scholarly journals Renal involvement and Strømme syndrome

2020 ◽  
Author(s):  
Gianluca Caridi ◽  
Francesca Lugani ◽  
Margherita Lerone ◽  
Maria Teresa Divizia ◽  
Gian Marco Ghiggeri ◽  
...  
Keyword(s):  
Renal Involvement ◽  
Genetic Diagnosis ◽  
Intestinal Atresia ◽  
Causative Gene ◽  
Multiple Systems ◽  
Apple Peel ◽  
Centromeric Protein ◽  
Stage Renal Disease ◽  
End Stage ◽  
Multiorgan Disease

Abstract Strømme syndrome is a rare autosomal recessive congenital disorder involving multiple systems. Centromeric protein F (CENPF) is the causative gene of the disease, and variants are usually linked to lethal outcomes either during the foetal stage or in early life. We present a young adult with a genetic diagnosis of Strømme syndrome who—in addition to classic microcephalia, microphthalmia and intestinal atresia (apple peel-type)—experienced slow and unexpected evolution to end-stage renal disease (ESRD). In conclusion, Strømme syndrome is a complex multiorgan disease that needs multidisciplinary clinical management, and potential evolution to ESRD should be taken into account.

scholarly journals AB0820 INFLUENCE OF ANCA ANTIBODIES ON DEMOGRAPHIC AND CLINICAL CHARACTERISTIC OF AAV

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1434.1-1434
Author(s):  
K. Wójcik ◽  
A. Masiak ◽  
Z. Zdrojewski ◽  
R. Jeleniewicz ◽  
M. Majdan ◽  
...  
Keyword(s):  
Renal Disease ◽  
End Stage Renal Disease ◽  
Clinical Characteristics ◽  
Renal Involvement ◽  
Organ Involvement ◽  
Unknown Etiology ◽  
Anca Associated Vasculitis ◽  
Stage Renal Disease ◽  
End Stage ◽  
Male To Female

Background:ANCA associated vasculitides (AAV) are a heterogeneous group of rare diseases with unknown etiology and the broad clinical spectrum ranging from life-threatening systemic disease, through single organ involvement to minor isolated skin changes. Unfortunately the clinical classification, ANCA specificity or genetic characteristics alone is not able to categorize AAV patients in a satisfactory manner. As a consequence advanced statistical techniques were used to identify and stratify AAV subphenotypes [1, 2]. Here we have analyzed influence of the ANCA type on clinical manifestations and demographic characteristics in various types of AAV, based on data from the POLVAS registryObjectives:We decided to retrospectively analyze a large cohort of Polish AAV patients deriving from several referral centers – members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS) – and concentrate on demographic and clinical characteristics of anti-PR3 and anti-MPO positive patients regardless of their clinical diagnosis.Methods:We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016. Patients were enrolled by 9 referral centers. We analyzed dichotomous variables: gender; ANCA status – anti-PR3+ or anti-MPO+, ANCA negative; organ involvement - skin, eye, ENT, respiratory, heart, GI, renal, urinary, CNS, peripheral nerves and polytomous variable (number of relapses), supported by quantitative covariates (e.g., age at diagnosis, CRP at diagnosis, maximal serum creatinine concentration ever)[3].Results:MPO-positive patients (both GPA and EGPA phenotype) were older at the time of diagnosis with a substantial percentage diagnosed > 65 years of age, and with high rate of renal involvement. Interestingly, while in the whole group of patients diagnosed with EGPA male to female ratio was 1:2, the MPO+ EGPA patients showed M:F ratio of 1:1.The analysis of ANCA negative AAV reveled significant differences in GPA, ANCA negative group is characterized with significantly lower frequency of renal involvement compared to rest GPA (11,5% vs 63,7%) p<0,05 what should be emphasized ANCA negative AAV never lead to ESRD (end stage renal disease) or even transient dialysis.Conclusion:ANCA specificity is indispensable as a separate variable in any clinically relevant analysis of AAV subcategories. MPO+ group is characterized by older age at time of diagnosis, male to female ration 1:1, kidney involvement, and shows more homogenous clinical phenotype than PR3+ AAV patients. In our group ANCA negative AAV never lead to ESRD (end stage renal disease) or even transient dialysis.References:[1]Mahr A, Specks U, Jayne D. Subclassifying ANCA-associated vasculitis: a unifying view of disease spectrum. Rheumatol Oxf Engl 2019;58:1707–9. https://doi.org/10.1093/rheumatology/kez148.[2]Wójcik K, Biedroń G, Wawrzycka-Adamczyk K, Bazan-Socha S, Ćmiel A, Zdrojewski Z et al. Subphenotypes of ANCA-associated vasculitis identified by latent class analysis. Clin Exp Rheumatol. 2020 Sep 1. Epub PMID: 32896241.[3]Wójcik K, Wawrzycka-Adamczyk K, Włudarczyk A, Sznajd J, Zdrojewski Z, Masiak A, et al. Clinical characteristics of Polish patients with ANCA-associated vasculitides—retrospective analysis of POLVAS registry. Clinical Rheumatology. 1 wrzesień 2019;38(9):2553–63.Disclosure of Interests:None declared

scholarly journals Neutrophils associate with Bowman’s capsule rupture specifically in PR3-ANCA glomerulonephritis

2021 ◽  
Author(s):  
Samy Hakroush ◽  
Björn Tampe
Keyword(s):  
Plasma Cells ◽  
Immune Cell ◽  
Renal Involvement ◽  
Kidney Injury ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Stage Renal Disease ◽  
End Stage ◽  
Bowman's Capsule ◽  
Tubulointerstitial Inflammation ◽  
Capsule Rupture

Abstract Background Renal involvement is a common and severe complication of ANCA (antineutrophil cytoplasmic antibody) associated vasculitis (AAV) potentially resulting in a pauci-immune necrotizing and crescentic antineutrophil cytoplasmic antibody (ANCA) glomerulonephritis (GN) with acute kidney injury (AKI), end-stage renal disease (ESRD) or death. We recently described that Bowman’s capsule rupture links glomerular damage to tubulointerstitial inflammation in ANCA-associated glomerulonephritis. Herein we provide a comprehensive histological subtyping of immune cell infiltrates in association with Bowman’s capsule rupture in ANCA GN. Methods A total of 44 kidney biopsies with ANCA GN were retrospectively included in a single-center observational study. Within a renal biopsy specimen, each glomerulus was scored separately for the presence of extensive and focal Bowman’s capsule rupture in injured glomeruli. Infiltrates of neutrophils, eosinophils, plasma cells, and mononucleated cells (macrophages, lymphocytes) were quantified as a fraction of the area of total cortical inflammation. Results Extensive Bowman’s capsule rupture was associated with tubulointerstitial inflammation containing infiltrates of neutrophils, eosinophils and plasma cells. A similar association was observed for the presence of focal Bowman’s capsule rupture, correlating with tubulointerstitial inflammation containing neutrophils, eosinophils and plasma cells. Multiple logistic regression confirmed that extensive Bowman’s capsule rupture correlated with tubulointerstitial inflammation containing neutrophils, and focal Bowman’s capsule rupture correlated with neutrophil and plasma cell infiltration. Furthermore, this association was specifically observed in PR3-ANCA GN. Conclusion To our knowledge, this is the first report linking Bowman’s capsule rupture directly to tubulointerstitial inflammation by immune cell subtypes. This underscores a pathomechanistic link between tubulointerstitial and glomerular lesions in ANCA GN and needs further investigation. Graphical abstract

scholarly journals Kidney Disease in HIV Infection

2019 ◽  
Vol 8 (8) ◽  
pp. 1254 ◽  
Author(s):  
Gaetano Alfano ◽  
Gianni Cappelli ◽  
Francesco Fontana ◽  
Luca Di Lullo ◽  
Biagio Di Iorio ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Life Expectancy ◽  
Early Recognition ◽  
Renal Involvement ◽  
People Living With Hiv ◽  
Risk Of Death ◽  
Age Related ◽  
Stage Renal Disease ◽  
End Stage ◽  
Living With Hiv

Antiretroviral therapy (ART) has significantly improved life expectancy of infected subjects, generating a new epidemiological setting of people aging withHuman Immunodeficiency Virus (HIV). People living with HIV (PLWH), having longer life expectancy, now face several age-related conditions as well as side effects of long-term exposure of ART. Chronic kidney disease (CKD) is a common comorbidity in this population. CKD is a relentlessly progressive disease that may evolve toward end-stage renal disease (ESRD) and significantly affect quality of life and risk of death. Herein, we review current understanding of renal involvement in PLWH, mechanisms and risk factors for CKD as well as strategies for early recognition of renal dysfunction and best care of CKD.

The patient with vasculitis

2018 ◽  
Author(s):  
Alan D. Salama
Keyword(s):  
Patient Outcomes ◽  
End Stage Renal Disease ◽  
Impaired Renal Function ◽  
Systemic Vasculitis ◽  
Renal Involvement ◽  
Small Vessel Vasculitis ◽  
Organ Systems ◽  
Primary Systemic Vasculitis ◽  
Stage Renal Disease ◽  
End Stage

Primary systemic vasculitis frequently leads to renal involvement and is responsible for significant numbers of patients progressing to end-stage renal disease. Frequently this is due to small vessel vasculitis, in association with antineutrophil cytoplasm antibody, which requires prompt recognition and timely therapeutic intervention to optimize renal and patient outcomes. Other organ systems are often affected. Relapses occur in about 50%.Less commonly medium or larger vessel vasculitis may involve the kidneys and through ischaemia lead to impaired renal function and renovascular hypertension, as in Takayasu’s or Kawasaki disease, and polyarteritis nodosa (PAN).

scholarly journals The Clinical and Pathological Features of Children With Microscopic Polyangiitis

2021 ◽  
Vol 9 ◽  
Author(s):  
Qian Li ◽  
Li-Chun Yu ◽  
Feng-Xia Li ◽  
Jing Wang ◽  
Yuan Chen ◽  
...  
Keyword(s):  
Microscopic Polyangiitis ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Antithyroid Drug ◽  
Good Prognosis ◽  
Factors Associated ◽  
Stage Renal Disease ◽  
End Stage ◽  
Lung Impairment

Objective: The aim of this study was to explore the clinical features, pathological characteristics, and the prognosis of children with microscopic polyangiitis (MPA).Methods: Ten children with MPA that were hospitalized in our hospital were included in this study. The children's pre-diagnosis status, clinical manifestations, renal pathology, treatment, and prognosis data were analyzed retrospectively.Results: All 10 cases included female patients with a median age of 8.9 years old at the time of diagnosis. MPO-ANCA antibody was positive in all cases, combined with a positive anti-GBM antibody in two cases. Nine cases had primary AAV and one had antithyroid drug (ATD)-associated MPA (secondary to methimazole). Renal involvement was found in all 10 patients, lung impairment was present in eight cases, and anemia was present in nine patients. Renal biopsies were performed in all 10 patients. Segmental focal or global glomerular necrosis was observed in 70% of the patients (7/10). The treatment mainly included steroid use combined with Cyclophosphamide and Mycophenolate. The follow-up s of the patients revealed normal renal function in eight patients and progression to end-stage renal disease (ESRD) in two patients.Conclusions: Female predisposition and positive MPO-ANCA antibody were prominent in children with MPA. The patients' kidneys and lungs were the most frequently involved organs. Corticosteroid combined with immunosuppressive therapy was recommended for the treatment of MPA. Early diagnosis, prompt aggressive treatment, and regular follow-ups are also very important factors associated with a good prognosis.

scholarly journals Novel Mutations in NPHS2 Detected in Both Familial and Sporadic Steroid-Resistant Nephrotic Syndrome

2002 ◽  
Vol 13 (2) ◽  
pp. 388-393 ◽  
Author(s):  
Stephanie M. Karle ◽  
Barbara Uetz ◽  
Vera Ronner ◽  
Lisa Glaeser ◽  
Friedhelm Hildebrandt ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Disease ◽  
End Stage Renal Disease ◽  
Positional Cloning ◽  
Mutational Analysis ◽  
Causative Gene ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Stage Renal Disease ◽  
End Stage

ABSTRACT. Autosomal recessive steroid-resistant nephrotic syndrome (SRINS) belongs to the heterogeneous group of familial nephrotic syndrome and represents a frequent cause of end-stage renal disease in childhood. This kidney disorder is characterized by early onset of proteinuria, progression to end-stage renal disease, and histologic findings of focal segmental glomerulosclerosis, minimal change nephrotic syndrome, or both. A causative gene, NPHS2, has been mapped to chromosome 1q25-q31 and was recently identified by positional cloning. This study reports five novel NPHS2 mutations: A284V, R196P, V290M, IVS4-1G→T, and 460-467insT in 12 (46%) of 26 multiplex families and in 7 (28%) of 25 single patients with the clinical diagnosis of a SRINS. Because NPHS2 mutations were found in nearly 30% of these patients with “sporadic” SRINS, mutational analysis should also be performed in these patients. Besides better classification of the disease entity, identification of NPHS2 mutations may save some of these patients from unnecessary steroid treatment and also permit the prediction of absence of disease recurrence after kidney transplantation.

scholarly journals Early Renal Disease in Children with Sickle Cell Disease from Malawi

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 1316-1316
Author(s):  
Jonathan Brett Heimlich ◽  
Godwin Chipoka ◽  
Graham Ellis ◽  
Laila Elsherif ◽  
Emeraghi David ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Renal Disease ◽  
Sickle Cell ◽  
End Stage Renal Disease ◽  
Renal Involvement ◽  
Significant Proportion ◽  
Cell Disease ◽  
Clinical Biomarkers ◽  
Stage Renal Disease ◽  
End Stage

Abstract Sickle cell nephropathy (SCN) is a prevalent complication among adults with sickle cell disease (SCD) and has been observed in younger populations, suggesting potential early renal involvement in pediatric patients. Initial hyperfiltration and albuminuria followed by frank proteinuria, leading to declining GFR and eventual end stage renal disease is assumed to be the typical progression of SCN; however, few clinical biomarkers exist to identify early-stage renal disease. We describe the renal profile in 119 children with SCD at Kamuzu Central Hospital in Lilongwe, Malawi and propose a novel urinary biomarker for the identification of children with early renal disease. Among children with confirmed HbSS disease (females 47.9%; median age 9.0 years, IQR: 5, 11), 21.6% were found to have a urinary albumin to creatinine ratio (UACR) 30mg/g or above. Baseline laboratory and clinical parameters stratified by UACR are shown in Tables 1 and 2. Patients with increased levels of UACR were found to be significantly older, and have significantly elevated plasma levels of lactate dehydrogenase (LDH), total bilirubin, and indirect bilirubin when compared to those without albuminuria (p<0.05). No association was seen between albuminuria and either hemoglobin or plasma hemoglobin. Albuminuria was also significantly associated with elevated levels of nephrin, a urinary marker of glomerular injury (p<0.01). Multivariable logistic regression was used to investigate risk factors that are associated with albuminuria (UACR >= 30). Urine nephrin was significantly associated with albuminuria (regression coefficient estimate: 0.00188, SE: 0.000571, p = 0.0010). Additional analysis using a nephrin cut-point of 293 pg/mL, the median value in the cohort, revealed a 17.8 times greater odds of having albuminuria in children with nephrinuria above this value. These data taken together suggests that a significant proportion of children with SCD in Malawi exhibit renal involvement early in life and could be at risk for worsening nephropathy and end-stage renal disease as they grow older. Our data further indicates that urinary nephrin could be utilized as an early marker of glomerular disease in SCN and possibly prompt earlier intervention in these children. The discordant association of albuminuria with clinical markers of hemolysis suggests that hemolysis may not play a substantial role in the pathogenesis of albuminuria in this population. Increased surveillance of children with SCD for renal complications can ultimately inform management strategies to improve outcomes and increase life expectancy among children with SCD. Disclosures No relevant conflicts of interest to declare.

scholarly journals Schistosomiasis Associated Glomerulopathy (Glomerulonephristis / Nephrotic Syndrome): A review and Update of the Literature

2020 ◽  
Vol 3 (4) ◽  
pp. 01-19
Author(s):  
Anthony Kodzo-Grey Venyo
Keyword(s):  
Nephrotic Syndrome ◽  
Urinary Tract ◽  
Hepatitis C ◽  
Kidney Disease ◽  
Schistosoma Mansoni ◽  
End Stage Renal Disease ◽  
Renal Involvement ◽  
Stage Renal Disease ◽  
End Stage ◽  
Uncommon Complication

Schistosomiasis may affect a number of organs within the human body. Schistosomiasis may also be associated with glomerular disease of the kidney in the form of glomerulonephritis and nephrotic syndrome. The association between Schistosomiasis and glomerulopathy may not be well known by a number of practitioners and its pathophysiology may not be very well understood and this could be due to the underdiagnosis of the disease due to the possibility of lack of facilities within the Schistosomiasis endemic areas of the world especially within tropical Africa. Nevertheless, there are a number of patterns of renal involvement in Schistosomiasis which include (a) upper urinary tract sequelae of lower urinary tract Schistosomiasis pathology, (b) immune-related glomerulonephritis, (c) as well as oxidant-stress-mediated renal tubular glomerulonephritis. The renal involvement by Schistosomiasis does tend to be ensued by the development of Schistosomiasis-associated Glomerulopathy (Glomerulonephritis / Nephrotic Syndrome) of varying severity. Individuals affected by Schistosomiasis-associated kidney disease may develop (a) asymptomatic disease which tends to related to self-limited and asymptomatic glomerular disease (b) symptomatic disease which most commonly would tend to present with nephrotic syndrome most often in patients who develop hepato-splenic schistosomiasis with liver fibrosis associated with Schistosoma mansoni infection. Symptomatic patients tend to develop severe hypo-proteinemia, half of the patients tend to have elevated blood pressure. In the absence of nephrotic syndrome, patients who have Schistosomiasis-associated glomerulopathy may manifest with: (a) isolated non-nephrotic syndrome proteinuria (b) acute glomerulonephritis associated with haematuria and heavy proteinuria, (c) Nephrotic syndrome together with systemic manifestations of co-infection with salmonella (class II) or hepatitis C virus (Class I), (d) End stage renal disease (ESRD). Some of the patients who have Schistosomiasis of the kidney may present with Haematuria, Hypertension, Hepato-splenic Schistosomiasis. The diagnosis should be suspected with regard to the following scenarios: (a) clinical suspicion in a patient with kidney disease who is known or has been known to have Schistosoma mansoni; (b) exposure to an endemic area, (c) clinical evidence / demonstration of chronic hepatosplenic schistosomiasis, (d) if the patient has not been diagnosed as having been afflicted with schistosomiasis, then schistosomal infection should be documented, (e) majority of patients with schistosomiasis and kidney disease should have kidney biopsy. (f) The patients should be evaluated for co-infection with salmonella, hepatitis C virus, and hepatitis B virus in order to ensure adequate treatment of the disease. Schistosomiasis is the second most devastating tropical parasitic disease globally which tends to be responsible for many urological complications. Nevertheless, glomerular injury is an uncommon complication which has mainly or most often described with Schistosoma Mansoni. When patients who have Schistosomiasis-associated Kidney disease are managed and followed-up on long-term basis with renal end points, one third of the patients independent of the histopathology examination features of the kidney biopsies would tend to progress dialysis. Membranoproliferative glomerulonephritis (MPGN) is an uncommon complication associated with Schistosoma Haematobium infection which tends to be associated with potentially poor prognosis. MPGN could lead quickly to End Stage Renal Disease (ESRD). Anti-helminthic and immunosuppressive medicaments tend not to be effective at advanced stages of the disease and hence efforts need to be focused upon the prevention, early detection, as well as treatment of Schistosoma infections among at-risk groups of individuals. In order to reduce morbidity related to Schistosoma-associated nephropathy, public health policy, should be concentrated upon the prevention of the disease by the control of snail, improved sanitation, and health education, as well as by the implementation, and sustenance of chemotherapy-based control strategies. Considering that many individuals are found yearly to be inflicted by Schistosomiasis who tend to dwell in more rural parts of their countries where facilities for the undertaking of renal function tests and biopsies of the kidney tend not to be readily available, there is the likelihood that Schistosomiasis-associated nephropathies may be highly underdiagnosed globally.

Nierenbeteiligung bei ANCA-assoziierten Vaskulitiden

2018 ◽  
Vol 143 (02) ◽  
pp. 79-88 ◽  
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.

MO268GLOMERULAR C3 DEPOSITS IN ANCA ASSOCIATED PATIENTS – IS IT AN IMPORTANT FINDING?

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Matija Crnogorac ◽  
Ivica Horvatić ◽  
Patricia Kacinari ◽  
Miroslav Tišljar ◽  
Ana Brechelmacher ◽  
...  
Keyword(s):  
Statistical Significance ◽  
Renal Involvement ◽  
Kidney Tissue ◽  
Strong Tendency ◽  
Positive Staining ◽  
Chi Square ◽  
Tissue Samples ◽  
Cox Proportional Hazard Regression ◽  
Stage Renal Disease ◽  
End Stage

Abstract Background and Aims In recent years there were many studies explaining the role of alternative complement pathway (aCP) in etiopathogenesis of the ANCA associated vasculitis (AAV) and weather it affects patient outcomes. We hypothesized the importance of the C3 positive staining in glomeruli and that there could be differences between clinical, serological and histological phenotypes in AAV patients. Method This study included 106 consecutive AAV patients with renal involvement in the period from 2007-2017. We performed renal biopsy on patients using automatic 16 Gauge needle. Light, immunofluorescent and electronic microscopy were performed. Category variables were analysed with Fisher Exact testom and continuous with Kruskal-Wallis testom. Statistical difference was then analysed posthoc with Chi-square test. Primary outcomes were combined outcome progression to end-stage renal disease, defined as persistent (more than three months) need for renal replacement therapy or permanent reduction of EGFR to &lt;15ml/minute (according to CKD EPI formula) and/or death (ESRDD), death (D) and ESRD alone, and disease relapse. Kaplan Meyer survival analysis and multivariate Cox proportional hazard regression analysis were used to explore difference between phenotypes and finding significant predictors regarding outcomes. Results The study included 106 AAV patients with renal involvement: 66 (61,1%) MPA, 20 (18,5%) GPA, 20 (18,5%) RLV. There were 14 (13%) PR3-ANCA positive patients, 57 (52,8%) MPO ANCA positive, 5 (4,6%) PR3-ANCA+MPO-ANCA and 32 (29,6%) ANCA negative patients. Average SCr was 316,5 μmol/l (IQR 207,0-548,5), 24-hour proteinuria median was 1,7g/24h (IQR 0,8-2,8). Histologicaly (Berden classification) 43 (39,8%) patients had crescentic, 19 (17,6%) focal, 34 (31,5%) mixed and 12 (11,1%) sclerotic class. Out of all, 48,5% MPA patients had C3 deposits in glomeruli compared to 25% GPA and 35% RLV though statistical significance was not reached (p=0,1). Same applied for serological phenotypes: MPO-ANCA and ANCA negatives had more C3 deposits but statistical significance was not reached (p=0,6). When comparing histopathological classes there was strong tendency of crescentic class having more C3 deposits (p=0,076) compared to focal and mixed. In sclerotic class 50% had C3 deposits but C3 deposits can be ordinary found in sclerotic lesions so the interpretation is more challenging. Interestingly patients requiring haemodialysis had strong tendency of having more C3 deposits compared to those not needing haemodialysis treatment (p=0,09). Conclusion Glomerular C3 deposits in kidney tissue samples could prove to be a useful tool for perhaps predicting the severity and the course of the disease. Though differences between various groups in our cohort didn't have statistical significance, there was a tendency for MPA patients, those with MPO-ANCA and those with crescentic class of having higher proportion of C3 deposits in glomeruli compared to other groups. This could suggest more aCP activation in MPO positive AAV. Also there was strong tendency of patients requiring dialysis having higher proportion of C3 deposits compared to non-dialysis patients which could signify C3 deposition of being the hallmark of more severe renal involvement in AAV patients. This data needs further confirmation from future studies.

Sign in / Sign up

Export Citation Format

Share Document