AB0160 LUPUS PODOCYTOPATHY-CASE REPORT
Authors: Hana Alahmari
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Lupus podocytopathy is a recently recognized new entity of lupus nephritis characterized by diffuse foot process effacement with or without mesangial expansion. Capillary wall immune deposits and glomerular proliferation are lacking in the histopathological analysis. (1) Clinically, it is manifesting as nephrotic proteinuria in the majority of reported cases.(1) Nevertheless, there is insufficient knowledge about the epidemiology, precipitant etiology and choices of management. Here, we describe a case presented with significant proteinuria secondary to foot process effacement with the absence of immunocomplement deposition or glomerular inflammation. <h3>Objectives</h3> To describe unusual presentation of proteinuria in lupus patient secondary to podocyte effacement which is unusual underlying pathology. <h3>Methods</h3> 45 years old female diagnosed to have SLE 14 years back in the setting of malar rash, alopecia, arthritis and positive serology (ANA, Ds DNA). She was doing fine on HCQ till a years ago when she presented to the clinic with gradual onset lower limbs swelling. Laboratory results showed a significant proteinuria 2781 mg/dl/day, urine analysis showed RBC 1-2 cells/microliter, WBC 2-3 cells/microliter, normal renal profile and normal C3-C4 counts. Kidney Kidney biopsy was taken and described a picture of minimal change glomerulonephritis with mild mesangial expansion. In addition, the study was negative for the presence of Immunocomplement deposition with the absence of capillary basement membrane and tubulointerstitial inflammation. Patient was treated with Mycophenolate Mofetil 2 grams twice per day and 1mg/kg/day oral steroid. 3 months later, protein was noticeably reduced in urine to 731 mg/dl/day but the patients had a side effect of MFM inform of significant weight loss, gastritis, and diarrhea. Azathioprine had been instituted alternatively with low dose prednisolone 10 mg. Proteinuria level had peaked again after 4 months of starting azathioprine to reach 5660 mg/dl. Kidney biopsy performed again which showed minimal light microscopic changes, the ultrastructural study showed extensive podocyte injury and foot process effacement up to 70% by the electron microscope. Mild mesangial proliferation and focal tubulointerstitial chronic changes. There was no evidence of endocapillary proliferation, subendothelial deposits or glomerular scarring. Immunoflourecance was negative for immunocompetent deposition. Mycophenolate Mofetil has been instituted again but unfortunately, proteinuria inadequately responded and remained between 3-4 grams. Lateraly, Cyclosporin 1.25 mg/kg/day has been commence few days ago. All laboratory results like renal profile and complements are with in normal values. <h3>Results</h3> Proteinuria was found to be attributed to diseased podocyte namely lupus podocytopathy. Unfortunately, she has inadequate response to azathioprine and mycophenolate mofetil. <h3>Conclusion</h3> Lupus podocytopathy is a recently recognized new entity of lupus nephritis characterized by diffuse foot process effacement without capillary wall immune deposits and glomerular proliferation. This case presentation illustrate an attention toward this new lupus entity. However, the lack of efficient knowledge about the frequency, clinical features and treatment necessitate further investigation. (2) <h3>References</h3> [1] Weixin H, Yinghua C, Shaofan W, Hao C, Zhengzhao L, Caihong Z, Haitao Z, Zhihong L, Clinical–Morphological Features and Outcomes of Lupus Podocytopathy. CJASN.2016;11(4):585-592 [2] Suzuki K, Kaneko Y, Anan R, et al. FRI0370 Clinical features and outcomes of lupus nephritis with podocyte injury in patients with systemic lupus erythematosus. BMJ. 2018;77:720. <h3>Acknowledgement</h3> Abdulrahman K. Alabdulsalam, MBBS. Anatomic Pathology Assistant Consultant Department of Pathology and Laboratory Medicine King Saud bin Abdulaziz University for Health Sciences Ministry of National Guard <h3>Disclosure of Interests</h3> None declared