A case of PR3–ANCA-positive anti-GBM disease associated with intrarenal arteritis and thrombotic microangiopathy

Manabe, Shun; Banno, Mayuko; Nakano, Marie; Fujii, Teruhiro; Kakuta, Yukio; Nitta, Kosaku; Hatano, M

doi:10.1007/s13730-016-0240-3

Cited by 7 publications

(7 citation statements)

References 29 publications

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“…DAPD has a reported incidence of 0.00006% worldwide and accounts for about 25% of cases of GPS [ 8 , 9 ]. In addition, an estimated 20–40% of GPS patients have a coexistence of ANCAs [ 1 , 4 ]. It is more common in Caucasians and in ages between 50–59 and ≥70 years, with a median age of 57.1 years [ 4 , 8 , 10 ].…”

Section: Discussionmentioning

confidence: 99%

“…In addition, sclerotic glomeruli are present in DAPD likely due to the coexistence of ANCAs (MPO-ANCA) [ 8 ]. Intrarenal arteritis has also been described in PR3-ANCA double-positive patients [ 1 ].…”

Section: Discussionmentioning

confidence: 99%

“…Rapidly progressive glomerulonephritis resulting from the coexistence of antiglomerular basement membrane (anti-GBM) disease and antineutrophil cytoplasmic antibodies (ANCAs) is an unusual cause of renal failure and is regarded as a “double-positive” disease (DP) [ 1 ]. DPD is a rare disease that was first coined in the 1980s by Omotoso et al [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

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Triple Jeopardy: Rapidly Progressive Glomerulonephritis Induced by Triple Seropositive Disease—A Rare Case

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Upadrasta

Udongwo

et al. 2022

Case Reports in Medicine

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The double-positive disease is the co-occurrence of antiglomerular basement membrane (anti-GBM) disease and antineutrophil cytoplasmic antibodies (ANCAs) and is an uncommon cause of renal failure. Our case of triple-positive disease is an even rarer cause of isolated renal failure, as it includes anti-GBM, antimyeloperoxidase (MPO), and antiproteinase 3 (PR3). We present a case of a 62-year-old Caucasian male with a history of multiple comorbidities, who presented to the emergency department (ED) with worsening dyspnea on exertion that started about one month prior to admission. He was found to be in renal failure secondary to triple-positive disease. We believe that the likely mechanism of our patient’s triple-positive disease was a drug-induced ANCA vasculitis overlapping with Goodpasture’s syndrome. We believe our case to be a valuable addition to the literature as it is a rare overlap syndrome without a previously established disease course or etiology.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Triple Jeopardy: Rapidly Progressive Glomerulonephritis Induced by Triple Seropositive Disease—A Rare Case

Vedire

Upadrasta

Udongwo

et al. 2022

Case Reports in Medicine

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“…Therefore, TMA is considered more likely to occur in cases of anti-GBM disease with APSN, and careful attention should be paid to the increased risk of TMA in such cases. Furthermore, the cause of thrombocytopenia may be associated with anti-GBM nephritis and severe hypertension ( 18 ). The thrombocytopenia in the present case may thus have resulted from the combined superposition of anti-GBM nephritis and TMA due to severe hypertension and APS.…”

Section: Discussionmentioning

confidence: 99%

Anti-glomerular Basement Membrane Disease with Antiphospholipid Syndrome

et al. 2021

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A 48-year-old woman presented with a fever, microscopic hematuria, proteinuria, and rapid deterioration of the renal function. Pulmonary alveolar hemorrhaging and a high level of anti-glomerular basement membrane (GBM) antibodies (700 IU/mL) were observed. Based on her medical history and positive findings of serum lupus anticoagulant, anti-phospholipid antibody syndrome (APS) was suspected. A renal biopsy revealed cellular crescentic glomerulonephritis with thrombosis, suggesting anti-GBM disease with catastrophic APS. The patient was treated with pulse steroid therapy, plasma exchange, hemodialysis, and intravenous cyclophosphamide pulse therapy. To our knowledge, this is the first report of a patient with anti-GBM disease and APS.

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“…Autoimmune diseases, such as systemic lupus erythematosus, systemic sclerosis, antiphospholipid syndrome, and anti-neutrophil cytoplasmic autoantibody (ANCA)associated vasculitis, have been reported to be the cause of secondary TMA [2,3,5]. Though there are some existing case reports of anti-GBM disease with TMA [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20], the clinical features of this phenomenon have not been described extensively.…”

Section: Introductionmentioning

confidence: 99%

Clinical characteristics of anti-GBM disease with thrombotic microangiopathy: a case report and literature review

et al. 2023

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The coexistence of anti-glomerular basement membrane (anti-GBM) disease with thrombotic microangiopathy (TMA) is rarely encountered, and the clinical characteristics of this phenomenon are not well known. A 76-year-old Japanese woman with a history of idiopathic pulmonary disease was diagnosed with anti-GBM disease due to rapidly progressive glomerulonephritis and a positive anti-GBM antibody test result. We treated the patient with hemodialysis, glucocorticoids, and plasmapheresis. During treatment, the patient suddenly became comatose. TMA was then diagnosed because of thrombocytopenia and microangiopathic hemolytic anemia. The activity of a disintegrin-like and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS-13) was retained at 48%. Although we continued the treatment, the patient died of respiratory failure. An autopsy revealed the cause of respiratory failure to be an acute exacerbation of interstitial pneumonia. The clinical findings of the renal specimen indicated anti-GBM disease; however, there were no lesions suggestive of TMA. A genetic test did not reveal an apparent genetic mutation of the atypical hemolytic uremic syndrome. We conducted a literature review of past case reports of anti-GBM disease with TMA. The following clinical characteristics were obtained. First, 75% of the cases were reported in Asia. Second, TMA tended to appear during the treatment course for anti-GBM disease and usually resolved within 12 weeks. Third, ADAMTS-13 activity was retained above 10% in 90% of the cases. Fourth, central nervous system manifestations occurred in more than half of the patients. Fifth, the renal outcome was very poor. Further studies are required to understand the pathophysiology of this phenomenon.

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A case of PR3–ANCA-positive anti-GBM disease associated with intrarenal arteritis and thrombotic microangiopathy

Cited by 7 publications

References 29 publications

Triple Jeopardy: Rapidly Progressive Glomerulonephritis Induced by Triple Seropositive Disease—A Rare Case

Triple Jeopardy: Rapidly Progressive Glomerulonephritis Induced by Triple Seropositive Disease—A Rare Case

Anti-glomerular Basement Membrane Disease with Antiphospholipid Syndrome

Clinical characteristics of anti-GBM disease with thrombotic microangiopathy: a case report and literature review

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