Crescentic Glomerulonephritis with Anti-GBM and p-ANCA Antibodies

Javed, Tariq; Vohra, Parag

doi:10.1155/2012/132085

Cited by 3 publications

(4 citation statements)

References 16 publications

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“…Up to 20-25% of patients with anti-GBM antibody nephritis have associated ANCA positivity (3). On the other hand, 5% of ANCA-associated GN patients have anti-GBM antibodies in their sera (4). Interestingly, our patient had both types of ANCA antibodies, a phenomenon very rarely reported in literature.…”

Section: What Is the Significance Of Dual Positivity Of Anti-gbm And mentioning

confidence: 39%

Rapidly progressive renal failure followed by respiratory failure in an elderly woman

Naqvi¹,

Mubarak²

2017

J Nephropathol

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Section: What Is the Significance Of Dual Positivity Of Anti-gbm And mentioning

confidence: 39%

Rapidly progressive renal failure followed by respiratory failure in an elderly woman

Naqvi¹,

Mubarak²

2017

J Nephropathol

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“…McAdoo et al in Although co-presentation with both ANCA (MPO or PR3) and anti-GBM antibodies is a well-recognized entity [3], the underlying mechanism of this association remains unclear [2,3,5,6]. Several hypotheses have been proposed and the most attractive seems to be the existence of an ANCA-induced glomerular inflammation as a trigger to produce GBM autoantibodies [1,2,[6][7][8][9]. In this patient, the longer duration of constitutional symptoms and the presence of both active and chronic lesions in renal biopsy also suggest that ANCA-mediated glomerular inflammation may have precede the onset of anti-GBM disease.…”

Section: Discussionmentioning

confidence: 99%

“…About 10-40% [5,6] of anti-GBM antibody-positive patients also had positive ANCA, usually myeloperoxidase (MPO)-ANCA [1,2,6,7], and 5-14% [6] of ANCA-positive patients had positive anti-GBM antibodies [1]. The clinical presentation may be atypical [2], resulting in delay of the correct diagnosis and appropriate treatment [2,8] with significant morbidity and mortality [2].…”

Section: Introductionmentioning

confidence: 99%

A Rare Case of Pulmonary-Renal Syndrome With Triple-Seropositive for Myeloperoxidase-Anti-Neutrophil Cytoplasm Antibody (MPO-ANCA), Proteinase 3 (PR3)-ANCA and Anti-Glomerular Basement Membrane (GBM) Antibodies

et al. 2021

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Anti-glomerular basement membrane (anti-GBM) disease and antineutrophil cytoplasm antibody (ANCA)-associated vasculitis are the main causes of pulmonary-renal syndrome (PRS). The concurrence of both ANCA -myeloperoxidase (MPO) and proteinase 3 (PR3) -and anti-GBM antibodies has been described, although positivity for all three antibodies has rarely been reported. The natural history of triplepositive patients as well as the best therapeutic approach remains unknown. We describe a case of an 80-year-old woman that presented to the emergency department with a 3-month history of progressive fatigue, malaise and anorexia, and 5 weeks of cough with bloodstreaked sputum and progressive peripheral edema. Through the complementary study, a rare diagnosis of PRS with triple-seropositive for both ANCA (MPO and PR3) and anti-GBM antibodies was made in a patient with untreated chronic hepatitis B virus infection. She was treated with glucocorticoid, cyclophosphamide, plasma exchange and entecavir, with pulmonary recovery. Renal function did not improve. After 2 years, the patient is still in dialysis, but did not have relapse of alveolar hemorrhage and ANCA and anti-GBM antibody titers remain negative. The authors intend to warn to PRS, in particular this rare cause, since delaying diagnosis can lead to significant morbidity and mortality for patients.

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“…It is hypothesized that ANCA may play an important role in the development of anti-GBM disease by causing damage to the GBM and exposing the target antigen, thereby triggering production of anti-GBM antibodies ( 7 ). The correct diagnosis and initiation of treatment may be delayed in some patients with DPD due to an atypical presentation of pulmonary-renal syndrome (PRS) ( 8 ).…”

Section: Introductionmentioning

confidence: 99%

Pulmonary Hemorrhage and Crescentic Glomerulonephritis in a Patient with Seropositive Anti-Glomerular Basement Membrane Disease and Anti-Neutrophil Cytoplasmic Antibodies

Sreter

2022

ACC

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SUMMARY Anti-glomerular basement membrane (anti-GBM) disease is an acute and life-threatening systemic autoimmune disorder. The coexistence of circulating anti-neutrophil cytoplasmic antibodies (ANCA) and anti-GBM disease, the so-called double-positive disease (DPD), is exceptionally rare. We report a unique case of DPD manifesting as pulmonary-renal syndrome (PRS) in a 46-year-old woman who first presented with clinical and radiological suspicion of pneumonia. Chest computed tomography scan later revealed bilateral alveolar hemorrhage. Kidney biopsy showed necrotizing crescentic (100% glomeruli) glomerulonephritis. On immunofluorescence microscopy, glomeruli were global linear positive for IgG, confirming anti-GBM disease. Double positivity was detected for circulating anti-myeloperoxidase ANCA (p-ANCA) and anti-GBM antibodies. Acute renal failure evolved rapidly. Therapeutic plasma exchange (TPE) and hemodialysis (HD) were initiated early in combination with intravenous pulse corticosteroid therapy followed by oral methylprednisolone and cyclophosphamide. Pulmonary hemorrhage resolved, but renal function could not be preserved. The patient remains HD dependent. This case report highlights that pulmonary symptomatology may be the leading clinical presentation of PRS, with initially normal renal function at DPD onset. Early recognition and diagnosis are therefore crucial to timely clinical intervention. The role of prompt kidney biopsy and initiation of TPE and HD in PRS must not be underestimated.

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Crescentic Glomerulonephritis with Anti-GBM and p-ANCA Antibodies

Cited by 3 publications

References 16 publications

Rapidly progressive renal failure followed by respiratory failure in an elderly woman

Rapidly progressive renal failure followed by respiratory failure in an elderly woman

A Rare Case of Pulmonary-Renal Syndrome With Triple-Seropositive for Myeloperoxidase-Anti-Neutrophil Cytoplasm Antibody (MPO-ANCA), Proteinase 3 (PR3)-ANCA and Anti-Glomerular Basement Membrane (GBM) Antibodies

Pulmonary Hemorrhage and Crescentic Glomerulonephritis in a Patient with Seropositive Anti-Glomerular Basement Membrane Disease and Anti-Neutrophil Cytoplasmic Antibodies

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