Delayed Onset Minimal Change Disease as a Manifestation of Lupus Podocytopathy

Lupus podocytopathy (LP) is an uncommon manifestation of systemic lupus erythematosus (SLE) and is not included in the classification of lupus nephritis. The diagnosis of LP is confirmed by the presence of diffuse foot process effacement in the absence of capillary wall deposits with or without mesangial immune deposits in a patient with SLE. Here we describe a 13-year-old female who presented with nephrotic syndrome (NS) seven years after the diagnosis of SLE. The renal function had been stable for seven years since the SLE diagnosis, as manifested by the normal serum creatinine, serum albumin and absence of proteinuria. Renal biopsy showed evidence of minimal change disease without immune complex deposits or features of membranous nephropathy. Renal function was normal. The patient had an excellent response to steroid therapy with remission within two weeks. The patient remained in remission five months later during the most recent follow-up. This report highlights the importance of renal histology to determine the accurate etiology of NS in patients with SLE. Circulating factors, including cytokines such as interleukin 13, may play a role in the pathophysiology of LP and needs to be studied further in future larger studies.

MO325YOU CAN BE DIAGNOSED AND CURED FOR LUPUS CYSTITIS AND LUPUS PODOCYTOPATHY DESPITE BEING A POOR, VILLAGE TEENAGE GIRL IN ALBANIA

Background and Aims We present the case of a 16 years old girl who was admitted to our Emergency Unit in May 2019 for diarrhea, nausea, dysuria, foamy urine, urinary incontinence, malnutrition, polyserositis, and hypertension. Her medical history started 8 months ago with diarrhea and urinary incontinence for which first was hospitalized in the Gastroenterology unit and then in the Infectious Disease Unit. There she was completed with colonoscopy, contrast CT scan and then was discharged with the diagnosis of Gastrocolitis. In January 2019 due to the persistence of symptoms they did a specialized consultation in Athens, Greece. After a series of examinations the patient was diagnosed with Anorexia Nervosa and antidepressant therapy was started. In February 2019, the patient was rehospitalized with nephritic grade proteinuria and the kidney ultrasound showed stage four bilateral hydronephrosis and urinary bladder with thick and trabecular walls. To exclude urological problems, an MRI was performed which results in no obstructive problems. Arterial hypertension and lower extremities edema were present. She was then transferred to our University Hospital “Mother Teresa”, Nephrology Department for further examinations. During hospitalization her blood investigation showed severe anemia (HGB = 6.7gr / dl), kidney failure (creatinine = 1.5mg / dl, urea = 83mg / dl), elevated liver enzymes (Alt:162u/ml, Ast:101u/ml), albuminemia: 2.9 g / dl, total proteinemia: 5.9g / dl. The lipid profile showed cholesterolemia: 300mg / dl, triglyceridemia: 170mg / dl. Electrolytes were within normal limits. Coombs test resulted positive. Urinalysis showed microscopic hematuria with leukocyturia and grave albuminuria around16gr/ 24 hours. Immunologic workup showed: AntiDna = 383.5U / ml, Ena profile SSA poz, ANA ++++, C3 101, C4 18. Tumoral markers and hepatitis resulted in negativ. Method Renal biopsy was performed which resulted: Lupus Podocytopathy Results The patient was diagnosed with a case of lupus cystitis with lupus podocytopathy. She was treated with methylprednisolone, immunosuppressive therapy, and Plaquenil. It was started with intravenous methylprednisolone 0.5 g / day for 3 days and then switched to oral methylprednisolone 0.5 mg /kg /day. Mycophenolate mofetil was started with 1 gr increased to 2 grams. After 6 months of therapy Hydroureteronephrosis completely disappeared and 24 h urinary protein became normal. The dose of therapy was tapered and switch to maintenance doses, methylprednisolone 8 mg, MMF 500gr, and Plaquenil. Laboratory examinations Hgb: 12gr / dl, Urea: 36mg / dl, Creatine: 0.6mg / dl, Alt: 23u / l, Ast: 26u / l. Urinalysis: albumin trace, RBC: 0, Wbc 8 / field. The autoimmune workup was normalized, AntiDna; C3, C4, Ana. In a realized ultrasound hydronephrosis was gone, kidney structure was in normal parameters and bladder wall was in a normal structure. Conclusion Disseminated Eritematous Lupus and its rare forms like Lupus Cystitis and Lupus Podocytopathies can be diagnosed, cured successfully, and followed up in the best way despite you are a simple teenager in a village of Albania or a noticed and famous actor or singer in the USA.

Is podocytopathy another image of renal affection in p-SLE?

Background Lupus podocytopathy (LP) is a renal affection described in systemic lupus erythematosus (SLE) patients with nephrotic range proteinuria, characterized by diffuse foot process effacement without immune deposits and glomerular proliferation. This study describes LP, its pathological features and outcomes of pediatric (p-SLE) patients in comparison to the usual lupus nephritis (LN) cases. Methodology A retrospective cohort study conducted on a 10-year registration (2010–2019) of 140 p-SLE patients at the Pediatric Department, Tanta University. Histopathological analysis with light microscopy (LM) and immunofluorescence (IF) of all renal biopsies were evaluated according to the International Society of Nephrology Renal Pathology Society (ISN/RPS) grading system. In addition, some biopsies were examined with electron microscopy (EM). Results Eighty-six p-SLE cases (61.4%) had renal involvement; seventy-nine biopsies (91.86%) of them met the classification criteria of LN as defined by ISN/RPS system. Five biopsies were normal (MCD) and two showed focal segmental sclerosis (FSGN) that did not meet any known classification of LN. Hence, they were reevaluated using EM that revealed diffuse effaced podocytes without glomerular sub-epithelial, endocapillary or basement membrane immune deposits, and were classified as having lupus podocytopathy, representing (8.14%) of all LN biopsies. Those seven cases showed good response to steroids with a complete remission duration of 3.40 ± 1.95 weeks. However, some case had 1–3 relapses during the duration of follow up. Conclusions LP is a spectrum of p-SLE, not an association as it is related to disease activity and its initial presentation.

A case of lupus podocytopathy and a review of the literature

Nephrotic syndrome in systemic lupus erythematosus patients with histological evidence of minimal change disease, mesangial proliferation or focal and segmental glomerulosclerosis on light microscopy, represents a distinct clinical entity – lupus podocytopathy. This entity is characterized by a diffuse foot process effacement on electron microscopy and by absence of subepithelial or subendothelial immune -complex deposition. We report the case of a 49 -year -old woman admitted on suspicion of lupus nephritis flare, characterized by nephrotic syndrome and acute kidney injury, whose renal biopsy revealed histological features of lupus podocytopathy. Six months after discharge, under prednisolone and azathioprine, she presented 300 mg/day proteinuria, normal kidney function, without hematuria. A review of the pathogenesis, clinical features, diagnostic criteria, treatment and prognosis of lupus podocytopathy is provided. This case highlights the mounting evidence that lupus podocytopathy encompasses distinct clinical and morphological features, that should be included in the upcoming pathological classification of lupus nephritis.

Identification of Lupus Podocytopathy with Coexistent Glomerular Diseases – A Case Report

Introduction/Objective Lupus podocytopathy (LP), featured by nephrotic syndrome, is a unique subtype of lupus nephritis that mimics minimal change disease or primary focal segmental glomerulosclerosis (FSGS) on renal biopsy with diffuse podocyte foot process effacement and no capillary-loop immune deposits. LP usually presents on a background of ISN/RPS class I or class II lupus nephritis, and very rarely may present without immune deposits. Diagnosis of LP, when confounded by other glomerular diseases associated with nephrotic syndrome, can be very challenging and requires thorough clinical and pathology correlations. Methods Here we report a case of LP in a patient with nephrotic syndrome and multiple comorbidities affecting kidneys. A 24-year-old female with type-I diabetes, psoriasis, and intermittent arthritis/rash of unknown etiology, presented with abrupt onset of nephrotic proteinuria attributed to recent low dose prednisone therapy, and renal insufficiency. A renal biopsy showed nodular glomerulosclerosis and FSGS. No immune deposits were identified by immunofluorescence or electron microscopy. Ultrastructurally there was also diffuse glomerular basement membrane thickening and over 90% podocyte foot process effacement. With no established systemic lupus erythematosus (SLE), the case was initially diagnosed as diabetic nephropathy with coexistent FSGS as the etiologies for nephrotic proteinuria, and the patient was put on ACEI and diuretics. However, massive proteinuria persisted, and the patient also developed pancytopenia. Serology concurrent with the biopsy came out later showing positive autoantibodies against dsDNA, Smith, and Histone. With continued worsening of creatinine, a renal biopsy was repeated revealing essentially similar findings to the first biopsy. Results Integrating the serology results and clinical presentation, SLE was favored. The pathology findings were re- evaluated and considered to be most consistent with LP and coexistent diabetic nephropathy, with FSGS either a component of LP or an independent lesion secondary to diabetes or hypertension. The patient was started with high dose prednisone at 60 mg/day. One month later, her proteinuria, serum creatinine, pancytopenia, skin rash, and arthritis were all significantly improved. Conclusion LP can be easily masked by coexistent glomerular diseases. Sufficient awareness of the entity is necessary for the appropriate diagnosis and treatment.

The Various Forms of Nephrotic Syndrome in a Patient with Systemic Lupus Erythematosus

Kidney involvement is frequent in patients with systemic lupus erythematosus (SLE), although it may not be present from disease onset. Renal lupus itself is highly heterogenous with respect to the combination and/or severity of clinical and/or laboratory manifestations. This is a case of a 45-year-old Caucasian female with an established diagnosis of SLE, who presented four times with new onset of proteinuria during a follow-up time of ten years, since the diagnosis of SLE. Specifically, she experienced two episodes of lupus membranous nephropathy, and after she achieved remission, she developed twice overt nephrotic syndrome associated with new and biopsy proven lupus podocytopathy. All these episodes of nephrotic syndrome were combined with systemic symptoms, attributed to lupus itself, while serological activity of lupus was also noted. This case highlights the importance of performing a kidney biopsy in all patients with SLE who have new renal manifestations, including nephrotic proteinuria.