Henoch-Schönlein purpura outcome in children: A ten-year clinical study

Introduction. Henoch-Sch?nlein purpura (HSP) is the most common vasculitis of childhood. It is characterized by symptoms including nonthrombocytopenic purpura, abdominal pain, haematuria/proteinuria, and arthralgia/arthritis. The pleiomorphism of clinical signs in HSP could be confused with other conditions or other vasculitis forms. Objective. Evaluation of HSP clinical presentation, the onset and severity of renal manifestation in affected children and their outcome. Methods. A retrospective study of 49 patients diagnosed with HSP was conducted from September 1999 to September 2009. Children with severe renal manifestations (nephrotic range proteinuria, with or without nephrotic or nephritic syndrome) have undergone kidney biopsy. Results. Twenty-five patients developed renal manifestations after onset of the disease. In our study child?s older age was a risk factor for association with HSP nephritis. Six of the patients required kidney biopsy. They were successfully treated with various immunosuppressive protocols, as well as three of nine patients with nephrotic range proteinuria. Two patients developed most severe form of HSP nephritis, nephrotic-nephritic syndrome with histology grade IIIb/IVb. During the study period (average follow-up 6 years), all patients had a normal global renal function with mild proteinuria in only two cases. The prognosis of renal involvement was better than reports from other patient series. Conclusion. Long-term morbidity of HSP is predominantly attributed to renal involvement. During the study period, no patient had renal insufficiency or end stage renal disease after various combinations of immunosuppressive treatment. It is recommended that patients with HSP nephritis are followed for longer periods of time with a regular measurement of renal function and proteinuria.

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P0498CLINICAL PRESENTATION AND RENAL HISTOPATHOLOGICAL FINDINGS IN PATIENTS WITH MONOCLONAL GAMMOPATHY

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p0498 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Gaetano Alfano ◽

Alice Delrio ◽

Francesco Fontana ◽

Annachiara Ferrari ◽

Andrea Solazzo ◽

...

Keyword(s):

Renal Function ◽

Monoclonal Gammopathy ◽

Kidney Biopsy ◽

Renal Involvement ◽

M Protein ◽

Histological Evaluation ◽

Al Amyloidosis ◽

Creatinine Ratio ◽

Urine Protein ◽

Cast Nephropathy

Abstract Background and Aims Monoclonal gammopathy is associated with renal lesions due to the toxic effect of M-protein. The aim of our study was to evaluate the prevalence and the clinical presentation of monoclonal gammopathy in patients who underwent kidney biopsy for renal impairment. Method We conducted a retrospective study at the Nephrology and Dialysis Unit of the University Hospital of Modena from 2005 to 2017. The diagnosis of renal disease was proved histologically. Results Monoclonal gammopathy was found in 179 out of 1334 patients (13.4%). Mean age was 66.1±13.4 years with a predominance of males (63.7%). There was no differences (P=0.16) between the age of patients with benign (64.9±14.3) and malignant lymphoproliferative diseases (67.6±12). The hematologic disorders involved in the production of M-protein were MGUS (51.9%), myeloma multiple (MM) (25.7%), amyloidosis (8.9%) smoldering MM (5 %), non-Hodgkin lymphoma (NHL) (6.7%) and HL (1.7%). The prevalence of MGUS was estimated to be 6.97% (93/1334). Mean serum creatinine was 2.68±2.12 mg/dl (eGFR of 35.24±29.32 ml/min) and urine protein/creatinine ratio of 5.1±6.5. None of the study subjects progressed to MM or other lymphoproliferative diseases. The most common kidney disease was membranoproliferative (GN) (19.3%). MGRS has been identified in five patients (5.4%). Mean age of MM was 66.84±13 years. Serum concentration M-protein was 1.47±0.98. Among patients with AKI (89.1%), 13 patients (28.2%) required urgent hemodialysis. Histological evaluation showed cast nephropathy (71.7%), MM-associated AL amyloidosis (15.2%), MM-associated-light chain deposition disease (4.3%) and interstitial nephritis (8.7%). Nine patients had a diagnosis of smoldering MM. Average age was 69.17±10.8 years old. At presentation, creatinine was 2.31±2.6 mg/dl (GFR of 41.35 ml/min). Evaluation of renal biopsies allowed us to identify different patterns of glomerular diseases, expression of an aspecific renal involvement of this hematological disease. AL amyloidosis was secondary to MGUS (75%) and smoldering MM (33%). Mean age was 66.04±11.7 years old. At presentation mean urine protein/creatinine ratio was 8.33±3.2 concomitant to a serum albumin level of 2.74±0.84 gr/dl. Mean creatinine was 1.4 mg/dl corresponding to eGFR= 56.5 ml/min. Average age of NHL patients was 72.6±9.6 years. Renal function was extremely variable at presentation; mean creatinine was 2.4±1.6 mg/dl (eGFR of 30.4±22.7 ml/min). Histological evaluation of biopsy specimen revealed amyloidosis (25%), cryoglobulinemic GN (25%), LCDD (16.6%), cast-nephropathy (8.3%), LCDD (8.3%) and other renal diseases (16.8%). Three patients (1.12%) had a diagnosis of HL at mean age of 69.04±5.3 years. At presentation, renal function was normal in all patients with a creatinine of 0.93±0.07mg/dl (eGFR of 62.7.3±7.4 ml/min). Urine protein/creatinine ratio was 0.3±0.2. Kidney biopsy revealed cryoglobulinemic GN (75%) and hypertensive nephrosclerosis (25%). ANOVA analysis did not found statistically significant differences in age (p=0.11) and serum concentration of M-protein (P=0.42) between groups. The differences in mean serum creatinine and mean urine protein/creatinine ratio were statistically significant between groups, (P<0.0001) and (P=0.003), respectively. A post hoc Tukey test showed that proteinuria was higher in AL amyloidosis compared to MM and HL, whereas renal function was worse in MM patients compared to the others. Conclusion MGUS was the most common monoclonal gammopathy. Surprisingly, it is frequently associated with membranoproliferative glomerulonephritis. MGRS is a rare histopathological entity (5.4%). MM manifests frequently with AKI whereas AL amyloidosis with nephrotic syndrome. Renal function was extremely variable in NHL patients; on the other hand, the limited number of HL patients with renal involvement in our cohort does not allow generalization of our findings.

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Fingertips Ischemia, Nephroangiosclerosis, and Focal Segmental Glomerulosclerosis: Is Genetic Thrombophilia the Unique Explanation?

Case Reports in Medicine ◽

10.1155/2014/832592 ◽

2014 ◽

Vol 2014 ◽

pp. 1-4

Author(s):

Lisa Giovannini ◽

Carlo Donadio

Keyword(s):

Renal Function ◽

Methylenetetrahydrofolate Reductase ◽

Kidney Biopsy ◽

Clinical Signs ◽

Plasminogen Activator Inhibitor ◽

Plasminogen Activator Inhibitor 1 ◽

Case Presentation ◽

Previous Diagnosis ◽

Prothrombin Gene ◽

Activator Inhibitor

Case Presentation. 53-years-old-man with essential hypertension and nonnephrotic proteinuria (1.3 gr/24 h) and with normal renal function (eGFR-MDRD 123 mL/min/1.73 m2) was admitted to nephrology department; kidney biopsy showed FSGS; two years later the patient presented with ulceration and ischemic gangrene of the IV and V right-hand fingertips; genetic analysis demonstrated polymorphism of the methylenetetrahydrofolate reductase genes C677T (heterozygote C677T/1298AC with normal value of homocysteine) and mutations of prothrombin gene G20210A and of plasminogen activator inhibitor-1 4G/5G 675 with slight increase of its value. After five years from biopsy, 24-hours proteinuria was still around 1–1.3 g/die; renal function was still normal (eGFR 107 ml/min/1.73 m2). These data are against the previous diagnosis of primary FSGS. We hypothesize that genetic thrombophilia may explain all the clinical signs of our patient.Conclusions. Alterations in genes of thrombophilia should be ruled out in patients with bioptic diagnosis of “primary” FSGS, in particular if clinically atypical.

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HIV Infection with Membranous Nephropathy in a Low HIV Prevalent Muslim Country, Bangladesh: A Case Report

Journal of Medicine ◽

10.3329/jom.v16i1.22408 ◽

2015 ◽

Vol 16 (1) ◽

pp. 61-63

Author(s):

Tabassum Samad ◽

Wasim Md Mohosin Ul Haque ◽

Mehruba Alam Ananna ◽

Muhammad Abdur Rahim ◽

Sarwar Iqbal

Keyword(s):

Human Immunodeficiency Virus ◽

Renal Function ◽

Case Report ◽

Hiv Infection ◽

Membranous Nephropathy ◽

Infected Patient ◽

Muslim Country ◽

Renal Manifestation ◽

Immunodeficiency Virus ◽

Nephrotic Range Proteinuria

The most common renal manifestation of Human immunodeficiency virus (HIV), is HIV associated nephropathy (HIVAN). In this report, we describe a case that was referred for evaluation of proteinuria. Diagnostic workup revealed HIV infection with membranous nephropathy (MN). As he had sub-nephrotic range proteinuria and normal renal function we did not start any treatment for membranous nephropathy and for anti-retroviral therapy he was sent to a referral center. Being an uncommon variety of nephropathy in HIV infected patient in one of the lowest HIV prevalent country, we are reporting the case.DOI: http://dx.doi.org/10.3329/jom.v16i1.22408 J MEDICINE 2015; 16 : 61-63

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Nierenbeteiligung bei ANCA-assoziierten Vaskulitiden

DMW - Deutsche Medizinische Wochenschrift ◽

10.1055/s-0043-106566 ◽

2018 ◽

Vol 143 (02) ◽

pp. 79-88 ◽

Cited By ~ 2

Author(s):

Marion Haubitz

Keyword(s):

Renal Function ◽

Maintenance Treatment ◽

Renal Involvement ◽

Young Patients ◽

Induction Treatment ◽

Renal Manifestation ◽

Increased Risk ◽

Stage Renal Disease ◽

End Stage

AbstractIn patients with ANCA-associated vasculitis renal involvement is frequently seen and the severity of renal manifestation is very important for therapeutic strategies and prognosis. Clinically rapid loss of renal function, nephritic sediment and proteinuria in a non-nephrotic range are characterizing a focal segmental necrotizing pauci-immune glomerulonephritis with extrarenal proliferations. Induction treatment depends on the severity of manifestations. With a normal renal function methotrexate can be used in combination with steroids. In patients with organ threatening involvement but creatinine below 500 µmol/l cyclophosphamide pulses or Rituximab should be used together with steroids, initially with i. v. pulses. Rituximab is more effective in PR3-ANCA vasculitis and should be used in relapsing disease, in young patients to avoid gonadal toxicity and in patients with an increased risk of malignancies. In patients on dialysis or with creatinine > 500 µmol/l plasma exchange should be added. Maintenance treatment (mainly with azathioprine) is necessary as at least 50 % of the patients develop relapses. Rituximab seems more effective, however it is not approved for maintenance treatment and no long-term data are available. Adjuvant treatment, long-term side effects and the increased incidence of cardiovascular events have to be included in the follow-up of vasculitis patients. In end-stage renal disease patients relapses occur but are more difficult to diagnose and treat with higher incidence of infections. Transplantation should be offered as patient and transplant survival is good.

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Nephrotic-range proteinuria in a patient with dengue fever: a case report from Bangladesh

IMC Journal of Medical Science ◽

10.3329/imcjms.v12i2.39667 ◽

2019 ◽

Vol 12 (2) ◽

pp. 86-89 ◽

Cited By ~ 1

Author(s):

Shapur Ikhtaire

Keyword(s):

Renal Function ◽

Nephrotic Syndrome ◽

Dengue Fever ◽

Renal Involvement ◽

Dengue Infection ◽

Specific Treatment ◽

Severe Dengue ◽

Urine Protein ◽

Nephrotic Range Proteinuria

We report a case of nephrotic range proteinuria with 24-hour urine protein level of 18.3 g/day, which developed following dengue fever (DF). The patient did not exhibit classical features of nephrotic syndrome (NS) and his renal function was not compromised during his illness. Proteinuria resolved without any specific treatment and precluded renal biopsy. Though the dengue fever and associated renal disorders are self-limiting, renal involvement in severe dengue infection is growingly seen in recent years and could cause increased mortality and long-term morbidity. IMC J Med Sci 2018; 12(2): 86-89

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AB1234 MICRO-RNA 155 AND MIR-34A: POSSIBLE BIOMARKERS OF INFLAMMATORY BURDEN AND DISEASE ACTIVITY IN ANCA-ASSOCIATED VASCULITIS WITH RENAL INVOLVEMENT

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.6011 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1908.2-1908

Author(s):

D. Bruno ◽

P. G. Cerasuolo ◽

C. Di Mario ◽

S. L. Bosello ◽

L. Gigante ◽

...

Keyword(s):

Renal Function ◽

Kidney Biopsy ◽

Urine Analysis ◽

Renal Involvement ◽

Kidney Tissue ◽

Tissue Expression ◽

Micro Rna ◽

Renal Outcome ◽

Markers Of Inflammation ◽

Inflammatory Burden

Background:Predicting clinical outcomes in ANCA-related glomerulonephritis remains a major challenge. To date, there is no reliable biomarker able to predict renal prognosis in patients with ANCA-associated vasculitis (AAV). Micro-RNA (miRNA) are non-coding RNAs involved in the fine tuning of immune cells biology and this epigenetic modulation associates with different phenotypes and prognosis in several diseases.Objectives:To investigate the expression of miR-155 and miR-34a in kidney biopsies of AAV patients according with renal outcome.Methods:Fifteen patients with AAV and renal involvement (mean age 63.0 ±13.3 years, disease duration 4.9±2.2 months), who underwent renal biopsy. Demographic, clinical and autoimmune parameters were recorded for each patient. Each kidney biopsy was classified according to the Berden Classification, Risk group (according to the ANCA Renal Risk Score) and the chronicity Classification of the Mayo Clinic’s proposed score.MiR-155 and miR-34a expression was investigated on kidney biopsy tissue using the miRNeasy FFPE kit (Qiagen). The quantitative expression of miR-155, miR-34a and housekeeping gene U1, used as control, was assessed by Real Time-PCR. MiR-155 and miR-34a expression was correlated with histopathological and clinical-laboratory parameters.Each patient was followed for 12 months and renal outcome was considered according toKDIGO CKDClassification. Markers of inflammation (ESR, CRP) and urine analysis data were recorded at baseline and after 12 months.Results:Six (40%) patients were p-ANCA positive and 9 (60%) c-ANCA positive. Eight patients (53%) also had pulmonary involvement. The mean baseline GFR was 30.7±28.8 ml/min/1.73 m2and 10 patients (66%) showed an active urinary sediment.At disease onset, the mean expression of miR-155 was 9.5±21.1, while the expression of mir-34a was 13.1±46.2. Considering the autoimmune profile, kidney tissue of p-ANCA positive patients was enriched of mir-155 (19.6±30.6 fold) compared to c-ANCA positive patients (1.9±2.9 fold; p=0.001). Particularly, considering the renal function, kidney tissue of patients with greater impairment of renal function (KDIGO stage 5) was enriched of miR-155 (21.5±38.3 fold) compared to patients with less renal impairment (KDIGO stage 1-4) (4.72±8.16 fold, p=0.004).Tissue expression of miR-155 and miR-34a did not correlated with the abovementioned histopathological classifications.After 12 months from kidney biopsy, 3(20%) patients had a worsening of renal function, 5 (33%) still presented elevated markers of inflammation and 3 (20%) still had proteinuria at urine analysis. At baseline, kidney tissue of patients with higher CRP plasma levels and proteinuria at follow-up presented higher expression of miR-155 (p=0.002 and p=0.001), whereas no significant differences were found about miR-34a kidney tissue expression.Conclusion:MiRNAs may play a potential role in the pathogenesis of ANCA-related glomerulonephritis. MiR-155 kidney enrichment seems to mirror the disease inflammatory burden and activity at the onset and after 12 months representing a possible biomarker in ANCA vasculitis with renal involvement. This finding may represent the basis for further studies on miRNA expression in blood samples, aiming to identify a non-invasive biomarker of kidney damage, predicting disease’s relapses and patients’ prognosis.References:[1]Renauer et al, Clin Rev Allergy Immunol. 2016Disclosure of Interests:Dario Bruno: None declared, Pier Giacomo Cerasuolo: None declared, Clara Di Mario: None declared, Silvia Laura Bosello Speakers bureau: Abbvie, Pfizer, Boehringer, Laura Gigante: None declared, Alessia Musto: None declared, Gisella Vischini: None declared, Stefano Costanzi: None declared, Stefano Alivernini: None declared, Barbara Tolusso: None declared, Giuseppe Grandaliano: None declared, Elisa Gremese Speakers bureau: Abbvie, BMS, Celgene, Jannsen, Lilly, MSD, Novartis, Pfizer, Sandoz, UCB

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Renal Involvement and Outcome In Monoclonal Lightchain Disease

Blood ◽

10.1182/blood.v116.21.4985.4985 ◽

2010 ◽

Vol 116 (21) ◽

pp. 4985-4985

Author(s):

Raoul Bergner ◽

Michael J. Uppenkamp ◽

Martin Hoffmann

Keyword(s):

Renal Function ◽

Kidney Disease ◽

Renal Disease ◽

Light Chain ◽

Kidney Biopsy ◽

Renal Involvement ◽

The Other ◽

Renal Outcome ◽

Al Amyloidosis ◽

Light Chain Disease

Abstract Abstract 4985 Background: Renal involvement is very common in monoclonal light chain disease (mLCD). However, the types of renal disease are manifold. Also the prognosis and outcome is very different. We analysed the data and renal outcome of patients with monoclonal light chain disease and renal disease. Methods: We analysed the data of patients with monoclonal light chain disease, who underwent a kidney biopsy due to proteinuria and/or renal insufficiency of unclear origin, retrospectively. Data of renal function, proteinuria, the type of renal disease and the renal outcome were collected. The mLCD were subclassified in multiple myeloma (MM), AL-amyloidosis (ALA), monoclonal gammopathy of unclear significance (MGUS) and B-cell non hodgkin lymphona (B-NHL). The kidney biopsy findings were classified in cast nephropathy (CN), ALA, light chain deposit disease (LCDD) and other renal disease (ORD). Results: 88 patients were included in the analysis. 47 patients suffered from MM, 15 from systemic ALA, 21 from MGUS and 5 from B-NHL, respectively. In 17 patients the mLCD was not known before kidney biopsy but detected by typical kidney disease. Of the 47 patients with MM 24 had CN, 4 LCDD, 4 ALA and 15 ORD, respectively. All patients with ALA had renal amyloidosis except one, who had an IgA-glomerulonephritis. All patients with MGUS suffered from ORD only. Patients with B-NHL had CN one patient, LCDD one patient, ALA one patient and ORD two patients, respectively. The ORD were also associated with the mLCD in 21 cases (interstitial nephritis n=7, nephrocalcinosis n=7 and membranoproliferative glomerulonephritis n=4), the other patients had kidney disease independent from mLCD (e.g. diabetic nephropathy, focal segmental glomerulosclerosis, IgA-glomerulonephritits or nephroangiosclerosis). The mean follow up time was 20.4±24.8 month. Patients with CN had a significant worse renal function at time of kidney biopsy [serum creatinine [mg/dl]: CN 4.7±4.0; LCDD 3.36±1.38; ALA 1.46±1.0; ORD 2.0±2.3; p< 0.001 CN vs. ALA and ORD]. Patients with ALA had a significant greater proteinuria [g/d], than the other patients [CN 3.3±2.5; LCDD 1.7±0.9; ALA 5.6±5.2; ORD 2.1±1.9; p< 0.05 ALA vs. LCDD and CN; p<0.001 ORD vs. ALA]. 3.7 months after kidney biopsy 50% of patients with CN were on dialysis (HD). At 12 month 59% of patients with CN were on HD, compared to 37% of patients with ALA, 20% of patients with LCDD and 8% of patients with ORD (p=0.0004). Patients on HD had a significant worse survival compared to those without HD [50% survival 5.3 vs. 42 months; p=0.005]. Discussion: Our data demonstrate, that not all patients with mLCD suffered from a mLCD associated renal disease. The renal prognosis was very different between the types of renal disease. The worst renal outcome had patients with CN followed by patients with ALA. The best renal outcome had patients with ORD. Once on HD the survival is worse than without HD. Based on our data we would recommend to clarify the exact type of renal disease in all patients with mLCD and any evidence of renal disease by kidney biopsy. Disclosures: No relevant conflicts of interest to declare.

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Apresentação e Evolução Atípicas da Granulomatose de Wegener: Relato de Caso/Atypical Presentation and Evolution of Wegener's Granulomatosis: Case Report

REVISTA CIÊNCIAS EM SAÚDE ◽

10.21876/rcsfmit.v5i3.379 ◽

1970 ◽

Vol 5 (3) ◽

pp. 53-67

Author(s):

Aline Dos Santos ◽

Ana Caroline Balducci Scafi ◽

Luciene Azevedo Morais ◽

Pablo Girardelli Mendonça Mesquita

Keyword(s):

Renal Failure ◽

Renal Function ◽

Case Report ◽

Chronic Renal Failure ◽

Early Diagnosis ◽

Wegener’S Granulomatosis ◽

Immunosuppressive Treatment ◽

Clinical Manifestations ◽

Wegener's Granulomatosis ◽

Creatinine Concentration

RESUMOIntrodução: A Granulomatose de Wegener (GW) é uma vasculite rara e idiopática associada à presença do anticorpo Anticitoplasma de Neutrófilo (ANCA) que acomete, preferencialmente, os pequenos vasos. As manifestações clínicas são diversas, ocorrendo em mais de 90% dos casos, sintomas do trato respiratório. O comprometimento renal é tardio e preditor de mau prognóstico. Sua morbidade a médio e longo prazo inclui insuficiência renal crônica. A probabilidade de sucesso de manutenção da função renal depende da concentração sérica de creatinina ao início do tratamento, o que indica a importância do diagnóstico e terapêutica adequada precoces. Casuística: Relata-se o caso de uma paciente do sexo feminino, 61 anos, portadora de GW com comprometimento renal avançado à apresentação não precedido por sintomas pulmonares esperados. O tratamento imunossupressor associado a plasmaferese permitiu a melhora da função renal da paciente poupando-a de tornar-se dialítica- dependente. Discussão: A paciente iniciou a doença através de insuficiência renal assintomática, com valores de função renal compatíveis com o estágio mais avançado de doença renal crônica, ultrassonografia dos rins sem alterações compatíveis e sem os sintomas respiratórios esperados. Segundo a literatura, a combinação de imunossupressores e plasmaferese associa-se à recuperação renal em três meses com sobrevivência sem necessidade de diálise por 12 meses, no caso relatado, obteve-se tal resultado em 22 dias sem a necessidade de diálise após um ano. Conclusão: Devido ao diagnóstico precoce, o tratamento adequado foi instalado rapidamente proporcionando à paciente um aumento da expectativa e da qualidade de vida, evitando dependência de terapia renal substitutiva.Palavras-Chave: Granulomatose de Wegener, Plasmaferese, Doença renal crônica. ABSTRACTIntroduction: The Wegener's Granulomatosis (WG) is a rare and idiopathic vasculitis associated with the presence of Antineutrophil Cytoplasmic Antibody (ANCA), that affects, preferentially, the small vessels. The clinical manifestations are diverse, occurring in over 90% of cases, symptoms in the respiratory tract. Kidney damage is a late and bad prognostic predictor. Morbidity in the medium and long term includes chronic renal failure. The probability of renal function maintenance success depends on serum creatinine concentration at the beginning of treatment that indicates the importance of early diagnosis and deployment of an appropriate therapy. Case Report: We present a case of a 61-year-old female patient, carrier of GW with advanced renal impairment presentation, not preceded by expected pulmonary symptoms. The immunosuppressive treatment associated with plasmapheresis allowed the improvement of the patient’s renal function, saving her from becoming dialysis-dependent Discussion: The patient developed the disease through asymptomatic renal failure, renal function with values that are compatible with the most advanced stage of chronic kidney disease, ultrasound of the kidneys without compatible changes and without the expected respiratory symptoms. According to the literature, the combination of immunosuppressive drugs and plasmapheresis is associated with renal recovery in three months with survival without dialysis for 12 months. In this case, a result was obtained in 22 days without the need for dialysis after one year. Conclusion: Due to the early diagnosis, appropriate treatment was quickly installed giving the patient increased life expectancy and quality, preventing dependence on renal replacement therapy.Keywords: Wegener’s granulomatosis, Plasmapheresis, Chronic renal failure.

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AB0750 FUNCTIONAL CONDITION OF KIDNEYS IN THE LONG-TERM COURSE OF SLE IN ADOLESCENTS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.3930 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 1403.2-1403

Author(s):

L. Bohmat ◽

N. Shevchenko ◽

I. Bessonova

Keyword(s):

Renal Function ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Disease Duration ◽

Renal Involvement ◽

Clinical Manifestations ◽

Clinical Analysis ◽

Average Dose ◽

One Year

Background:Lupus nephritis is the most severe and adverse systemic lupus erythematosus (SLE) syndrome. According to modern recommendations, the clinical manifestations of active nephritis should be taken under medical control in 6 months after the start of the disease’s treatment1.Objectives:The aim of this study was to examine the functional status of the kidneys in children with SLE in the course of the disease for more than one year.Methods:The analysis included case histories of 43 patients with SLE, mostly females (41), aged 7 to 18 years (mean age 14.4 years) with disease duration of 4.75 ± 0.58 years of whom 22 were less than three years, 21 - more than three years. All children received corticosteroid therapy, at the time of the examination the average dose was 13.85 ± 1.86 mg per day in terms of prednisolone. The second component of therapy was azathioprine (average dose 97.61 ± 2.11 mg). All children received hydroxychloroquine (5 mg/kg per day).To determine the functional state of the kidneys a clinical analysis of urine, a study of the scope of specific gravity of urine during the day (Zymnytsky test), the content of creatinine and urea in serum to determine the glomerular filtration rate (GFR), the level of microalbuminuria per day were evaluated.Results:Renal involvement in the developed SLE occurred in 73.08% of patients. Among them, therapy during the first 6 months was considered quite effective in 58.06% of patients. It was found that in children with disease duration from one to three years proteinuria was registered in 68.18%, a decrease in GFR in 4.45% and hyperfiltration in 9.09%. In the group of children with duration of SLE more than three years revealed deeper changes in renal function; there was proteinuria in 90.47%, the frequency of GFR decreased was in 19.04%, a decrease of renal concentration function was in 14.28% of cases.Indicators of renal function in children with SLE depending on the duration of the disease (M ± m)IndicatorDuration of the diseasefrom 1 year to 3 years n = 22over 3 yearsn = 21Creatinine, mmol/l0,080 ± 0,0140,090 ± 0,018Мочевина, mmol/l5,66 ± 1,425,63 ± 1,61GFR, ml/min117,05 ± 19,68100,20 ± 18,98 *Microalbuminuria, mg/day24,41 ± 13,1334,73 ± 24,76Density min1,007 ± 0,0051,006 ± 0,005Density max1,024 ± 0,0051,019 ± 0,005 ***р<0,03;**р<0,01 the probability of differences when comparing between groupsConclusion:Long-term follow-up of children with SLE over one year reveals a prolongation of renal dysfunction, which worsens after three years, and is the basis for the development of irreversible renal impairment.References:[1]European evidence-based recommendations for the diagnosis and treatment of childhood-onset lupus nephritis: the SHARE initiative /Noortje Groot, Nienke de Graeff, Stephen D Marks et all. //Ann Rheum Dis. 2017 Dec;76(12):1965-1973.Disclosure of Interests:None declared

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

Pediatric Rheumatology ◽

10.1186/s12969-021-00547-9 ◽

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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