Back
Lupus podocytopathy (LP) is a rare glomerular lesion resulting from systemic lupus erythematosus (SLE), however, it is not included in the 6 classes of the International Society of Nephrology/Renal Pathology Society [ISN/RPS]. In general, on renal biopsy, patients with nephrotic syndrome and confirmation of SLE autoantibodies in the blood, present proliferative lupus nephritis (LN) (classes III/IV) or membranous LN (class V) type lesions. Podocytopathies are podocyte lesions that range from minimal lesions (only possible to be visualized under electron microscopy) to severe lesions that lead to glomerular collapse. In the 1980s, there were the first reports of patients with findings compatible with podocytopathies – minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS) – in kidney biopsies performed during the course of the evolution of SLE with suspected LN. However, only from 2002 onwards, a series of 18 cases showed the entity in a more robust form, including immunofluorescence findings that would become more typical of LP [no subepithelial or subendothelial deposits were described, but 44% of patients (8 of 18 cases) had mesangial deposits concurrent with a class I or class II LN]. In 2016, a cohort with 50 cases with a similar clinical profile (nephrotic syndrome in a patient with confirmed SLE who underwent renal biopsy with findings compatible with podocyte injury – MCD or FSGS), confirmed mesangial immune deposits by immunofluorescence and electron microscopy, improving our understanding and recognition of lupus podocytopathy as a distinct entity. Today it is believed that LP affects approximately 1% of individuals with nephrotic proteinuria due to SLE.
Data information was obtained through medical record review and literature review.
We present the case of a woman, who initially did not have a formal diagnosis of SLE, but had a history of positive antinuclear factor (ANA) for many years and a positive family history of SLE (sister and mother diagnosed with SLE, on immunosuppressive therapy). The patient manifested nephrotic syndrome at 28 years of age and, in the kidney biopsy, FSGS was identified, but with immunofluorescence showing deposits of immune complexes in mesangial regions (IgG, C3, and kappa and lambda light chains), characterizing LP. The patient received corticosteroid pulse therapy. As the initial condition worsened quickly, she required hemodialysis for 1 week. She presented a satisfactory response to pulse therapy, making it possible to suspend hemodialysis. Treatment with oral corticosteroid therapy continued, but the patient developed corticosteroid dependence, and it was decided to combine cyclosporine and hydroxychlorquine. After this association, complete weaning of corticosteroids was possible within 16 weeks and subsequently, with complete remission of proteinuria and nephrotic syndrome, cyclosporine was weaned 6 months after the start of treatment. The patient remained only on hydroxychloroquine.
Conclusions
There are still few studies with conclusive information on the most appropriate treatment for LP. We consider that this report contributes to advancing understanding of the disease, its diagnosis and treatment. The entity is becoming increasingly known and elucidated, however, more studies are needed to elucidate the points that remain unclear.