Loading

Journal of Immunology and Clinical Research

Lupus Podocytopathy: An Unusual Entity Requiring Inclusion in the Conventional Classification of Lupus Nephritis

Case Report | Open Access

  • 1. University College of Medical Sciences and Guru Teg Bahadur Hospital, India
+ Show More - Show Less
Corresponding Authors
Roli Bansal, University College of Medical Sciences and Guru Teg Bahadur Hospital, India
Abstract

Lupus podocytopathy, which is characterized by diffuse foot process effacement without peripheral capillary wall immune deposits and glomerular proliferation, has been described in SLE patients with proteinuria in case reports and small series. We present the case of a 38 year old female with systemic lupus erythematosus and significant proteinuria who had normal glomeruli on light microscopy. Immune florescence showed minimal mesangial deposits and electron microscopy revealed podocytopathy. Lupus podocytopathy is a relatively unusual subclass of Lupus Nephritis which should be considered for inclusion in the classification of SLE. It responds well to steroids and physicians and pathologists should keep a high index of suspicion to pick up such cases.

Keywords


•    Systemic lupus erythematosus (SLE)
•    Lupus podocytopathy
 

INTRODUCTION

Proteinuria is a ubiquitous finding in lupus nephritis (LN), and is usually associated with immune complex deposition in the glomerular capillary wall. It is also frequently accompanied by endocapillary proliferation or necrosis. A subset of systemic lupus erythematosus (SLE) patients with significant proteinuria have been found to have either normal glomerulus or mild mesangial proliferation [1]. They have diffuse foot process effacement on electron microscopy (EM) without immune deposits in the capillary walls, suggestive of podocytopathy. Very few patients with lupus podocytopathy have been presented so far, that too in case reports or small series [2].

Citation

Bansal R (2016) Lupus Podocytopathy: An Unusual Entity Requiring Inclusion in the Conventional Classification of Lupus Nephritis. J Immunol Clin Res 3(1): 1031

CASE REPORT

A 38 year old lady presented with photosensitive hypopigmented plaques over bilateral malar region of face and trunk sine last 5 years. She had also been complaining of hair loss and hard palate ulcers. Patient did not take any treatment in the past. She denied any frothy urine, hematuria, use of Non Steroidal Anti Inflammatory Drugs or tuberculosis in the past. She was found to be hypertensive during her evaluation on admission. She had hypopigmented plaques over the face bilaterally, abdomen and upper back. Rest systemic examination did not reveal any significant finding. Initial investigations revealed Hb- 11.6gm/ dl, TLC- 5100/cumm, platelets- 125000/cumm. Urine routine showed proteinuria with no active sediments. 24 hour urinary protein excretion was 2.08gm. She had normal renal function (urea- 18mg/dl, creatinine- 0.5mg/dl). Serum sodium- 141meq/ Lt, potassium- 4.5meq/Lt. Liver function tests were normal with serum albumin being 3.5gm/dl. Her total cholesterol was 123mg/ dl and triglycerides were 345mg/dl. Serology was positive for ANA (speckled pattern on IF with1:160 titres) and anti-doublestranded DNA with low C3. SSA 3+, SSB 3+ and U1SnRNP 1+ were also positive. Serology was negative for rheumatoid factor, anti-proteinase 3 and anti-myeloperoxidase antibodies to cyclic citrullinated peptide. C Reactive Protein < 0.6mg/dl. Also serology for hepatitis B virus, hepatitis C virus and HIV was negative. Ultrasound abdomen showed left kidney- 12x4cm, right kidney- 10.5 x 3.5cm, with raised echotexture bilaterally. Kidney biopsy revealed unremarkable glomerular basement membrane and tubules with no significant interstitial inflammation on light microscopy (Figure 1). Immune Fluorescence (IF) showed mild mesangial staining for IgG and IgM. EM showed thickened glomerular basement membranes with extensive foot process effacement (Figure 2). Patient was managed with prednisolone 60 mg once a day for 12 weeks, which was then gradually tapered down over a period of next 3 to 4 months. Also she was started on angiotensin receptor blockers and statins. She showed improvement in the proteinuria after initiating therapy. 24 hour urinary protein was 1000mg at 4 weeks and 640 mg at 12 weeks.

She was also advised hydroxychloroquine 200mg once day for her cutaneous manifestations.

DISCUSSION

The clinical presentation of nephrotic syndrome in a patient with SLE, with either absence of subendothelial or subepithelial immune deposits or confined to mesangium and foot process effacement on EM is suggestive of lupus podocytopathy. The current classification of lupus nephritis, revised by the International Society of Nephrology/Renal Pathology Society (ISN/RPS) in 2003, does not recognize lupus podocytopathy as a distinct entity [3]. A series of 7 SLE patients who presenting with the nephrotic syndrome having diffuse podocyte effacement on renal biopsy was reported by Dube et al. [4]. The statistical chance of idiopathic minimal change disease and SLE occurring in the same individual is less than 1 in 10000 [5]. Similar series of cases have been reported the prevalence of this entity as 2 in 132 and 7 in 470 [5,6]. Various theories have been proposed for the pathogenesis of the mesangial glomerulopathy of lupus (World Health Organization class II) in patients with the nephrotic syndrome. Most common hypothesis suggests that a product of aberrant T cell function may be responsible for glomerular epithelial cell injury resulting in diffuse podocyte effacement [7]. There is activation of Th2 subset which overproduces IL-6, IL10, IL-13, and tumor necrosis factor-α whereas Th1 subset under produces IL-2, interferon-γ and transforming growth factor-β. Some authors favour a circulating glomerular toxin hypothesis, where IL-13 has been the suggested toxin on the basis of overexpression in rats, apparently inducing minimal change picture on EM [8]. Our case provides support for the addition of a class or subclass of lupus podocytopathy to the histological classification of lupus nephritis. Podocytopathy in SLE responds to treatment with oral corticosteroids, and the response can be monitored by a decline in the magnitude of proteinuria. Clinicians and renal pathologists should be aware of this entity as a cause of nephrotic range proteinuria in patients with SLE.

Received : 23 Aug 2016
Accepted : 30 Nov 2016
Published : 01 Dec 2016
Journals
Annals of Otolaryngology and Rhinology
ISSN : 2379-948X
Launched : 2014
JSM Schizophrenia
Launched : 2016
Journal of Nausea
Launched : 2020
JSM Internal Medicine
Launched : 2016
JSM Hepatitis
Launched : 2016
JSM Oro Facial Surgeries
ISSN : 2578-3211
Launched : 2016
Journal of Human Nutrition and Food Science
ISSN : 2333-6706
Launched : 2013
JSM Regenerative Medicine and Bioengineering
ISSN : 2379-0490
Launched : 2013
JSM Spine
ISSN : 2578-3181
Launched : 2016
Archives of Palliative Care
ISSN : 2573-1165
Launched : 2016
JSM Nutritional Disorders
ISSN : 2578-3203
Launched : 2017
Annals of Neurodegenerative Disorders
ISSN : 2476-2032
Launched : 2016
Journal of Fever
ISSN : 2641-7782
Launched : 2017
JSM Bone Marrow Research
ISSN : 2578-3351
Launched : 2016
JSM Mathematics and Statistics
ISSN : 2578-3173
Launched : 2014
Journal of Autoimmunity and Research
ISSN : 2573-1173
Launched : 2014
JSM Arthritis
ISSN : 2475-9155
Launched : 2016
JSM Head and Neck Cancer-Cases and Reviews
ISSN : 2573-1610
Launched : 2016
JSM General Surgery Cases and Images
ISSN : 2573-1564
Launched : 2016
JSM Anatomy and Physiology
ISSN : 2573-1262
Launched : 2016
JSM Dental Surgery
ISSN : 2573-1548
Launched : 2016
Annals of Emergency Surgery
ISSN : 2573-1017
Launched : 2016
Annals of Mens Health and Wellness
ISSN : 2641-7707
Launched : 2017
Journal of Preventive Medicine and Health Care
ISSN : 2576-0084
Launched : 2018
Journal of Chronic Diseases and Management
ISSN : 2573-1300
Launched : 2016
Annals of Vaccines and Immunization
ISSN : 2378-9379
Launched : 2014
JSM Heart Surgery Cases and Images
ISSN : 2578-3157
Launched : 2016
Annals of Reproductive Medicine and Treatment
ISSN : 2573-1092
Launched : 2016
JSM Brain Science
ISSN : 2573-1289
Launched : 2016
JSM Biomarkers
ISSN : 2578-3815
Launched : 2014
JSM Biology
ISSN : 2475-9392
Launched : 2016
Archives of Stem Cell and Research
ISSN : 2578-3580
Launched : 2014
Annals of Clinical and Medical Microbiology
ISSN : 2578-3629
Launched : 2014
JSM Pediatric Surgery
ISSN : 2578-3149
Launched : 2017
Journal of Memory Disorder and Rehabilitation
ISSN : 2578-319X
Launched : 2016
JSM Tropical Medicine and Research
ISSN : 2578-3165
Launched : 2016
JSM Head and Face Medicine
ISSN : 2578-3793
Launched : 2016
JSM Cardiothoracic Surgery
ISSN : 2573-1297
Launched : 2016
JSM Bone and Joint Diseases
ISSN : 2578-3351
Launched : 2017
JSM Bioavailability and Bioequivalence
ISSN : 2641-7812
Launched : 2017
JSM Atherosclerosis
ISSN : 2573-1270
Launched : 2016
Journal of Genitourinary Disorders
ISSN : 2641-7790
Launched : 2017
Journal of Fractures and Sprains
ISSN : 2578-3831
Launched : 2016
Journal of Autism and Epilepsy
ISSN : 2641-7774
Launched : 2016
Annals of Marine Biology and Research
ISSN : 2573-105X
Launched : 2014
JSM Health Education & Primary Health Care
ISSN : 2578-3777
Launched : 2016
JSM Communication Disorders
ISSN : 2578-3807
Launched : 2016
Annals of Musculoskeletal Disorders
ISSN : 2578-3599
Launched : 2016
Annals of Virology and Research
ISSN : 2573-1122
Launched : 2014
JSM Renal Medicine
ISSN : 2573-1637
Launched : 2016
Journal of Muscle Health
ISSN : 2578-3823
Launched : 2016
JSM Genetics and Genomics
ISSN : 2334-1823
Launched : 2013
JSM Anxiety and Depression
ISSN : 2475-9139
Launched : 2016
Clinical Journal of Heart Diseases
ISSN : 2641-7766
Launched : 2016
Annals of Medicinal Chemistry and Research
ISSN : 2378-9336
Launched : 2014
JSM Pain and Management
ISSN : 2578-3378
Launched : 2016
JSM Women's Health
ISSN : 2578-3696
Launched : 2016
Clinical Research in HIV or AIDS
ISSN : 2374-0094
Launched : 2013
Journal of Endocrinology, Diabetes and Obesity
ISSN : 2333-6692
Launched : 2013
Journal of Substance Abuse and Alcoholism
ISSN : 2373-9363
Launched : 2013
JSM Neurosurgery and Spine
ISSN : 2373-9479
Launched : 2013
Journal of Liver and Clinical Research
ISSN : 2379-0830
Launched : 2014
Journal of Drug Design and Research
ISSN : 2379-089X
Launched : 2014
JSM Clinical Oncology and Research
ISSN : 2373-938X
Launched : 2013
JSM Bioinformatics, Genomics and Proteomics
ISSN : 2576-1102
Launched : 2014
JSM Chemistry
ISSN : 2334-1831
Launched : 2013
Journal of Trauma and Care
ISSN : 2573-1246
Launched : 2014
JSM Surgical Oncology and Research
ISSN : 2578-3688
Launched : 2016
Annals of Food Processing and Preservation
ISSN : 2573-1033
Launched : 2016
Journal of Radiology and Radiation Therapy
ISSN : 2333-7095
Launched : 2013
JSM Physical Medicine and Rehabilitation
ISSN : 2578-3572
Launched : 2016
Annals of Clinical Pathology
ISSN : 2373-9282
Launched : 2013
Annals of Cardiovascular Diseases
ISSN : 2641-7731
Launched : 2016
Journal of Behavior
ISSN : 2576-0076
Launched : 2016
Annals of Clinical and Experimental Metabolism
ISSN : 2572-2492
Launched : 2016
Clinical Research in Infectious Diseases
ISSN : 2379-0636
Launched : 2013
JSM Microbiology
ISSN : 2333-6455
Launched : 2013
Journal of Urology and Research
ISSN : 2379-951X
Launched : 2014
Journal of Family Medicine and Community Health
ISSN : 2379-0547
Launched : 2013
Annals of Pregnancy and Care
ISSN : 2578-336X
Launched : 2017
JSM Cell and Developmental Biology
ISSN : 2379-061X
Launched : 2013
Annals of Aquaculture and Research
ISSN : 2379-0881
Launched : 2014
Clinical Research in Pulmonology
ISSN : 2333-6625
Launched : 2013
Annals of Forensic Research and Analysis
ISSN : 2378-9476
Launched : 2014
JSM Biochemistry and Molecular Biology
ISSN : 2333-7109
Launched : 2013
Annals of Breast Cancer Research
ISSN : 2641-7685
Launched : 2016
Annals of Gerontology and Geriatric Research
ISSN : 2378-9409
Launched : 2014
Journal of Sleep Medicine and Disorders
ISSN : 2379-0822
Launched : 2014
JSM Burns and Trauma
ISSN : 2475-9406
Launched : 2016
Chemical Engineering and Process Techniques
ISSN : 2333-6633
Launched : 2013
Annals of Clinical Cytology and Pathology
ISSN : 2475-9430
Launched : 2014
JSM Allergy and Asthma
ISSN : 2573-1254
Launched : 2016
Journal of Neurological Disorders and Stroke
ISSN : 2334-2307
Launched : 2013
Annals of Sports Medicine and Research
ISSN : 2379-0571
Launched : 2014
JSM Sexual Medicine
ISSN : 2578-3718
Launched : 2016
Annals of Vascular Medicine and Research
ISSN : 2378-9344
Launched : 2014
JSM Biotechnology and Biomedical Engineering
ISSN : 2333-7117
Launched : 2013
Journal of Hematology and Transfusion
ISSN : 2333-6684
Launched : 2013
JSM Environmental Science and Ecology
ISSN : 2333-7141
Launched : 2013
Journal of Cardiology and Clinical Research
ISSN : 2333-6676
Launched : 2013
JSM Nanotechnology and Nanomedicine
ISSN : 2334-1815
Launched : 2013
Journal of Ear, Nose and Throat Disorders
ISSN : 2475-9473
Launched : 2016
JSM Ophthalmology
ISSN : 2333-6447
Launched : 2013
Journal of Pharmacology and Clinical Toxicology
ISSN : 2333-7079
Launched : 2013
Annals of Psychiatry and Mental Health
ISSN : 2374-0124
Launched : 2013
Medical Journal of Obstetrics and Gynecology
ISSN : 2333-6439
Launched : 2013
Annals of Pediatrics and Child Health
ISSN : 2373-9312
Launched : 2013
JSM Clinical Pharmaceutics
ISSN : 2379-9498
Launched : 2014
JSM Foot and Ankle
ISSN : 2475-9112
Launched : 2016
JSM Alzheimer's Disease and Related Dementia
ISSN : 2378-9565
Launched : 2014
Journal of Addiction Medicine and Therapy
ISSN : 2333-665X
Launched : 2013
Journal of Veterinary Medicine and Research
ISSN : 2378-931X
Launched : 2013
Annals of Public Health and Research
ISSN : 2378-9328
Launched : 2014
Annals of Orthopedics and Rheumatology
ISSN : 2373-9290
Launched : 2013
Journal of Clinical Nephrology and Research
ISSN : 2379-0652
Launched : 2014
Annals of Community Medicine and Practice
ISSN : 2475-9465
Launched : 2014
Annals of Biometrics and Biostatistics
ISSN : 2374-0116
Launched : 2013
JSM Clinical Case Reports
ISSN : 2373-9819
Launched : 2013
Journal of Cancer Biology and Research
ISSN : 2373-9436
Launched : 2013
Journal of Surgery and Transplantation Science
ISSN : 2379-0911
Launched : 2013
Journal of Dermatology and Clinical Research
ISSN : 2373-9371
Launched : 2013
JSM Gastroenterology and Hepatology
ISSN : 2373-9487
Launched : 2013
Annals of Nursing and Practice
ISSN : 2379-9501
Launched : 2014
JSM Dentistry
ISSN : 2333-7133
Launched : 2013
Author Information X