P0288CLINICOPATHOLOGICAL FEATURES OF FOCAL AND SEGMENTAL GLOMERULOSCLEROSIS: STEPS FOR AN ACCURATE CLASSIFICATION - A SINGLE CENTRE CASE SERIES

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Afonso Santos ◽  
Pedro Campos ◽  
Anna Lima ◽  
Ana Gaspar ◽  
Catarina Brás ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Clinical Practice ◽  
Median Time ◽  
Kidney Biopsy ◽  
Genetic Group ◽  
Focal And Segmental Glomerulosclerosis ◽  
Histological Pattern ◽  
Nephrotic Proteinuria ◽  
Foot Process

Abstract Background and Aims Focal and segmental glomerulosclerosis (FSGS) is a histological pattern that is defined by the presence of segmental sclerosis of at least one glomerulus in the kidney biopsy. However, the etiological classification of this lesion remains challenging in clinical practice. FSGS can be divided into primary/idiopathic, genetic or secondary. Nowadays here is no biochemical marker that allows clinicians to distinguish between these categories nor a pathognomonic feature associated to a particular subtype of FSGS. Also, genetic testing is not suitable for all patients. Although it is crucial to determine the cause of FSGS, this is not easily done in clinical practice. Herein we present a series of patients with histologic diagnosis of FSGS in which clinical and histological criteria were applied in order to establish the etiologic classification of FSGS. Method A retrospective analysis of 359 patients who performed kidney biopsy from January 2010 to December 2018 was performed. Patients with histological features of FSGS were identified. Clinical, laboratorial and pathological data were collected, including treatment and outcome. Patients were classified as having genetic FSGS if they accomplished at least one of the following: a) nephrotic syndrome resistant to corticosteroids; b) non-nephrotic proteinuria or nephrotic syndrome with normal serum albumin; c) non-nephrotic proteinuria or nephrotic syndrome with focal foot process effacement or d) non-nephrotic proteinuria with diffuse foot process effacement. The remaining patients were classified as secondary FSGS (if they presented a disease known to be associated with FSGS) or primary FSGS. Each group was divided according to treatment with corticoid, other immunosuppressive agents or and RAAS blockage. Renal outcomes were assessed (progression to ESKD). Results A total of 359 kidney biopsies were performed between January 2010 to December 2018, 66 with a histological pattern of FSGS. 65% (N=43) were males, 71% (N=47) were Caucasian and 23% (N=15) were black. 74% (N=49) had past medical history of hypertension, 26% (N=17) diabetes mellitus and 5% (N=3) had HIV. The majority of patients were classified as having secondary FSGS (52%, N=34), 23% (N=15) were classified as primary and as 25% (N=17) genetic/idiopathic. From the genetic group 58.8% (N=10) were treated with corticoid, 12% (N=2) with other IS agents and 71% (N=12) with ACE/ARB. 29% (N=5) evolved to ESKD (median time to dialysis 11 IQR 1-30). In the primary FSGS group 40% (N=6) were treated with corticoid, 7% (N=1) with other IS agents and 80% (N=12) with ACE/ARB. 27% (N=4) evolved to ESKD (median time to dialysis 0 months, IQR 0-33). In the genetic group 59% (N=10) were treated with corticoid, 12% (N=2) with other IS agents and 71%(N=12) with ACE/ARB. 29% (N=5) evolved to ESKD (median time to dialysis 11 months, IQR 1-30). Conclusion FSGS is a very prevalent glomerular disease and its correct etiologic classification aids in better treatment choice. Although its etiology is not straightforward in most of the patients, some clinical and histological characteristics aid in FSGS classification.

scholarly journals Focal and Segmental Glomerulosclerosis and Membranous Nephropathy overlapping in a patient with Nephrotic Syndrome: a case report

2020 ◽  
Vol 42 (1) ◽  
pp. 113-117
Author(s):  
Crislaine Aparecida da Silva ◽  
Fabiano Bichuette Custódio ◽  
Maria Luíza Gonçalves dos Reis Monteiro ◽  
Stanley de Almeida Araújo ◽  
Liliane Silvano Araújo ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Membranous Nephropathy ◽  
Morphological Characteristics ◽  
Stage Ii ◽  
Primary Case ◽  
Focal And Segmental Glomerulosclerosis ◽  
Morphological Findings ◽  
Segmental Glomerulosclerosis ◽  
Podocyte Detachment ◽  
Foot Process

Abstract Introduction: Some cases of membranous nephropathy (MGN) present focal segmental glomerulosclerosis (FSGS) typically associated with disease progression. However, we report a case of a patient who seemed to have MGN and FSGS, both primary. Case presentation: A 17-year-old female, Caucasian, presenting lower extremity edema associated with episodes of foamy urine and high blood pressure, had physical and laboratorial exams indicating nephrotic syndrome. A renal biopsy was performed and focal and segmental glomerulosclerosis were observed under light microscopy in some glomeruli presented as tip lesion, and in others it was accompanied by podocyte hypertrophy and podocyte detachment in urinary space, compatible with podocytopathy FSGS. Besides, there were thickened capillary loops with basement membrane irregularities due to "spikes" compatible with MGN stage II. Immunofluorescence showed finely granular IgG, IgG4, and PLA2R deposits in capillary loops and, in electron microscopy, subepithelial deposits and foot process effacement. These morphological findings are compatible with FSGS and MGN stage II. Conclusions: In the present case, clinical and morphological characteristics showed a possible overlap of primary FSGS and MGN as focal and segmental glomerulosclerosis does not seem to be related with MGN progression but with the podocytopathy FSGS.

INVESTIGATING ETIOLOGIES AND RENAL HISTOLOGIC PATTERNS IN 6 ELDERLY PATIENTS WITH NEPHROTIC SYNDROME

2015 ◽  
pp. 85-92
Author(s):  
Bach Nguyen ◽  
Thi Thuy Trang Le ◽  
Ngoc Linh Huynh
Keyword(s):  
Nephrotic Syndrome ◽  
Elderly Patients ◽  
Kidney Biopsy ◽  
The Elderly ◽  
Minimal Change ◽  
Renal Pathology ◽  
Invasive Technique ◽  
Renal Amyloidosis ◽  
Glomerular Diseases

Background: Nephrotic syndrome (NS) is the most common manifestation of glomerular diseases in the elderly and a most common indication of kidney biopsy. NS in the elderly is not as common as the young but more difficult to make diagnosis of etiologies, classification of renal histologic patterns and treatment because NS is frequently associated with various coexisting conditions. In Vietnam, the elderly population has been increased significantly therefore frequency of the elderly patients with NS is also increasing. Kidney biopsy is an invasive technique that is useful in diagnosing etiologies and classifying renal pathology. During recent years, renal pathology and biochemical immunology have been progressing rapidly. Therefore, the results of kidney biopsy are usually potential and valuable in clinical practice. We reported 6 elderly patients with NS performed kidney biopsy in Department of Nephrology, HCMCity during the period from 2/2012 to 12/2014 to investigate etiologies and renal histologic patterns. Materials and method: case report. The reported clinical cases were primary renal amyloidosis, IgA nephropathy secondary to liver cancer, minimal change NS associated with diabetes, NS caused by renal lymphoma infiltration, NS with minimal change associated by interferon and thrombotic microangiopathy. Conclusions: Nephrotic syndrome in the elderly might be associated with coexisting conditions and caused by several primary and secondary causes. Therefore, kidney biopsy should be considered to perform to make exact diagnosis in etiology, and to classify histologic patterns. Key words: Nephrotic syndrome, elderly, histologic patterns, kidney biopsy

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

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S68-S69
Author(s):  
L Ding ◽  
J Tomaszewski ◽  
L Liu ◽  
B Murray
Keyword(s):  
Nephrotic Syndrome ◽  
Diabetic Nephropathy ◽  
Lupus Nephritis ◽  
Renal Biopsy ◽  
Glomerular Diseases ◽  
Nephrotic Proteinuria ◽  
Immune Deposits ◽  
Foot Process ◽  
Lupus Podocytopathy ◽  
Dose Prednisone

Abstract 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.

scholarly journals Recurrent podocytopathy in a patient with systemic lupus erythematosus

2017 ◽  
Vol 5 ◽  
pp. 2050313X1769599
Author(s):  
Shereen Paramalingam ◽  
Daniel D Wong ◽  
Gursharan K Dogra ◽  
Johannes C Nossent
Keyword(s):  
Electron Microscopy ◽  
Systemic Lupus Erythematosus ◽  
Nephrotic Syndrome ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Systemic Lupus Erythematosus Activity ◽  
Growing Body ◽  
Foot Process ◽  
Lupus Podocytopathy

Podocytopathy in systemic lupus erythematosus is characterised by diffuse foot process effacement without significant peripheral capillary wall immune deposits as seen on electron microscopy. Lupus podocytopathy falls outside the scope of the current International Society of Nephrology and the Renal Pathology Society classification of lupus nephritis. We present a case of relapsing podocytopathy with nephrotic syndrome occurring simultaneously with two extra-renal and serological disease flares, which makes it likely that podocytopathy was related to systemic lupus erythematosus activity. This case adds to the growing body of evidence that lupus podocytopathy must be considered in the differential diagnosis of systemic lupus erythematosus patients presenting with nephrotic syndrome.

scholarly journals A case of lupus podocytopathy and a review of the literature

2021 ◽  
Vol 35 (1) ◽  
pp. 39-42
Author(s):  
José Silvano ◽  
◽  
Augusta Praça ◽  
Inês Ferreira ◽  
Ana Nunes ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Minimal Change Disease ◽  
Kidney Injury ◽  
Mesangial Proliferation ◽  
Foot Process ◽  
Complex Deposition ◽  
Lupus Podocytopathy

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.

scholarly journals “It’s not lupus”. A placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. A grand round case

Lupus ◽  
2021 ◽  
pp. 096120332098176
Author(s):  
Sarah J van der Lely ◽  
Jeffrey Boorsma ◽  
Marc Hilhorst ◽  
Jesper Kers ◽  
Joris Roelofs ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Lupus Erythematosus ◽  
Kidney Biopsy ◽  
Gestational Trophoblastic Disease ◽  
Grand Round ◽  
Placental Site Trophoblastic Tumor ◽  
Trophoblastic Tumor ◽  
Anchoring Bias ◽  
Placental Site ◽  
History Of

Introduction: Placental site trophoblastic tumor (PSTT) is a rare subtype of gestational trophoblastic disease. Association of PSTT and nephrotic syndrome is exceedingly rare and has been described in 8 cases thus far. In all cases hysterectomy was performed within months after onset of symptoms, leading to immediate remission of nephrotic syndrome, except for one patient who died of complications of PSTT. Case: We describe the history of a woman in which PSTT was discovered years after onset of nephrotic syndrome. Kidney biopsy revealed lupus-like mesangiocapillary nephritis and over time the patient developed additional symptoms mimicking systemic lupus erythematosus (SLE). Discussion: We provide an overview of the literature on this clinical entity and elaborate on its pathophysiology. In addition, we reflect on the phenomenon of anchoring bias, that led physicians to assume the patient had SLE without questioning this diagnosis in the light of the unexplained finding of increased tumor markers.

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

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Hend H. Abdelnabi
Keyword(s):  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Histopathological Analysis ◽  
Immune Deposits ◽  
Foot Process ◽  
Lupus Podocytopathy ◽  
Focal Segmental Sclerosis ◽  
Nephrotic Range Proteinuria

Abstract 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.

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