravenous immunoglobulin. After 4 wk of hospitalization, the patient was discharged. Indocyanine green angiography showed no leakage from choroidal vessels, and ocular coherence tomography detected low amounts of subretinal fluid right before discharge. The patient was prescribed oral methylprednisolone, HCQ, and MMF. Two months after the first visit, ophthalmological examination revealed a visual acuity of 20/20 bilaterally, and SLE disease activity was well controlled; her symptoms disappeared completely. CONCLUSION Here we presented a case of lupus choroidopathy, successfully treated with systemic corticosteroids, and discussed previously reported cases, focusing on differential diagnosis with a central serous chorioretinopathy.

Subretinal fluid in a patient with systemic lupus erythematosus

“…Lupus choroidopathy is a rare ocular manifestation of SLE that has been reported in no more than 60 patients up to 2019. [ 14 ] It is observed in patients with severe active or hypertensive SLE; therefore, it is considered an indicator of SLE activity. [ 4 ] Lupus choroidopathy is also associated with lupus nephritis because of the similar structure and pathogenesis of both the kidney and choroid.…”

“…reported that lupus nephritis was the most prominent comorbidity of lupus choroidopathy, occurring in approximately 78.6% of all patients. [ 14 ] In lupus nephritis, systemic hypertension is usually observed and exacerbates lupus choroidopathy because it contributes to choroidal vessel occlusion, which promotes choroidal ischemia and destruction of the blood–retinal barrier at the RPE. [ 16 17 ]…”

“…[ 21 ] Therefore, these various causative factors lead to RPE dysfunction, and accumulation of fluid in the subretinal space in patients with lupus choroidopathy. [ 14 ]…”

“…[ 14 ] Ocular manifestations associated with lupus choroidopathy include serous or exudative retinal detachment, serous detachment of the sensory retina, detachment of the RPE, retinal pigment epitheliopathy, clumping, and atrophy. [ 4 14 ] Fluorescein leakage, delayed choroidal perfusion, and choriocapillary nonperfusion have been observed as angiographic findings in lupus choroidopathy. [ 4 14 ]…”

“…[ 4 14 ] Fluorescein leakage, delayed choroidal perfusion, and choriocapillary nonperfusion have been observed as angiographic findings in lupus choroidopathy. [ 4 14 ]…”