Answer to the last #DiagnosticDilemma
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The answer to the last Diagnostic Dilemma (A 40-year-old woman presented with retinal lesions found by an optometrist. Fundus examination showed drusen-like deposits in the macula and the periphery of the retina.) is C) Dense deposit disease related drusen

A 40-year-old woman was referred to the clinic by her optometrist because she had lesions on her retina suggestive of age-related macular degeneration (AMD). She was otherwise well: with no systemic or visual symptoms. At the age of 10 years, she had developed end-stage renal disease—initially diagnosed as post-streptococcal glomerulonephritis—which eventually resulted in her having a right kidney transplant when she was 22 years old. 6 years later, aged 28 years, she developed signs of rejection and required hospitalisation. At that stage, a kidney biopsy led to a diagnosis of dense deposit disease (DDD). She received a second kidney transplant at age 39 years. She had been prescribed a combination of immunosuppressive drugs—prednisolone, mycophenolate, and tacrolimus—for the past 2 years, and her blood pressure had remained stable without pharmacotherapy.

On examination, her best-corrected visual acuity was 20/25 in the right eye and 20/20 in the left eye. Fundus examination showed drusen-like deposits in the macula and numerous lesions in the peripheral retina of both eyes. Optical coherence tomography showed subretinal dome-shaped deposits— known as drusen—in the peripheral retina, with some encroaching in the macular region of both eyes. Drusen is the plural form of druse, a German word meaning node or geode. Considering the situation in the round, including the patient’s age and medical history, and the appearance and distribution of the fundus lesions, it was concluded that the drusen were a feature of DDD rather than AMD. The patient continues to be followed up by both her nephrologist and ophthalmologist. DDD, which was previously known as type II membranoproliferative glomerulonephritis, is classified as either complement or immune complex mediated; DDD is considered a subtype of complement C3- mediated glomerulopathy. Patients with DDD have diffuse, dense deposits in the glomerular basement membrane, and unlike other forms of membranoproliferative glomerulonephritis, they also typically have drusen deposits in Bruch’s membrane of the eye. It is a rare disease that usually presents in children aged 5–15 years old and eventually leads to end-stage renal disease.

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Source: The Lancet
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