scholarly journals The misleading laboratory results: Renal impairment associated with high anti myeloperoxidase titer (anti-MPO) does not always mean ANCA vasculitis

2021 ◽  
Vol 35 (1) ◽  
pp. 55-58
Author(s):  
Mariana Sousa ◽  
◽  
Cristina Santos ◽  
Rita Gouveia ◽  
Fernando Pereira ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Biopsy ◽  
High Titer ◽  
Elevated Serum ◽  
Oral Prednisolone ◽  
Positive Finding ◽  
Anca Vasculitis ◽  
Impact On Treatment ◽  
The Relationship ◽  
Antineutrophil Cytoplasmic Autoantibodies

Introduction: Antineutrophil cytoplasmic autoantibodies (ANCAs) are crucial in the pathogenesis of ANCA-associated glomerulonephritis. Although highly specific for this disease, these antibodies can appear in healthy individuals or occur in other settings such as malignance, other inflammatory disease, or drugs. Renal biopsy remains essential for diagnosis and as a prognostic tool. Failing to perform a biopsy risks an incomplete evaluation or misdiagnosis. Clinical case: A 60-year-old male known to have had previous normal kidney function was admitted with generalized pitting edema, nonpruritic erythematous rash, and decreased urine output. He had been recently started on clozapine for depression. Laboratory tests revealed nephrotic syndrome with massive proteinuria (11g/day), hematuria and elevated serum creatinine that reached a maximum of 4.9 mg/dl. The only positive finding was a high titer of myeloperoxidase (MPO) ANCA antibody (111 UI/ml). Clozapine was discontinued, and he was started on conservative management of the nephrotic syndrome. The rash disappeared within one week. Kidney biopsy showed cellular variant focal segmental glomerulosclerosis (FSGS) with mild acute tubular injury. The patient was treated with oral prednisolone with complete remission. Repeat MPO-ANCA was negative after one month. Discussion: We present a challenging case that highlights the importance of kidney biopsy for accurate diagnosis with major impact on treatment and prognosis. This case also underlines the presentation and evolution of FSGS cellular variant, a rare and poorly understood disease. The relationship between skin rash with high MPO titer and the administration of clozapine with clinical remission after its discontinuation favors the hypothesis of drug associated ANCA-vasculitis.

scholarly journals The Transition From Minimal Change Disease to Focal and Segmental Glomerulosclerosis in a Patient With Nephrotic Syndrome: a Case Report

2021 ◽  
Author(s):  
Long Tang ◽  
Zhen Cai ◽  
Yuan Meng ◽  
Wen-jing Zhao ◽  
Su-Xia Wang
Keyword(s):  
Nephrotic Syndrome ◽  
Minimal Change Disease ◽  
Kidney Biopsy ◽  
Elevated Serum ◽  
Minimal Change ◽  
Segmental Glomerulosclerosis ◽  
Stage Renal Disease ◽  
End Stage ◽  
The Relationship

Abstract Background:Although minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) have been described as two separate forms of nephrotic syndrome(NS), they are not completely independent. We report a patient presenting a transition from MCD to FSGS, review the literature and explore the relationship between the two diseases.Case presentation:A 42-year-old male welder, Asian, presenting lower extremity edema and elevated serum creatinine, had laboratory exams indicating NS and end-stage renal disease(ESRD). The patient had a kidney biopsy 20 years earlier for NS, which indicated MCD, and this repeated kidney biopsy suggested FSGS. After treatment follow-up, the patient was eventually admitted to renal replacement therapy. Conclusions:MCD and FSGS may be different stages of the same disease. The transition from MCD to FSGS in this case indicates the progression of the disease, which may be related to the excessive metal caused by occupation.

scholarly journals Acute Kidney Injury Associated with Minimal Change Nephrotic Syndrome in an Elderly Patient Successfully Treated with both Fluid Management and Specific Therapy Based on Kidney Biopsy Findings

2020 ◽  
Vol 10 (1) ◽  
pp. 42-50 ◽  
Author(s):  
Yuko Oyama ◽  
Yoichi Iwafuchi ◽  
Tetsuo Morioka ◽  
Ichiei Narita
Keyword(s):  
Elderly Patient ◽  
Acute Kidney Injury ◽  
Nephrotic Syndrome ◽  
Kidney Biopsy ◽  
Fluid Management ◽  
Kidney Injury ◽  
Oral Prednisolone ◽  
Minimal Change Nephrotic Syndrome ◽  
Minimal Change ◽  
Fluid Removal

Oliguric acute kidney injury (AKI) with minimal change nephrotic syndrome (MCNS) has long been recognized. Several mechanisms such as hypovolemia due to hypoalbuminemia and the nephrosarca hypothesis have been proposed. However, the precise mechanism by which MCNS causes AKI has not been fully elucidated. Herein, we describe an elderly patient with AKI caused by MCNS who fully recovered after aggressive volume withdrawal by hemodialysis and administration of a glucocorticoid. A 75-year-old woman presented with diarrhea and oliguria, and laboratory examination revealed nephrotic syndrome (NS) and severe azotemia. Fluid administration had no effect on renal dysfunction, and hemodialysis was initiated. Her renal function improved upon aggressive fluid removal through hemodialysis. Renal pathological findings revealed minimal change disease with faint mesangial deposits of IgA. After administration of methylprednisolone pulse therapy followed by oral prednisolone, she achieved complete remission from NS. The clinical course of this case supports the nephrosarca hypothesis regarding the mechanism of AKI caused by MCNS. Furthermore, appropriate fluid management and kidney biopsy are also important in elderly patients with AKI caused by NS.

scholarly journals Adult-onset nephrotic syndrome following COVID-19 vaccination

2021 ◽  
Author(s):  
Vivek Biradar ◽  
Abhijit Konnur ◽  
Sishir Gang ◽  
Umapati Hegde ◽  
Mohan Rajapurkar ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Complete Remission ◽  
Minimal Change Disease ◽  
Kidney Biopsy ◽  
Oral Prednisolone ◽  
Minimal Change ◽  
Adult Onset ◽  
Healthy Man

Abstract A 22-year-old healthy man was admitted for edema 15 days after the first injection of the COVISHIELD COVID-19 vaccine (Oxford AstraZeneca) vaccine. Nephrotic syndrome was diagnosed and a kidney biopsy showed minimal change disease. Oral Prednisolone was started at 1mg/kg/day resulting in complete remission within one week.

scholarly journals Case Report: Cyclophosphamide in COVID-19 – when an absolute contraindication is an absolute necessity

F1000Research ◽  
2021 ◽  
Vol 10 ◽  
pp. 829
Author(s):  
Kamila Bołtuć ◽  
Ada Bielejewska ◽  
Alejandro Coloma-Millar ◽  
Robert Dziugieł ◽  
Arkadiusz Bociek ◽  
...  
Keyword(s):  
Kidney Function ◽  
Kidney Biopsy ◽  
High Titer ◽  
Kidney Injury ◽  
Intravenous Immunoglobulins ◽  
Alveolar Haemorrhage ◽  
Laboratory Findings ◽  
Anca Vasculitis ◽  
Drug Of Choice ◽  
Threatening Condition

Background: Despite many studies on COVID-19, our knowledge of it remains incomplete. In some cases, treating SARS-CoV-2 infection concomitant with other diseases can be particularly challenging, as finding an appropriate treatment may involve some risks. Case presentation: A 34-year-old SARS-CoV-2 positive patient admitted due to fever, dyspnoea, haemoptysis and pneumonia, developed alveolar haemorrhage and acute kidney injury. Due to his severe state, abnormalities in laboratory tests and rapidly progressing loss of kidney function, kidney biopsy, as well as antibody panel were carried out, in which perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) were found with a high titer (>200; N: <1:20). The results of kidney biopsy, combined with clinical manifestation and laboratory findings prompted the diagnosis of rapidly progressing glomerulonephritis (RPGN) in the course of p-ANCA vasculitis. Initial treatment consisted of heamodialyses, remdesivir, plasmaphereses, intravenous immunoglobulins, antibiotics, corticosteroids and fraxiparine. Once the haemorrhage had subsided, kidney function had been partially retrieved and heamodialyses had no longer been necessary, cyclophosphamide treatment was initiated, despite being contraindicated in COVID-19 according to its summary of product characteristics. Immunotherapy is still continued. The patient has already received a total of 2.4g of cyclophosphamide (4 cycles of 600mg each every three weeks). Pulmonary and radiological regression, as well as improvement of renal parameters have been achieved.        Conclusions: We suspect that cyclophosphamide, the drug of choice in p-ANCA vasculitis, could be a potential factor providing regression of the radiological changes in the lungs and it could have prevented the patient from developing acute respiratory distress syndrome. COVID-19 diagnosis should not exclude searching for other diseases which can have a similar course. When treating a patient in a life-threatening condition, a departure from trying to find the perfect timing of cyclophosphamide delivery should be considered, as delaying it could cause potentially greater harm.

Impact of detecting plasma EGFR mutations with ultrasensitive liquid biopsy in outcomes of NSCLC patients treated with first- or second-generation EGFR-TKIs

2021 ◽  
pp. 1-13
Author(s):  
Oscar Arrieta ◽  
Juan-Manuel Hernández-Martínez ◽  
Edgar Montes-Servín ◽  
David Heredia ◽  
Andrés F. Cardona ◽  
...  
Keyword(s):  
Disease Progression ◽  
Clinical Characteristics ◽  
Detection Rate ◽  
Second Generation ◽  
Elevated Serum ◽  
Positive Finding ◽  
Response Evaluation ◽  
Mutational Status ◽  
Nsclc Patients ◽  
Egfr Tkis

BACKGROUND: Few trials have evaluated the utility of liquid biopsies to detect epidermal growth factor receptor mutations (EGFRm) at the time of response evaluation and its association with the clinical characteristics and outcomes of non-small-cell lung cancer (NSCLC) patients. OBJECTIVE: This study aimed to evaluate, in a real-world clinical setting, the prevalence of plasma EGFRm and its association with the clinical characteristics, response and survival outcomes of NSCLC patients under treatment with EGFR-tyrosine kinase inhibitors (EGFR-TKIs). METHODS: This observational study enrolled advanced or metastatic NSCLC patients, with confirmed tumor EGFRm, receiving treatment with first- or second-generation EGFR-TKIs. Blood samples for the detection of plasma EGFRm were collected at the time of response evaluation and processed using the Target Selector™ assay. The main outcomes were the detection rate of plasma EGFRm, median Progression-Free Survival (PFS) and Overall Survival (OS) according to plasma EGFR mutational status. RESULTS: Of 84 patients, 50 (59.5%) had an EGFRm detected in plasma. After a median follow-up of 21.1 months, 63 patients (75%) had disease progression. The detection rate of plasma EGFRm was significantly higher in patients with disease progression than in patients with partial response or stable disease (68.3% versus 33.3%; P< 0.01). PFS and OS were significantly longer in patients without plasma EGFRm than among patients with plasma EGFRm (14.3 months [95% CI, 9.25–19.39] vs 11.0 months [95% CI, 8.61–13.46]; P= 0.034) and (67.8 months [95% CI, 39.80–95.94] vs 32.0 months [95% CI, 17.12–46.93]; P= 0.006), respectively. A positive finding in LB was associated with the presence of ⩾ 3 more metastatic sites (P= 0.028), elevated serum carcinoembryonic (CEA) at disease progression (P= 0.015), and an increase in CEA with respect to baseline levels (P= 0.038). CONCLUSIONS: In NSCLC patients receiving EGFR-TKIs, the detection of plasma EGFRm at the time of tumor response evaluation is associated with poor clinical outcomes.

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.

Serum uric acid levels and risk of prehypertension: a meta-analysis

2017 ◽  
Vol 55 (3) ◽  
Author(s):  
Menglin Jiang ◽  
Dandan Gong ◽  
Yu Fan
Keyword(s):  
Uric Acid ◽  
Serum Uric Acid ◽  
Meta Analysis ◽  
Elevated Serum ◽  
China National Knowledge Infrastructure ◽  
Cross Sectional ◽  
Knowledge Infrastructure ◽  
Increased Risk ◽  
Cross Sectional Studies ◽  
The Relationship

AbstractElevated serum uric acid (SUA) levels may increase the risk of prehypertension. However, the findings from these studies remain conflicting. The objective of this study was to determine the relationship between SUA levels and risk of prehypertension by conducting a meta-analysis. We conducted a comprehensive literature search of PubMed, Embase, China National Knowledge Infrastructure, VIP, and the Wangfang database without language restrictions through May 2015. Observational studies assessing the relationship between SUA levels and prevalence of prehypertension were included. Pooled adjust odds ratio (OR) and corresponding 95% confidence intervals (CI) of prehypertension were calculated for the highest vs. lowest SUA levels. Prehypertension was defined as systolic blood pressure (BP) ranging from 120 to 139 mmHg or diastolic BP ranging from 80 to 89 mmHg. Eight cross-sectional studies with a total of 21,832 prehypertensive individuals were included. Meta-analysis showed that elevated SUA levels were associated with increased risk of prehypertension (OR: 1.84; 95% CI: 1.42–2.38) comparing the highest vs. lowest level of SUA levels. Subgroup analyses showed that elevated SUA levels significantly increased the risk of prehypertension among men (OR: 1.60; 95% CI: 1.12–2.21) and women (OR: 1.59; 95% CI: 1.17–2.16). Elevated SUA levels are positively associated with the risk of prehypertension in the general population. However, more well-designed longitudinal studies are needed before a definitive conclusion can be drawn due to the cross-sectional studies included are susceptible to bias.

scholarly journals A patient with systemic lupus erythematosus and lupus nephritis: A 12-year follow-up

2011 ◽  
Vol 68 (8) ◽  
pp. 705-708
Author(s):  
Natasa Jovanovic ◽  
Jasmina Markovic-Lipkovski ◽  
Stevan Pavlovic ◽  
Biljana Stojimirovic
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Nephrotic Syndrome ◽  
Mycophenolate Mofetil ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Kidney Biopsy ◽  
Immunosuppressive Drugs ◽  
Systemic Lupus ◽  
Younger Women ◽  
The Right

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.

scholarly journals Lung adenocarcinoma and sequential antineutrophil cytoplasmic antibody-associated vasculitis: a case report

2021 ◽  
Vol 49 (2) ◽  
pp. 030006052199331
Author(s):  
Chun-Yang Zhang ◽  
Ran Miao ◽  
Wei Li ◽  
Hao-Yong Ning ◽  
Xiang-En Meng ◽  
...  
Keyword(s):  
Lung Cancer ◽  
Histological Examination ◽  
Crescentic Glomerulonephritis ◽  
Elevated Serum ◽  
Pulmonary Nodules ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Necrotizing Crescentic Glomerulonephritis ◽  
Transthoracic Biopsy ◽  
Nasal Inflammation ◽  
The Relationship

The relationship between antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and lung cancer remains unclear. A 66-year-old man presented with pulmonary nodules. Histological examination of a specimen from computed tomography-guided percutaneous transthoracic biopsy revealed adenocarcinoma. The patient was treated using cryoablation and systemic chemotherapy. Sixteen months later, the patient presented with fever, nasal inflammation, recurrent lung lesions, elevated serum creatinine levels, and high levels of ANCA. Histological examination of a specimen from ultrasound-guided percutaneous renal biopsy revealed pauci-immune necrotizing crescentic glomerulonephritis. The patient responded to treatment, but granulomatosis with polyangiitis recurred and he later died. This case highlights the possibility of sequential AAV with lung cancer. Although this is relatively rare, further research is needed to better understand the association or pathophysiological link between lung cancer and AAV.

scholarly journals Pattern of adverse effects in patients with nephrotic syndrome on oral prednisolone

2017 ◽  
Vol 6 (6) ◽  
pp. 1269
Author(s):  
Shankareswari S. ◽  
Jayapriya B. ◽  
Balamurugan P. V. ◽  
Lourdu Jafrin A. ◽  
Geetha K.
Keyword(s):  
Nephrotic Syndrome ◽  
Adverse Effects ◽  
Short Stature ◽  
Oral Prednisolone ◽  
Cross Sectional Study ◽  
Cross Sectional ◽  
Glucose Levels ◽  
Long Term Follow Up ◽  
Literature Evidence ◽  
Paediatric Age

Background: Nephrotic syndrome is a common illness affecting the paediatric age group and 80% of the idiopathic syndrome is steroid sensitive. Multiple relapses make them vulnerable to the adverse effects of corticosteroids. There is limited literature evidence for the adverse effects of steroids in children with renal pathology.Methods: This descriptive, cross sectional study analyses the adverse effects of oral prednisolone in children and adults with nephrotic syndrome Fifty-five patients with nephrotic syndrome, attending nephrology or paediatric OP, more than 3 years of age and who were on oral prednisolone for a minimum of eight weeks were included in the study. Demographic details, detailed history, lab investigations and ophthalmic examination were done and the results were analysed.Results: Hypertension and behavioural changes were the most common adverse effects followed by dermatological, endocrine and metabolic changes. Infections and gastrointestinal disturbances were more in adults (p <0.05). Short stature was more in children (p< 0.05). There was no abnormality in blood glucose levels and body weight. Hypertension, cushingoid habitus, infections and short stature were statistically less in patients on alternate day prednisolone. But no statistical association could be made between the occurrence of cataract and the pattern of prednisolone use.Conclusions: Adverse effects pattern is different among adults and children. Also, the adverse effects are less with alternate day prednisolone regimen. Long term follow up into their adulthood is needed to analyse the morbidity produced by corticosteroids in these subsets of population.

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