scholarly journals Elastase-ANCA-associated idiopathic necrotizing crescentic glomerulonephritis--a report of three cases

2007 ◽  
Vol 22 (7) ◽  
pp. 2068-2071 ◽  
Author(s):  
A. Seidowsky ◽  
M. Hoffmann ◽  
S. Ruben-Duval ◽  
R. Mesbah ◽  
E. Masy ◽  
...  
Keyword(s):  
Crescentic Glomerulonephritis ◽  
Necrotizing Crescentic Glomerulonephritis

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.

Two cases of necrotizing crescentic glomerulonephritis with skin lesions

1998 ◽  
Vol 8 (1) ◽  
pp. 79-87
Author(s):  
Hidekazu Moriya ◽  
Iwao Nakabayashi ◽  
Junichirou Nishiyama ◽  
Aki Ishida ◽  
Keiji Tazawa ◽  
...  
Keyword(s):  
Crescentic Glomerulonephritis ◽  
Skin Lesions ◽  
Necrotizing Crescentic Glomerulonephritis

scholarly journals Antineutrophil Cytoplasmic Autoantibody—Associated Glomerulonephritis in Children

2001 ◽  
Vol 12 (7) ◽  
pp. 1493-1500 ◽  
Author(s):  
MOTOSHI HATTORI ◽  
HIDEAKI KURAYAMA ◽  
YASUSHI KOITABASHI
Keyword(s):  
Renal Function ◽  
Renal Disease ◽  
End Stage Renal Disease ◽  
Crescentic Glomerulonephritis ◽  
Small Sample ◽  
Renal Outcome ◽  
Necrotizing Crescentic Glomerulonephritis ◽  
Antineutrophil Cytoplasmic Autoantibody ◽  
Stage Renal Disease ◽  
End Stage

Abstract. A retrospective investigation was conducted by members of the Japanese Society for Pediatric Nephrology from 1990 to 1997 to define the clinical features and outcome of antineutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis in children. Thirty-four ANCA-seropositive Japanese pediatric patients with biopsy-proven pauci-immune necrotizing crescentic glomerulonephritis were identified. Of these, 3 cases associated with Wegener's granulomatosis were excluded because of the small sample size. Among the 31 patients studied, 10 had a diagnosis of necrotizing crescentic glomerulonephritis alone and 21 had microscopic polyangiitis. Females predominated (87%), and the median age at onset was 12 yr. Twenty-six patients received treatment with cyclophosphamide and corticosteroids, and five patients received treatment with corticosteroids alone; 84% of patients achieved remission, and 39% of responders relapsed in a median of 24 mo. ANCA titers correlated with response to treatment and disease activity, with some exceptions. Patients were followed for a median of 42 mo (range, 3 to 96 mo). Nine of 31 patients (29.0%) progressed to end-stage renal disease, 6 (19.4%) had reduced renal function, and 15 (48.4%) had normal renal function at the last observation. One patient (3.2%) died from cytomegalovirus infection 3 mo after initiation of therapy. Life-table analysis showed 75% renal survival at 39 mo. Patients who subsequently developed end-stage renal disease (n= 9) had significantly higher average peak serum creatinine levels and more chronic pathologic lesions at diagnosis compared with patients with favorable renal outcome (n= 15). In conclusion, our clinical experience suggests that the clinical disease spectrum of ANCA-associated glomerulonephritis is similar in pediatric and adult patients, but there is a female predominance in children.

Two cases of necrotizing crescentic glomerulonephritis with skin lesions

1998 ◽  
Vol 8 (1) ◽  
pp. 79-87
Author(s):  
Hidekazu Moriya ◽  
Iwao Nakabayashi ◽  
Junichirou Nishiyama ◽  
Aki Ishida ◽  
Keiji Tazawa ◽  
...  
Keyword(s):  
Crescentic Glomerulonephritis ◽  
Skin Lesions ◽  
Necrotizing Crescentic Glomerulonephritis

scholarly journals AJKD Atlas of Renal Pathology: Pauci-immune Necrotizing Crescentic Glomerulonephritis

2016 ◽  
Vol 68 (5) ◽  
pp. e31-e32 ◽  
Author(s):  
Agnes B. Fogo ◽  
Mark A. Lusco ◽  
Behzad Najafian ◽  
Charles E. Alpers
Keyword(s):  
Crescentic Glomerulonephritis ◽  
Renal Pathology ◽  
Necrotizing Crescentic Glomerulonephritis

Characterization of a Rat Model of Myeloperoxidase-Anti-Neutrophil Cytoplasmic Antibody-Associated Crescentic Glomerulonephritis

Nephron ◽  
2021 ◽  
pp. 1-17
Author(s):  
Domenico Cerullo ◽  
Daniela Rottoli ◽  
Daniela Corna ◽  
Paola Rizzo ◽  
Mauro Abbate ◽  
...  
Keyword(s):  
Crescentic Glomerulonephritis ◽  
Neutrophil Infiltration ◽  
Double Positive ◽  
Ki 67 ◽  
Necrotizing Crescentic Glomerulonephritis ◽  
Neural Cell Adhesion ◽  
Current Therapies ◽  
Wistar Kyoto ◽  
Set Up ◽  
Parietal Epithelial Cells

<b><i>Background/Aim:</i></b> Necrotizing crescentic glomerulonephritis (GN) associated with anti-neutrophil cytoplasmic antibodies (ANCA) against myeloperoxidase (MPO) is a devastating disease that quickly progresses to kidney failure. Current therapies are broadly immunosuppressive and associated with adverse effects. We wanted to set up a model that could be suitable for testing narrowly targeted therapies. <b><i>Methods:</i></b> The model was constructed in male Wistar Kyoto rats through injections of human MPO (hMPO) and pertussis toxin, followed by a sub-nephritogenic dose of sheep anti-rat glomerular basement membrane (GBM) serum to boost the disease. Rats were monitored for 35 days. Rats given hMPO alone, saline, or human serum albumin with or without anti-GBM serum were also studied. <b><i>Results:</i></b> Rats receiving hMPO developed circulating anti-hMPO and anti-rat MPO antibodies. Challenging hMPO-immunized rats with the anti-GBM serum led to more glomerular neutrophil infiltration and MPO release, and severe haematuria, heavy proteinuria, and higher blood urea nitrogen than hMPO alone. Pauci-immune GN developed with crescents, affecting 25% of glomeruli. The majority of crescents were fibrocellular. Necrotizing lesions and Bowman capsule ruptures were detected. Cells double positive for claudin-1 (a marker of parietal epithelial cells [PECs]) and neural cell adhesion molecule (NCAM; progenitor PECs) were present in crescents. Double staining for NCAM and Ki-67 established proliferative status of progenitor PECs. Podocyte damage was associated with endothelial and GBM changes by electron microscopy. Monocyte/macrophages and CD4<sup>+</sup> and CD8<sup>+</sup> T cells accumulated in glomeruli and the surrounding area and in the tubulointerstitium. Lung haemorrhage also manifested. <b><i>Conclusion:</i></b> This model reflects histological lesions of human ANCA-associated rapidly progressive GN and may be useful for investigating new therapies.

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