Extensive Macular Atrophy With Pseudodrusen associated with Rheumatic Fever

• Grant number: 23/17936-0
• Support Opportunities: Regular Research Grants
• Start date: February 01, 2025
• End date: January 31, 2027
• Field of knowledge: Health Sciences - Medicine - Medical Clinics
• Principal Investigator: Sung Eun Song Watanabe
• Grantee: Sung Eun Song Watanabe
• Host Institution: Escola Paulista de Medicina (EPM). Universidade Federal de São Paulo (UNIFESP). Campus São Paulo. São Paulo , SP, Brazil
• Associated researchers: Guilherme Sobreira Spina ; Janete Maria Cerutti ; Luiz Eduardo Coelho Andrade ; Paula Yuri Sacai Munhoz ; Roney Orismar Sampaio ; Valdir Ambrósio Moises

Abstract

Rheumatic fever is an inflammatory disease that mainly affects children aged 5-15, characterized in the acute phase by polyarthritis, carditis, erythema marginatum, subcutaneous nodules and Sydenham's chorea. Recurrent episodes, which increase the risk of causing or exacerbating heart damage, occur until middle age. Rheumatic heart disease, the most common valvular heart disease in Brazil, is the result of a cross-reaction following pharyngoamygdalitis caused by group A ²-hemolytic Streptococcus in genetically susceptible individuals. The pathogenesis of rheumatic heart disease is not fully understood.The ocular manifestations related to rheumatic fever that have been reported so far are related to the acute phase of the disease, such as anterior uveitis, cystoid macular edema and choroiditis. Recently, a series of cases of degenerative retinopathy was published, called Extensive macular atrophy with pseudodrusen (AMEP), of unknown physiopathogenesis, characterized by the presence of sub-retinal drusiform deposits, macular atrophy and peripheral alteration like paving-stone degeneration. The retinal findings are related to central visual loss, nocturnal visual impairment, altered color vision and visual field loss. Its etiology is still unknown, but studies have shown a higher incidence in industrialized regions or those with agricultural activities, toxic exposure and the patient's inflammatory profile. The aim of the study is to evaluate the association between AMEP and rheumatic fever, given that both diseases have a predisposition and inflammatory profile of the patient as an etiological factor, and to identify possible biological and/or genetic markers. (AU)