scholarly journals Nine years old child with nephrotic syndrome: a case of biopsy proven lupus nephritis in tertiary level medical college hospital in Mymensingh, Bangladesh

2018 ◽  
Vol 7 (1) ◽  
pp. 40-44
Author(s):  
Mahmud Javed Hasan ◽  
Nitai Chandra Ray ◽  
ASM Ruhul Quddus

We observed a case of a 9 years old female patient who presented with nephrotic syndrome and impaired renal function diagnosed to have Systemic Lupus Erythematosus (SLE). Lupus nephritis (LN) is a common major organ manifestation of SLE and causes significantly increased morbidity and mortality. Thus, all patients with SLE should be regularly screened for LN. This patient presented with nephrotic syndrome, pleural effusion and pericardial effusion which depicts the multisystem effects of SLE. This patient was treated with Oral Mycophenolate Mofetilin combination with steroid as induction therapy and attained remission after a month of treatment. Systemic lupus erythematosus should be considered in patients with nephrotic syndrome and these patients should have renal biopsy to determine renal involvement. CBMJ 2018 January: Vol. 07 No. 01 P: 40-44

2020 ◽  
pp. 5001-5012
Author(s):  
Liz Lightstone ◽  
Hannah Beckwith

Many rheumatological conditions have systemic effects. Antibody production, complement activation, and protein deposition can all result in damage to the kidney, sometimes with devastating sequelae. Systemic lupus erythematosus—lupus nephritis is clinically evident in up to 75% of patients with systemic lupus erythematosus (SLE), and endstage renal disease is seen in 5 to 10% of patients at 10 years. Proteinuria is the most common clinical presentation, closely followed by nonvisible haematuria and tubular abnormalities. Patients with active lupus nephritis often have features of active SLE. The gold standard for lupus nephritis diagnosis is a renal biopsy, with treatment related to histopathological features observed. Adjunctive immunosuppressive agents such as rituximab and tacrolimus are emerging as increasingly important lupus nephritis therapies. Systemic sclerosis is a multiorgan connective tissue disease. Most renal manifestations are clinically silent. By contrast, the scleroderma renal crisis is characterized by accelerated-phase hypertension and impaired renal function. It carries a high mortality risk. Rheumatoid arthritis can affect the kidneys in many ways, most commonly by causing amyloid A amyloidosis. This presents with proteinuria, often severe enough to cause nephrotic syndrome, with 50% progressing to endstage renal failure after 5 years (90% at 10 years). Renal vasculitis, mesangiocapillary glomerulonephritis, and mesangial IgA proliferative glomerulonephritis are also described. Gold and penicillamine (now rarely used) can cause proteinuria, sometimes with nephrotic syndrome. Renal involvement in Sjögren’s syndrome is generally mild, but up to a quarter of patients develop acute or chronic kidney disease, typically with evidence of tubular dysfunction. Glomerular abnormalities are rare and the most common histological abnormality is tubulointerstitial nephritis. Drug nephrotoxicity—conventional antirheumatics and over-the-counter nonsteroidal anti-inflammatory drugs are used exceptionally widely in the community and are nephrotoxic. Their almost ubiquitous use, especially during intercurrent illnesses, means they are frequent contributors to acute and chronic kidney damage.


2011 ◽  
Vol 68 (8) ◽  
pp. 705-708
Author(s):  
Natasa Jovanovic ◽  
Jasmina Markovic-Lipkovski ◽  
Stevan Pavlovic ◽  
Biljana Stojimirovic

Introduction. Systemic lupus erythematosus (SLE) is a chronic immunological disease causing a significant morbidity and mortality in younger women and involving several organs and systems, most often the kidneys, being consequently the incidence of lupus nephritis (LN) about 60%. Case report. We reported a 57 year-old patient with the diagnosed SLE in 1995. Pathohistological analysis of kidney biopsy revealed LN type V. The patient was treated with corticosteroid pulses and azathioprine during one year. A remission was achieved and maintained with prednisone, 15 mg daily. Nephrotic relapse was diagnosed in 2006 and the second kidney biopsy revealed recent kidney infarction due to extensive vasculitis. Soon, a cerebrovascul insult developed and CT-scan revealed endocranial infarctus. The patient was treated with corticosteroids and cyclophosphamide pulses (totally VI monthly pulses), and also with low-molecular heparine, anticoagulants and salicylates because of the right leg phlebothrombosis. After the pulses, the patient was adviced to take prednisone 20 mg daily and azothioprine 100 mg daily, and 6 months later mycophenolate mofetil because of persistent active serological immunological findings (ANA 1 : 320) and nephrotic syndrome. Mycophenolate mofetil was efficient in inducing and maintaining remission of nephrotic syndrome. Conclusion. The aim of LN treatment is to achieve and maintain remission, improve patients? outcome, reduce the toxicity of immunosuppressive drugs and the incidence of relapses. Mycophenolate mofetil was shown to be efficient in inducing and maintaining remission of nephrotic syndrome in the frame of LN.


2019 ◽  
Vol 89 (2) ◽  
Author(s):  
Anshul Mittal ◽  
Jagdish Chander Suri ◽  
Shibdas Chakrabarti ◽  
Pranav Ish

It is uncommon for Systemic lupus erythematosus (SLE) to present with diffuse alveolar hemorrhage (DAH) as the initial presentation. To diagnose this in a young male with no renal involvement is further uncommon. We report a case of a 16-year-old boy, who presented with hemoptysis and was eventually diagnosed as DAH with underlying SLE. Treatment with steroids and immunosuppressant helped in rapid recovery from this potentially life-threatening condition. This case highlights the need of defining diagnostic criteria for SLE in patients presenting as DAH and formulating guidelines for treatment of the same, especially in absence of co-existing lupus nephritis.


Author(s):  
Sonia L La’ulu ◽  
Brenda B Suh-Lailam ◽  
K Wayne Davis ◽  
Joely A Straseski ◽  
Anne E Tebo

Background Lupus nephritis is one of the most serious complications of systemic lupus erythematosus. This study evaluates the prevalence and correlation between neutrophil gelatinase-associated lipocalin and other biomarkers associated with renal involvement in systemic lupus erythematosus. Methods Paired serum and urine specimens from 50 suspected systemic lupus erythematosus patients, characterized by antinuclear antibodies detected by indirect immunofluorescence assay and varying positive concentrations of anti-double stranded DNA antibodies by Crithidia luciliae immunofluorescence assay, were investigated. Of these 50 patients, 18 were identified with renal involvement based upon laboratory serology. Patients and healthy control serum samples ( n = 50) were also evaluated for high avidity double stranded DNA IgG antibodies, anti-C1q IgG antibodies, and serum creatinine. The prevalence and relationship between biomarkers were evaluated using statistical methods. Results Serum and urine neutrophil gelatinase-associated lipocalin concentrations were significantly elevated in patients compared to controls, with a prevalence of 24% and 36%, respectively. These concentrations were also more markedly increased in systemic lupus erythematosus patients with renal involvement than those without. Spearman’s correlations between neutrophil gelatinase-associated lipocalin and other biomarkers tested ranged from 0.06 to 0.66 in all patients. Combined concordance as determined by Cronbach alpha coefficient between biomarkers was reduced from 0.71 to 0.58 (serum) and 0.62 (urine) when neutrophil gelatinase-associated lipocalin was removed. Conclusions Neutrophil gelatinase-associated lipocalin concentrations are elevated and demonstrate variable associated with other laboratory markers for renal involvement in systemic lupus erythematosus. Prospective longitudinal studies are needed to determine the optimal biomarker combinations for use in routine management of systemic lupus erythematosus patients at-risk for lupus nephritis.


2018 ◽  
Vol 8 (1) ◽  
Author(s):  
M Enrica ◽  
A Tjandrawati ◽  
S Rachmayati ◽  
Laniyati Hamijoyo

Background: Lupus nephritis is defined as renal involvement in systemic lupus erythematosus (SLE) patients and the most important cause of morbidity and mortality. The diagnostic criteria that used to diagnose lupus nephritis are 1997 American Collegeof Rheumatology is 24 hours urine protein ≥500 mg and/or cellular cast, but significant renal damage can occur without proteinuria or cellular cast. Anti-C1q is an autoantibody that is produced by a chronic alteration of C1q collagen domain. Anti-C1q is a new specific marker for renal marker.Objective: To determine the validity of anti-C1q serum by using 1997 American College of Rheumatology criteria as a gold standard. Methods: This is a cross sectional study, conducted in October to December 2014 at Hasan Sadikin Hospital Bandung. The subjects had systemic lupus erythematosus with and without renal involvement, based on 1997 American College of Rheumatology criteria for SLE.Results: There were 65 subjects included in this study, 64 subjects were female and 1 subject was male. The age average was 32 (SD 11.7) years old. As many as 66.2% subjects had been diagnosed with lupus erythematosus systemic at least 3 years. Twenty four hours urine protein was measured using spectrophotometry, urine sediment was examinedfor cellular cast, and anti-C1q serum was measured using micro enzyme linked immunosorbent assay. Based on American College of Rheumatology criteria, 34 subjects were classified as lupus nephritis group while 31 subjects were classified as non-lupus nephritis group. The area under the curve of anti-C1q was 0.610. The cut-off value used in this study was 10.43 U/ml. The sensitivity, specificity, positive predictive value,negative predictive value and accuracy of anti-C1q assay were 41.18%, 77.42%, 66.67%, 54.55% and 58.46% respectively.Conclusion: Anti-C1q assay, based on this study, hasa low sensitivity and medium specificity to detect lupusnephritis


Lupus ◽  
2018 ◽  
Vol 28 (1) ◽  
pp. 34-43 ◽  
Author(s):  
L. Pacheco-Lugo ◽  
J. Sáenz-García ◽  
E Navarro Quiroz ◽  
H. González Torres ◽  
L. Fang ◽  
...  

Background Systemic lupus erythematosus is a heterogeneous chronic inflammatory autoimmune disorder characterized by an exacerbated expression of cytokines and chemokines in different tissues and organs. Renal involvement is a significant contributor to the morbidity and mortality of systemic lupus erythematosus, and its diagnosis is based on renal biopsy, an invasive procedure with a high risk of complications. Therefore, the development of alternative, non-invasive diagnostic tests for kidney disease in patients with systemic lupus erythematosus is a priority. Aim To evaluate the plasma levels of a panel of cytokines and chemokines using multiplex xMAP technology in a cohort of Colombian patients with active and inactive systemic lupus erythematosus, and to evaluate their potential as biomarkers of renal involvement. Results Plasma from 40 systemic lupus erythematosus non-nephritis patients and 80 lupus nephritis patients with different levels of renal involvement were analyzed for 39 cytokines using Luminex xMAP technology. Lupus nephritis patients had significantly increased plasma eotaxin, TNF-α, interleukin-17-α, interleukin-10, and interleukin-15 as compared to the systemic lupus erythematosus non-nephritis group. Macrophage-derived chemokine, growth regulated oncogene alpha, and epidermal growth factor were significantly elevated in systemic lupus erythematosus non-nephritis patients when compared to lupus nephritis individuals. Plasma eotaxin levels allowed a discrimination between systemic lupus erythematosus non-nephritis and lupus nephritis patients, for which we performed a receiver operating characteristic curve to confirm. We observed a correlation of eotaxin levels with active nephritis (Systemic Lupus Erythematosus Disease Activity Index). Our data indicate that circulating cytokines and chemokines could be considered good predictors of renal involvement in individuals with systemic lupus erythematosus.


2011 ◽  
Vol 39 (1) ◽  
pp. 79-85 ◽  
Author(s):  
DAISUKE WAKASUGI ◽  
TAKAHISA GONO ◽  
YASUSHI KAWAGUCHI ◽  
MASAKO HARA ◽  
YUMI KOSEKI ◽  
...  

Objective.To determine the frequency of International Society of Nephrology/Renal Pathology Society (ISN/RPS) class III or IV lupus nephritis in patients with systemic lupus erythematosus (SLE) without clinical renal involvement.Methods.We investigated the renal pathology of 195 patients with SLE, including 86 patients without clinical renal involvement.Results.Lupus nephritis other than class I was found in 58% of the patients without clinical renal involvement, and class III and IV nephritis was found in 15% of these patients. To reveal the predictive measures involved in class III or IV lupus nephritis, we explored the clinical measures in patients with SLE who did not have clinical renal involvement. Anti-dsDNA antibody titers were significantly higher (p = 0.0266) and C3 values were significantly lower (p = 0.0073) in patients with class III or IV lupus nephritis than in patients without class III or IV lupus nephritis. The sensitivity and specificity values were 77% and 73%, respectively, for cutoff levels of both 40 IU/ml for anti-dsDNA antibodies and 55 mg/dl for C3 (OR 8.8, p = 0.0011).Conclusion.The frequency of nephritis, including ISN/RPS class III and IV, was unexpectedly high in SLE patients without clinical renal involvement. ISN/RPS class III or IV lupus nephritis could be hidden in patients with SLE who present both a high titer of anti-dsDNA antibody and a low concentration of C3, even when they have clinically normal urinary findings and renal function.


2002 ◽  
Vol 13 (1) ◽  
pp. 42-52
Author(s):  
Eric Daugas ◽  
Dominique Nochy ◽  
Du Le Thi Huong ◽  
Pierre Duhaut ◽  
Hélène Beaufils ◽  
...  

ABSTRACT. In the course of the antiphospholipid syndrome (APS), the existence of vaso-occlusive lesions capable of affecting numerous organs is now well established. The renal involvement attributable to primary APS, APS nephropathy (APSN), corresponds to vaso-occlusive lesions of the intrarenal vessels, associating side-by-side, acute thromboses with chronic arterial and arteriolar lesions, leading to zones of cortical ischemic atrophy. A retrospective study of 114 lupus patients undergoing renal biopsy was undertaken to determine the following: (1) if APSN can be found in the course of systemic lupus erythematosus (SLE); (2) if certain clinical and biologic factors can permit the prediction of the presence of APSN; and (3) if APSN is a superadded renal morbidity factor in lupus patients. This study shows the following: (1) APSN occurs in SLE (32% of patients with renal biopsies) in addition to, and independently of, lupus nephritis; (2) APSN is statistically associated with lupus anticoagulant but not with anticardiolipin antibodies; (3) APSN is associated with extrarenal APS, mainly arterial thromboses and obstetrical fetal loss, but not with the venous thromboses of APS; (4) APSN is an independent risk factor, over and above lupus nephritis, that contributes to an elevated prevalence of hypertension, elevated serum creatinine, and increased interstitial fibrosis. Thus, it seems likely that, because of its associations with hypertension, elevated serum creatinine, and increased interstitial fibrosis, APSN may worsen the prognosis in these patients. APSN may also have therapeutic significance in that its recognition should permit a better balance between immunosuppressor and antithrombotic and/or vasoprotective therapy. Finally, this study suggests that APSN should be considered as an element to be included in the classification criteria of APS.


Systemic lupus erythematosus (SLE) is the most common disease systemic autoimmune disorders that cause kidney damage. AT Conversely, kidney damage is the most common and the most severe visceral involvement of SLE. The most frequent renal involvement is glomerular and there are several types of glomerulonephritis (GN) Lupus now evaluated according to classification histological ISN / RPS (International Society of Nephrology/Renal Pathology Society) [1]. Other glomerular disorders such as a Nephrotic syndrome with minimal glomerular lesions are possible but rare. Vascular or interstitial lesions related to lupus may be associated with glomerular damage; they are rarely isolated. Finally, lupus nephropathy is sometimes mixed with renal diseases associated with lupus, the most common being renal antiphospholipid Syndrome.


2016 ◽  
Vol 3 (2) ◽  
pp. 10-14
Author(s):  
Sanjit Karki ◽  
Roshan Shrestha ◽  
Buddhi Paudyal ◽  
Bimal Pandey ◽  
Nora Ranjitkar ◽  
...  

Introductions: Classifying morphological pattern of renal involvement is important in systemic lupus erythematosus (SLE) for definitive treatment and prognosis. This study aims to analyse the histopathological pattern of glomerula in SLE patients.Methods: This was a retrospective chart review of patients diagnosed with SLE who had renal biopsy during October 2013 to September 2015 at Patan Hospital.Results: There were 38 patients of SLE. Antinuclear antibody (ANA) was positive in all 38 (100 %), Anti-dsDNA seen in 18 (47.4%). Active urinary sediment & proteinuria was seen in 25 (65.8%) patients and proteinuria in 13 (34.2%) patients. Histopathological patterns were of glomerular involvement, ISN Class II in 2 (5.3%), Class III in 2 (5.3%), class IV in 20 (52.5%), Class V in 6 (15.8%) and mixed IV-V in 8 (21.1%).Conclusions: The diffuse proliferative lupus nephritis (ISN Class IV) was the most common pattern of lupus nephritis encountered in our study followed by mixed pattern (ISN Class IV & V) and membranous lupus nephritis (ISN class IV). Journal of Patan Academy of Health Sciences. 2016 Dec;3(2):10-14


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