scholarly journals Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement

2019 ◽  
Vol 41 (2) ◽  
pp. 293-295
Author(s):  
Mehtap Ezel Çelakıl ◽  
Burcu Bozkaya Yücel ◽  
Umay Kiraz Özod ◽  
Kenan Bek
Keyword(s):  
Crescentic Glomerulonephritis ◽  
Renal Involvement ◽  
Immunosuppressive Treatment ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Normal Serum ◽  
Laboratory Findings ◽  
Elevated Creatinine ◽  
Systemic Symptoms ◽  
Mild Clinical Presentation ◽  
Normal Serum Creatinine

ABSTRACT Pauci-immune glomerulonephritis (GN) is more common in elderly people compared to children and the etiology is not completely understood yet. Antineutrophil cytoplasmic antibody (ANCA) positivity occurs in 80% of the patients. We report a case of a 7-year-old girl who presented with malaise and mildly elevated creatinine diagnosed as ANCA-associated pauci-immune crescentic glomerulonephritis with crescents in 20 of 25 glomeruli (80%). Of these 20 crescents, 12 were cellular, 4 fibrocellular, and 4 globally sclerotic. She did not have purpura, arthritis, or systemic symptoms and she responded well to initial immunosuppressive treatment despite relatively severe histopathology. The patient was given three pulses of intravenous methylprednisolone (30 mg/kg on alternate days) initially and continued with cyclophosphamide (CYC; 2 mg/kg per day) orally for 3 months with prednisone (1 mg/kg per day). In one month, remission was achieved with normal serum creatinine and prednisone was gradually tapered. The case of this child with a relatively rare pediatric disease emphasizes the importance of early and aggressive immunosuppressive treatment in patients with renal-limited ANCA-associated pauci-immune crescentic GN even if with a mild clinical presentation. As in our patient, clinical and laboratory findings might not always exactly reflect the severity of renal histopathology and thus kidney biopsy is mandatory in such children to guide the clinical management and predict prognosis.

Focal necrotizing and crescentic glomerulonephritis in patients with normal serum creatinine

2013 ◽  
Vol 42 (4) ◽  
pp. 753
Author(s):  
S.P. Mcadoo ◽  
A. Tanna ◽  
O. Randone ◽  
M. Griffith ◽  
J. Levy ◽  
...  
Keyword(s):  
Serum Creatinine ◽  
Crescentic Glomerulonephritis ◽  
Normal Serum ◽  
Normal Serum Creatinine

MO293NEUTROPHIL-TO-LYMPHOCYTE RATIO AND OUTCOME IN CRESCENTIC GLOMERULONEPHRITIS

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Otilia Popa ◽  
Tudor Popa ◽  
Nicoleta Petre ◽  
Ana Stanciu ◽  
Cristina-Stela Capusa ◽  
...  
Keyword(s):  
Crescentic Glomerulonephritis ◽  
Inflammatory Marker ◽  
Immunosuppressive Treatment ◽  
Laboratory Data ◽  
Neutrophil To Lymphocyte Ratio ◽  
Therapeutic Interventions ◽  
Bivariate Analysis ◽  
Laboratory Findings ◽  
Anca Vasculitis ◽  
Lymphocyte Ratio

Abstract Background and Aims Crescent formation is a nonspecific response to severe injury to the glomerular capillary wall, which may be seen with any form of inflammatory glomerulopathy. Despite improved therapeutic interventions, patients with crescentic glomerulonephritis (CGN) still have a severe kidney prognosis and high mortality. Since neutrophil-to-lymphocyte ratio (NLR) is an inflammatory marker linked to worse outcomes in patients with malignancies, chronic kidney disease, myocardial infarction, and other clinical settings, we aimed to assess if NLR can predict kidney outcome and mortality in subjects with CGN. Method Eighty-four adults with biopsy-proven CGN between 1st Jan. 2008 and 31st Dec. 2017 [age 56 (95%CI 53 to 59) years, 50% males, eGFR 9.3 (95%CI 7.8-12.7) mL/min] were retrospectively enrolled in this single-centre study. Subjects were followed for a median of 31 (95%CI 6 to 56) months, until 31st May 2018. Seven subjects with inadequate biopsy sample and insufficient data were excluded. Demographic (age, gender), clinical and laboratory data at the time of biopsy were obtained from medical records. Kaplan-Meier method was used to evaluate kidney and patient survival. Variables related to kidney outcome were evaluated in a multivariate Cox proportional hazard (CPH) model. According to median NLR value (4.3, 95%CI 3.5 to 5.2) subjects were clustered in low NLR group (≤4.3; n=38 pts.) and high NLR group (>4.3; n=38 pts.). The primary endpoints were time to renal replacement therapy (RRT) initiation and all-cause mortality. Results The most common CGN subtype was pauci-immune GN (76.3%; i.e. myeloperoxidase-ANCA vasculitis - 48.7%, PR3-ANCA vasculitis - 15.8% and ANCA-negative vasculitis - 11.8%) followed by. anti-GBM antibody and immune complex related GN, with similar frequencies (11.8% each). According to kidney biopsy (KB) findings, half of the subjects had fibro-cellular crescents (55.3%), while cellular and fibrous crescents were found in 35.5% and 9.2%, respectively. Almost all subjects received corticosteroids (97.4%) and 82.9% received cyclophosphamide. Baseline eGFR was lower in the high-NLR group (8.5 vs. 11.6 mL/min, p=0.04), but no other differences in laboratory findings at baseline between the two groups were found. In bivariate analysis, NLR was negatively associated with serum albumin (rs=-0.26, p=0.02). NLR was not associated with other inflammation markers, Charlson comorbidity score, nor with the type of crescents at KB. During the follow-up period 53.9% started RRT and 19.7% died. There were no differences regarding mortality between the two groups. The mean kidney survival time was 47.6 (95%CI 33.5, 61.7) months. Kidney survival at 12, 24, 48 and 60 months were 44, 41, 38, and 33% respectively. In univariate time-dependent analysis (Figure) patients with low-NLR (68.95%CI 48 to 88 months) had better kidney survival than those with high-NLR (25, 95%CI 13 to 38 months; log rank p=0.004). Moreover, the kidney survival advantage remained (OR 1.06, 95%CI 1.002 to 1.16) after adjusting for eGFR, proteinuria, C reactive protein, immunosuppressive treatment and CGN etiology. Lower eGFR-ul was also associated with poor kidney survival (OR 0.96, 95%CI 0.88 to 0.97). Conclusion In adults with biopsy-proven crescentic glomerulonephritis and advanced kidney function decline, a higher neutrophil-to-lymphocyte ratio seems to predict worse kidney survival, but not the risk of mortality.

scholarly journals Ocular and Systemic Manifestations in Paediatric Multisystem Inflammatory Syndrome Associated with COVID-19

2021 ◽  
Vol 10 (13) ◽  
pp. 2953
Author(s):  
Tzu-Chen Lo ◽  
Yu-Yen Chen
Keyword(s):  
Serum Antibody ◽  
Case Series ◽  
Pooled Analysis ◽  
Rt Pcr ◽  
Laboratory Findings ◽  
Systemic Manifestations ◽  
Polymerase Chain ◽  
Exposure History ◽  
Systemic Symptoms ◽  
Inflammatory Syndrome

This study aimed to achieve a better understanding of the epidemiological and clinical characteristics of multisystem inflammatory syndrome in children (MIS-C) following coronavirus disease 2019 (COVID-19). We searched PubMed and Embase between December 2019 and March 2021 and included only peer-reviewed clinical studies or case series. The proportions of patients who had conjunctivitis, systemic symptoms/signs (s/s), Kawasaki disease (KD), and exposure history to suspected/confirmed COVID-19 cases were obtained. Moreover, positive rates of the nasopharyngeal real-time reverse transcriptase polymerase chain reaction (RT-PCR) and serum antibody for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) were recorded. Overall, 32 studies with 1458 patients were included in the pooled analysis. Around half of the patients had conjunctivitis. The five most common systemic manifestations were fever (96.4%), gastrointestinal s/s (76.7%), shock (61.5%), rash (57.1%), and neurological s/s (36.8%). Almost one-third presented complete KD and about half had exposure history to COVID-19 cases. The positivity of the serology (82.2%) was higher than that of the nasopharyngeal RT-PCR (37.0%). MIS-C associated with COVID-19 leads to several features similar to KD. Epidemiological and laboratory findings suggest that post-infective immune dysregulation may play a predominant role. Further studies are crucial to elucidate the underlying pathogenesis.

scholarly journals Proteinuria Indicates Decreased Normal Glomeruli in ANCA-Associated Glomerulonephritis Independent of Systemic Disease Activity

2021 ◽  
Vol 10 (7) ◽  
pp. 1538
Author(s):  
Désirée Tampe ◽  
Peter Korsten ◽  
Philipp Ströbel ◽  
Samy Hakroush ◽  
Björn Tampe
Keyword(s):  
Disease Activity ◽  
Systemic Disease ◽  
Renal Involvement ◽  
Kidney Injury ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Clinicopathological Characteristics ◽  
Renal Outcome ◽  
Study Results ◽  
Renal Biopsies ◽  
Stage Renal Disease

Background: Renal involvement is a common and severe complication of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), potentially resulting in a pauci-immune necrotizing and crescentic ANCA glomerulonephritis (GN) with acute kidney injury (AKI), end-stage renal disease (ESRD) or death. There is recent evidence that the degree of proteinuria at diagnosis is associated with long-term renal outcome in ANCA GN. Therefore, we here aimed to systematically describe the association between proteinuria and clinicopathological characteristics in 53 renal biopsies with ANCA GN and corresponding urinary samples at admission. Methods: A total number of 53 urinary samples at admission and corresponding renal biopsies with confirmed renal involvement of AAV were retrospectively included from 2015 to 2021 in a single-center study. Results: Proteinuria correlated with myeloperoxidase (MPO) subtype, diagnosis of microscopic polyangiitis (MPA) and severe deterioration of kidney function. Proteinuria was most prominent in sclerotic class ANCA GN and ANCA renal risk score (ARRS) high risk attributed to nonselective proteinuria, including both glomerular and tubular proteinuria. Finally, there was no association between proteinuria and systemic disease activity, suggesting that proteinuria reflected specific renal involvement in AAV rather that systemic disease activity. Conclusions: In conclusion, proteinuria correlated with distinct clinicopathological characteristics in ANCA GN, mostly attributed to a reduced fraction of normal glomeruli. Furthermore, proteinuria in ANCA GN reflected specific renal involvement in AAV rather than systemic disease activity. Therefore, urinary findings could further improve our understanding of mechanisms promoting kidney injury and progression of ANCA GN.

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 Case Report: Patient with Hepatitis C, p-ANCA, and Cryoglobulin Antibodies Presenting with Necrotizing Crescentic p-ANCA Glomerulonephritis

2018 ◽  
Vol 8 (2) ◽  
pp. 161-170
Author(s):  
Ramy M. Hanna ◽  
Naomi So ◽  
Marian Kaldas ◽  
Jean Hou ◽  
Farid Arman ◽  
...  
Keyword(s):  
Hepatitis C ◽  
Crescentic Glomerulonephritis ◽  
The United States ◽  
Cross Reactivity ◽  
Normal Serum ◽  
Hcv Infection ◽  
Low Grade ◽  
Glomerular Injury ◽  
Immune Assays

Hepatitis C (HCV) infection has a prevalence of 3 million infected individuals in the United States, according to recent Center for Disease Control reports, and can have various renal manifestations. Cryoglobulins, antibodies that precipitate at colder temperatures in vitro, are a relatively common cause of renal disease in HCV infection. The cryoglobulin proteins can form occlusive aggregates in small glomerular capillary lumina or deposit in other areas of the glomerulus, resulting in hypocomplementemia, proteinuria, hematuria, and renal injury. The typical biopsy pattern is that of membranoproliferative glomerulonephritis (MPGN). There are, however, other HCV-related patterns of glomerular injury. Anti-neutrophil cytoplasmic antibodies (ANCA) are known to exist in HCV-infected patients. In many reported cases, ANCA serologic testing may appear positive due to cross-reactivity of the immune assays; however, the biopsy findings do not support ANCA-associated crescentic glomerulonephritis (GN)/vasculitis as the primary cause of glomerular injury. There are rare reports of microscopic polyangiitis (MPA) p-ANCA vasculitis, in patients with HCV infection. In comparison with the MPGN pattern of cryoglobulinemic glomerular injury, biopsies from these HCV-infected patients with concomitant MPA revealed a crescentic GN, associated with normal serum complement levels. We present a case of HCV-associated glomerular disease with the surprising biopsy finding of necrotizing and crescentic p-ANCA GN, with a background, low-grade mesangial immune complex GN. Thus, p-ANCA disease should also be considered in HCV-infected patients, in addition to the more typical lesions of MPGN or cryoglobulinemic GN.

scholarly journals Chronic amphotericin nephropathy: morphometric, electron microscopic, and functional studies.

1993 ◽  
Vol 4 (1) ◽  
pp. 69-80
Author(s):  
S N Heyman ◽  
I E Stillman ◽  
M Brezis ◽  
F H Epstein ◽  
K Spokes ◽  
...  
Keyword(s):  
Renal Failure ◽  
Epithelial Cell ◽  
Direct Interaction ◽  
Lymphocytic Infiltration ◽  
Normal Serum ◽  
Electron Microscopic ◽  
Functional Studies ◽  
Salt Depletion ◽  
Direct Toxicity ◽  
Normal Serum Creatinine

The two major hypotheses for the pathogenesis of amphotericin nephrotoxicity are direct interaction with epithelial cell membranes and vasoconstriction. Studies indicating the special vulnerability of the medullary ray and medulla to hypoxia led to a reexamination of amphotericin nephrotoxicity. Twenty-four rats were divided into four groups: amphotericin injection (5 mg/kg daily for 3 wk), amphotericin plus salt depletion, vehicle, and salt depletion and vehicle. The amphotericin group had polyuria (P < 0.01) but normal serum creatinine. In contrast, amphotericin plus salt depletion rats exhibited renal failure (creatinine of 1.49 +/- 0.05 versus amphotericin alone 0.98 +/- 0.01; P < 0.01). Semiquantitative histologic analysis of cortical and medullary injury correlated with functional impairment. Cortical changes in the amphotericin group were largely restricted to the medullary ray, where focal rupture and calcification of thick ascending limbs were noted. The S2/S3 tubules in the medullary rays showed focally diminished cell complexity with histiocytic/lymphocytic infiltration. However, calcification was also seen in the area of the macula densa. Morphometry revealed that the thick ascending limbs in the medulla were hypertrophied (1,420 +/- 63 versus 1,195 +/- 48 microns 2 for vehicle; P < 0.05). In contrast, in the amphotericin and salt depletion group, the changes in the medullary ray extended to the labyrinth and the thick ascending limbs in the inner stripe showed atrophic changes (772 +/- 23 microns 2; P < 0.01 versus vehicle). Thus, changes as a result of amphotericin toxicity take place both in areas known to be most vulnerable to hypoxia (medullary ray and medulla), and in areas rich in oxygen (adjacent to glomerulus). Salt depletion potentiates the cortical changes and converts medullary hypertrophy to atrophy. These findings support a dual pathogenesis for amphotericin nephropathy (direct toxicity and vasoconstriction).

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

2011 ◽  
Vol 139 (3-4) ◽  
pp. 174-178 ◽  
Author(s):  
Brankica Spasojevic-Dimitrijeva ◽  
Mirjana Kostic ◽  
Amira Peco-Antic ◽  
Divna Kruscic ◽  
Mirjana Cvetkovic ◽  
...  
Keyword(s):  
Renal Function ◽  
Kidney Biopsy ◽  
Renal Involvement ◽  
Immunosuppressive Treatment ◽  
Clinical Signs ◽  
Objective Evaluation ◽  
Severe Form ◽  
Renal Manifestation ◽  
Nephritic Syndrome ◽  
Nephrotic Range Proteinuria

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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