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Lupus podocytopathy (LP) is an unusual entity in patients with systemic lupus erythematosus (SLE) with nephrotic syndrome. It is characterized by the effacement of podocyte processes, absence of immune complex deposition or confined to mesangium. Findings that, from a histopathological point of view, do not fit within the current classification system of lupus nephritis. Rather, it simulates what we know as minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS). Information about LP in Latin America is very limited. Considering how unusual but important this entity is, this study aims to describe the clinical, histopathological characteristics, and outcome of 4 patients diagnosed with lupus podocytopathy.
This is a retrospective study, where we describe a series of 4 cases of LP, using information from medical records of the Hospital Metropolitano de Santiago (HOMS), Dom Rep, from November 2010 to June 2023.
The diagnosis of LP can be challenging not only because it’s rare, affecting a small percentage of the population, but also because of its similarity to other nephrotic syndromes. A challenge that increases in difficulty, especially when the SLE is silent in these patients, simulating or pretending to be other glomerulopathies until a new FLARE or reactivation of the disease.
In our study, all patients showed a histopathological pattern that resembles primary focal and segmental glomerulonephritis, which at a therapeutic level did not respond to monotherapy with glucocorticoids, requiring other immunosuppressive agents. This was similar to other studies carried out in developed countries, in which this pattern, compared to MCD, tends to present with a higher rate of kidney injury and less sensitivity to glucocorticoid monotherapy. Despite our results, research is needed with a larger population where we can estimate and compare the frequency, clinical characteristics and treatment of this condition according to the morphological model that is present. Likewise, it would be optimal to include LP within the next pathological classification of lupus nephritis.