scholarly journals Lupus-like nephritis with positive anti-neutrophil cytoplasmic antibodies and negative antinuclear antibodies

2020 ◽  
Author(s):  
Joana Eugénio Santos ◽  
Rita Vicente ◽  
Beatriz Malvar ◽  
Iolanda Santos ◽  
Miguel Coimbra ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Antinuclear Antibodies ◽  
Kidney Biopsy ◽  
Kidney Injury ◽  
Steroid Treatment ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Small Vessel Vasculitis ◽  
Laboratory Findings ◽  
Clinical Criteria ◽  
Immunological Markers

Abstract Antineutrophil cytoplasmic antibodies (ANCAs) are associated with small vessel vasculitis but their prevalence is not rare in other immune diseases. In lupus nephritis (LN), their pathological role and clinical relevance have been the target of controversial views. We present a case of acute kidney injury and nephrotic syndrome in a young woman with diffuse global proliferative and membranous nephritis on her kidney biopsy, showing a full-house immunofluorescence pattern, very allusive of class IV + V LN, but lacking associated clinical criteria and laboratory findings to support the diagnosis of systemic lupus erythematosus (SLE). Furthermore, the patient presented with high titers of ANCA, steadily decreasing alongside the renal function and proteinuria improvements, with mycophenolate mofetil (MMF) and steroid treatment. The authors believe this is a case of lupus-like nephritis, in which ANCAs are immunological markers, although they are not directly involved in the pathogenesis.

scholarly journals Acute interstitial nephritis and drug-induced systemic lupus erythematosus due to chlorthalidone and amiodarone: A case report

2020 ◽  
Vol 8 ◽  
pp. 2050313X2091002 ◽  
Author(s):  
Umut Selamet ◽  
Ramy M Hanna ◽  
Anthony Sisk ◽  
Lama Abdelnour ◽  
Lena Ghobry ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Interstitial Nephritis ◽  
Lupus Erythematosus ◽  
Kidney Biopsy ◽  
Kidney Injury ◽  
Acute Interstitial Nephritis ◽  
Systemic Lupus ◽  
Drug Induced ◽  
Systemic Manifestations

Drug-induced lupus erythematosus has features distinct from primary systemic lupus erythematosus. It can occur with a wide variety of agents that result in the generation of anti-histone or other types of antibodies. Systemic manifestations of drug-induced systemic lupus erythematosus may include renal dysfunction due to circulating immune complexes or due to other immune reactions to the culprit medication(s). Acute interstitial nephritis occurs due to DNA–drug or protein–drug complexes that trigger an allergic immune response. We report a patient who developed acute kidney injury, rash, and drug-induced systemic lupus diagnosed by serologies after starting chlorthalidone and amiodarone. A renal biopsy showed acute interstitial nephritis and not lupus-induced glomerulonephritis. It is important to note that systemic lupus erythematosus and acute interstitial nephritis can occur together, and this report highlights the role of the kidney biopsy in ascertaining the pathological diagnosis and outlining therapy in drug-induced lupus erythematosus.

scholarly journals Urine Biomarkers to Predict Response to Lupus Nephritis Therapy in Children and Young Adults

2017 ◽  
Vol 44 (8) ◽  
pp. 1239-1248 ◽  
Author(s):  
Hermine I. Brunner ◽  
Michael R. Bennett ◽  
Gaurav Gulati ◽  
Khalid Abulaban ◽  
Marisa S. Klein-Gitelman ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Transforming Growth Factor ◽  
Kidney Biopsy ◽  
Characteristic Curve ◽  
Kidney Injury ◽  
Transforming Growth Factor Β ◽  
Response To Therapy ◽  
Spot Urine ◽  
Urine Biomarkers

Objective.To delineate urine biomarkers that forecast response to therapy of lupus nephritis (LN).Methods.Starting from the time of kidney biopsy, patients with childhood-onset systemic lupus erythematosus who were diagnosed with LN were studied serially. Levels of 15 biomarkers were measured in random spot urine samples, including adiponectin, α-1-acid glycoprotein (AGP), ceruloplasmin, hemopexin, hepcidin, kidney injury molecule 1, monocyte chemotactic protein-1, lipocalin-like prostaglandin D synthase (LPGDS), transforming growth factor-β (TGF-β), transferrin, and vitamin D binding protein (VDBP).Results.Among 87 patients (mean age 15.6 yrs) with LN, there were 37 treatment responders and 50 nonresponders based on the American College of Rheumatology criteria. At the time of kidney biopsy, levels of TGF-β (p < 0.0001) and ceruloplasmin (p = 0.006) were significantly lower among responders than nonresponders; less pronounced differences were present for AGP, hepcidin, LPGDS, transferrin, and VDBP (all p < 0.05). By Month 3, responders experienced marked decreases of adiponectin, AGP, transferrin, and VDBP (all p < 0.01) and mean levels of these biomarkers were all outstanding (area under the receiver-operating characteristic curve ≥ 0.9) for discriminating responders from nonresponders. Patient demographics and extrarenal disease did not influence differences in biomarker levels between response groups.Conclusion.Low urine levels of TGF-β and ceruloplasmin at baseline and marked reduction of AGP, LPGDS, transferrin, or VDBP and combinations of other select biomarkers by Month 3 are outstanding predictors for achieving remission of LN. If confirmed, these results can be used to help personalize LN therapy.

Immunological investigation of the patient with renal disease

2015 ◽  
Author(s):  
Jo H. M. Berden ◽  
Jack F. M. Wetzels
Keyword(s):  
Renal Disease ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Antinuclear Antibodies ◽  
Immunoglobulin A ◽  
Rapidly Progressive Glomerulonephritis ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Systemic Lupus ◽  
Laboratory Techniques ◽  
Immunological Investigation

Laboratory techniques (electrophoresis, indirect immunofluorescence, ELISA, and immunoblotting) required for immunological investigation of the patient with renal disease are described. Renal disease-related aspects of immunoglobulins (immunoglobulin A, paraproteins, cryoglobulins), complement, antinuclear antibodies, anti-C1q antibodies, antineutrophil cytoplasmic antibodies, anti-glomerular basement membrane antibodies, antipodocyte antibodies, antiphospholipid antibodies, and antimicrobial responses (streptococci, hepatitis C, hepatitis B) are reviewed. Laboratory assays which evaluate the immune response, in particular the identification of (auto)-antibodies are valuable tools in establishing a diagnosis and/or monitoring of the activity of the disease. Guidelines are given for immunological studies in patients with specific renal syndromes including nephrotic syndrome, rapidly progressive glomerulonephritis, systemic lupus erythematosus, and thrombotic microangiopathy.

P0466LUPUS NEPHRITIS IN SOUTH MOROCCO OVER 6 YEARS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Ramia BENHAMOU ◽  
Allibou Sara ◽  
Mariem Yahioui ◽  
Meriem Chettati ◽  
Wafaa Fadili ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Antinuclear Antibodies ◽  
Kidney Biopsy ◽  
Inclusion Criteria ◽  
Systemic Lupus ◽  
High Incidence ◽  
Neurological Features ◽  
The Moment ◽  
Cardiac Manifestations

Abstract Background and Aims Lupus nephritis is one of the most serious manifestation of systemic lupus. The study aimed at assessing the epidemiology and clinical features of systemic lupus erythematosus with lupus nephritis (LN) in south Morocco. Method A retrospective study was conducted in the Marrakesh-Tensift region by charts review from 2014 to 2019 (included). Patients with a SLE diagnosed with lupus nephritis over 6 years according to ACR/EULAR criteria were included. Results In total, 146 patients met inclusion criteria: 133 were women (94%) and mean age at diagnosis was 31,195 ± 13.5 years. Five percent of all patients had a least one parent with autoimmune disease, and 4,1% with lupus. The main manifestations were musculoskeletal (89%) and mucocutaneous with acute or subacute lesions (68,5%), alopecia (50%), ulcers (3,4%) and discoid lupus (10,27%). Cardiac manifestations in 19,2% and neurological features in 17,9% and psychiatric features in 13,7%. At the moment of diagnosis antinuclear antibodies were positive in 89% (threshold &gt;1/80), and associated to anti-DNA in 91 patients (62,3%), anti-SSA (21,2%), and anti-Sm (34,9%). APL syndrome was diagnosed in 8,2%. Kidney biopsy showed predominantly class IV in 54% and classe I-II in 28%. The average annual standardized incidence of LN between 2014 and 2019 was 2,4 cases per 1,000,000 inhabitants (95% confidence interval [CI]: 5.0–6.0). The prevalence was 21 cases per 1,000,000 inhabitants in 2018 (95% CI: 70–79). Conclusion Lupus nephritis in the multi-ethnic population of south moroccan is characterized by high incidence and high rates of diffuse proliferative nephritis.

Characteristics of lupus nephritis in Saudi lupus patients: A retrospective observational study

Lupus ◽  
2020 ◽  
Vol 29 (12) ◽  
pp. 1638-1643
Author(s):  
Majed R Albirdisi ◽  
Ibrahim A Al-Homood
Keyword(s):  
Saudi Arabia ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Kidney Biopsy ◽  
Childbearing Age ◽  
Systemic Lupus ◽  
Laboratory Findings ◽  
Medical City ◽  
Systemic Disorder ◽  
End Stage

Objectives Systemic lupus erythematosus (SLE) is a chronic autoimmune multi-systemic disorder of the connective tissue, characterized mainly by involvement of the skin, joints, kidneys, and serosal membranes. It affects females particularly at childbearing age more commonly than males. Lupus nephritis affects around half of patients with SLE. Data about SLE and lupus nephritis in Saudi Arabia are still scarce. In this study, we aimed to evaluate the prevalence, clinical and laboratory findings of SLE and different histological types of lupus nephritis among Saudi patients at King Fahad Medical City. Methods This is a retrospective study for adult patients who have been evaluated at king Fahad medical city between 2014 and 2019 and fulfilled the Systemic Lupus International Collaborating Clinics classification criteria (SLICC). Results 112 patients, 103 (92%) females and 9 (8%) males, with confirmed diagnoses of SLE were reviewed. Skin rash (69.6%), photosensitivity (61.6%), mucosal ulcerations (45.9%), arthralgia and/or arthritis (44.6%) are the most common clinical features. Ninety seven (86.6%) out of 112 patients had a recorded first visit 24 hour urine protein level, out of those only 26 (23.2) patients presented with significant proteinuria of more than 0.5grams per day. Forty four (39.2%) have undergone kidney biopsy. Class IV and III lupus nephritis are the most common reported biopsy results (43.18% and 27.28% respectively). During the study period, three patients (2.7%) developed end-stage kidney disease requiring dialysis and five (4.5%) had renal transplant. Conclusion Our study provided insight on the demographics, characteristics and presentation of SLE patients and the outcome of lupus nephritis in Saudi Arabia.

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.

scholarly journals Successful Treatment of Catastrophic Antiphospholipid Syndrome Using Rituximab: Case Report and Review of the Literature

Medicina ◽  
2021 ◽  
Vol 57 (9) ◽  
pp. 912
Author(s):  
Cristina Stanescu ◽  
Andreea Gabriella Andronesi ◽  
Ciprian Jurcut ◽  
Mihaela Gherghiceanu ◽  
Alexandra Vornicu ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Kidney Biopsy ◽  
Current Knowledge ◽  
Kidney Injury ◽  
Clotting Factor ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Case Presentation ◽  
Kidney Involvement ◽  
Head Computed Tomography

Background: Kidney involvement is a frequent complication of systemic lupus erythematosus (SLE) and kidney biopsy is essential in differentiating lupus nephritis (LN) from thrombotic microangiopathy (TMA) secondary to antiphospholipid autoantibodies (aPL). Association between antiphospholipid syndrome (APS) and acquired hemophilia due to inhibitors was very rarely described in SLE patients. Case presentation: We present the case of a 61-year-old male diagnosed with SLE who acquired deficiency of clotting factor VIII due to circulating inhibitors, admitted for acute kidney injury (AKI), microangiopathic hemolytic anemia, thrombocytopenia, and diplopia. Kidney biopsy showed TMA due to APS, but no signs of LN. Head computed tomography identified low dense areas in the white matter, suggesting small blood vessels’ involvement. A diagnosis of probable catastrophic antiphospholipid syndrome (CAPS) was established and treatment with low molecular weight heparin, intravenous methylprednisolone, plasmapheresis, and rituximab was initiated, followed by resolution of AKI, diplopia, and TMA with complete depletion of CD19+B-lymphocytes (CD19+B-Ly) after one month. We further review the current knowledge regarding pathogenesis and management of CAPS in SLE patients. Conclusions: Targeted therapy was possible after kidney biopsy, improving renal and general prognosis. CD19+B-Ly repopulation preceded biological relapse, so monitoring of CD19+B-Ly may serve as a tool to predict relapses and guide rituximab therapy.

Organized Intraglomerular Deposits in NZB Mouse Disease

1971 ◽  
Vol 29 ◽  
pp. 544-545
Author(s):  
Francis R. Comerford ◽  
Alan S. Cohen
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antinuclear Antibodies ◽  
Human Systemic Lupus Erythematosus ◽  
Systemic Lupus ◽  
Glomerular Lesion ◽  
Ultrastructural Studies ◽  
Dense Deposits ◽  
Experimental Nephritis ◽  
Glomerular Lesions

Mice of the inbred NZB strain develop a spontaneous disease characterized by autoimmune hemolytic anemia, positive lupus erythematosus cell tests and antinuclear antibodies and nephritis. This disease is analogous to human systemic lupus erythematosus. In ultrastructural studies of the glomerular lesion in NZB mice, intraglomerular dense deposits in mesangial, subepithelial and subendothelial locations were described. In common with the findings in many examples of human and experimental nephritis, including many cases of human lupus nephritis, these deposits were amorphous or slightly granular in appearance with no definable substructure.We have recently observed structured deposits in the glomeruli of NZB mice. They were uncommon and were found in older animals with severe glomerular lesions by morphologic criteria. They were seen most commonly as extracellular elements in subendothelial and mesangial regions. The deposits ranged up to 3 microns in greatest dimension and were often adjacent to deposits of lipid-like round particles of 30 to 250 millimicrons in diameter and with amorphous dense deposits.

Correlation Between Serological Makers and Immunofluorescence Deposits i n Kidney Tissue of Patients with Lupus Nephritis

2019 ◽  
Vol 9 (02) ◽  
Author(s):  
Haider S Al-Hadad ◽  
Aqeel Abbas Matrood ◽  
Maha Abdalrasool Almukhtar ◽  
Haider Jabur Kehiosh ◽  
Riyadh Muhi Al-Saegh
Keyword(s):  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Kidney Biopsy ◽  
Pearson Correlation ◽  
Standard Procedure ◽  
Kidney Tissue ◽  
P Value ◽  
American College Of Rheumatology ◽  
Immune Deposits ◽  
Number Of Patients

Background: Systemic lupus erythematosus (SLE) is an autoimmune disease. Few biomarkers for SLE have been validated and widely accepted for the laboratory follow-up of inflammatory activity. In SLE patients, with lupus nephritis (LN), complement activation leads to fluctuation of serum C3 and C4 that are frequently used as clinicalm biomarker of disease activity in SLE. Patients and Methods: In this study the number of patients were 37, seven patients were excluded for incomplete data collection, 28 were females ,2 were males. The duration of the study is two years from 2015 to 2017. Patients were considered to have SLE and LN according to American College of Rheumatology (ACR) criteria, and International Society of Nephrology/ Renal Pathology Society (ISN/RPS). All patients were evaluated withm clinical presentation, laboratory investigations. Our patients underwent kidney biopsy according to standard procedure by Kerstin Amann, and their tissue specimens were studied in the laboratory with light microscope (LM) and immunofluorescence microscope reagents. The relationship between the serological markers and immunofluorescence deposits in kidney biopsy of all patients were studied using the statistical analysis of Pearson correlation and single table student's T test. A P value 0.05 was considered statistically significant. Results: The granular pattern of IF deposits was present in all LN patients, and in more than two third of patients these IF deposits presented in glomerular, tubular, and mesangium sites. While less than one third of patients had IF deposits in the mesangium only. There was no statistically significant correlation between serum ANA, anti-dsDNA, and IF deposits of different types. There was significant correlation between serum C3 and C4 hypocomplementemia and IgG immune deposits in kidney biopsy, and there was significant relationship between serum C3 hypocomplementemia and full house immunofluorescence (FHIF) deposits inm kidney biopsy.Conclusions:Immunofluorescence deposits is mainly granular pattern in LN patients. There was no significant association between serum ANA, anti-dsDNA, and immune deposits in kidney tissue. Immunofluorescence deposits of IgG type correlates significantly with serum C3 and C4 hypocomplemetemia, and these immune deposits in association with low complement levels correlates with LN flare. There was significant correlation between C3 hypocomplementemia and FHIF.

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