ANCA-associated vasculitis with dual ANCA positivity in coexistence with mixed connective tissue disease

Murakami, Masanori; Shimane, Kenichi; Takahashi, Hiroshi; Tomiyama, Junji; Nagashima, Masakazu

doi:10.1007/s10165-012-0619-8

Cited by 6 publications

(6 citation statements)

References 27 publications

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“…These patients exhibited various patterns of other autoantibodies. Serum complement levels, not reported in two patients [9, 10], were depressed in three [11, 13] (this report) and normal in the remaining three patients [8, 12, 14]. …”

Section: Discussionmentioning

confidence: 73%

“…Proteinuria and hematuria were universal. Serum creatinine, not reported in one patient [9], was normal in two [14] (this report) and elevated in the remaining five patients [8, 10-13]. Renal histology was not available in two patients [11, 14].…”

Section: Discussionmentioning

confidence: 95%

“…Age at diagnosis of ANCA positivity varied between 38 and 68 years. Six patients had MPO-ANCA [8, 10-13] (this report), one had PR3-ANCA [9], and one patient had both MPO and PR3-ANCA [14]. These patients exhibited various patterns of other autoantibodies.…”

Section: Discussionmentioning

confidence: 98%

“…Serum creatinine, not reported in one patient [9], was normal in two [14] (this report) and elevated in the remaining five patients [8, 10-13]. Renal histology was not available in two patients [11, 14]. Among the remaining six patients, one had atherosclerosis of the renal arteries but no glomerulonephritis [9] and the other five patients had pauci-immune glomerulonephritis [8, 10, 12, 13] (this report).…”

Section: Discussionmentioning

confidence: 99%

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Sustained Remission of Antineutrophil Cytoplasmic Antibody-Mediated Glomerulonephritis and Nephrotic Syndrome in Mixed Connective Tissue Disease

et al. 2013

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A woman diagnosed with mixed connective tissue disease (MCTD) developed an anti-myeloperoxidase (MPO) antineutrophil cytoplasmic antibody (ANCA) and nephrotic syndrome with normal serum creatinine. Percutaneous kidney biopsy showed pauciimmune glomerulonephritis with superimposed immune complex deposition. After treatment with cyclophophamide and prednisone, proteinuria decreased progressively to a level of 0.4 g/g creatinine, ANCA became undetectable, while serum creatinine remained normal seven years after the beginning of treatment. Sustained remission of nephrotic proteinuria with preserved renal function may follow treatment of ANCA-mediated disease developing in patients with MCTD.

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Section: Discussionmentioning

confidence: 73%

Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

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Sustained Remission of Antineutrophil Cytoplasmic Antibody-Mediated Glomerulonephritis and Nephrotic Syndrome in Mixed Connective Tissue Disease

et al. 2013

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“…Table 2 lists previously reported cases of dual AAV [ 2 , 4 , 6 , 8 – 10 ] . In our case, we meticulously ruled out all possible secondary causes of dual AAV as given in Table 1 .…”

Section: Discussionmentioning

confidence: 99%

Rare Case of Renal Limited Dual Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: Double Trouble!

John

Roy

Mani

et al. 2022

European Journal of Case Reports in Internal Medicine

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Dual anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) characterized by the presence of both anti-proteinase-3 (PR3-ANCA) and anti-myeloperoxidase (MPO-ANCA) antibodies is a rare clinical entity. Only few cases have been reported previously, most of which were associated with infections, drugs, autoimmune diseases and malignancies. Herein, we describe a young woman who presented with rapidly progressive glomerulonephritis with hypocomplementemia and markedly elevated anti-PR3 and anti-MPO titres. Meticulous work-up ruled out all possible secondary causes. Renal biopsy showed the presence of focal fibrocellular crescents with focal mesangial hypercellularity. Immunofluorescence and electron microscopy showed pauci-immune deposits. The patient was treated with an induction regimen comprising oral prednisolone and cyclophosphamide. She attained both clinical and serological remission at 3 months and is currently on an azathioprine-based maintenance regimen. We have extensively reviewed all previous cases of dual AAV and have formulated an approach to diagnose and treat this rare entity.

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Dual anti-neutrophil cytoplasmic antibody-related pauci-immune crescentic glomerulonephritis in a patient with Sjögren’s syndrome

Lee

Kim

2016

Rheumatol Int

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Sjögren's syndrome is an autoimmune disease that primarily affects exocrine glands. Renal involvement of Sjögren's syndrome may lead to tubulointerstitial disease, whereas secondary glomerulopathies such as anti-neutrophil cytoplasmic antibody (ANCA)-related pauci-immune crescentic glomerulonephritis are rarely observed. In addition, crescent glomerulonephritis that is simultaneously positive for both myeloperoxidase (MPO)-ANCA and proteinase 3 (PR3)-ANCA has never been reported in Sjögren's syndrome. Here, we report a case of pauci-immune crescentic glomerulonephritis exhibiting positivity for both MPO- and PR3-ANCAs in a patient with primary Sjögren's syndrome. A 71-year-old female was hospitalized for cough, blood-tinged sputum, and dyspnea two weeks after diagnosis with Sjögren's syndrome. On admission, serum anti-nuclear antibody, anti-Ro/SS-A antibody, MPO-ANCA, and PR3-ANCA were all positive, and serum blood urea nitrogen and creatinine (Cr) levels were 42.7 and 2.9 mg/dL, respectively. On the seventh day of hospitalization, the patient's serum Cr level was 5.7 mg/dL, indicating rapidly progressive glomerulonephritis. Renal biopsy resulted in the diagnosis of ANCA-related pauci-immune crescentic glomerulonephritis, for which intravenous methylprednisolone (7 mg/kg/day) was administered for three consecutive days, followed by combination therapy with oral prednisolone (1 mg/kg/day) and intravenous cyclophosphamide (500 mg/m(2)). The patient was positive in the Schirmer's I test, and a salivary gland biopsy showed sialadenitis with lympho-plasmacytic infiltrations. On day 28 of hospitalization, the patient was discharged after amelioration of respiratory symptoms and azotemia. At 6 months after discharge, the patient continued to receive appropriate daily medications and was negative for both MPO- and PR3-ANCAs, with a slight elevation in serum Cr levels.

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ANCA-associated vasculitis with dual ANCA positivity in coexistence with mixed connective tissue disease

Cited by 6 publications

References 27 publications

Sustained Remission of Antineutrophil Cytoplasmic Antibody-Mediated Glomerulonephritis and Nephrotic Syndrome in Mixed Connective Tissue Disease

Sustained Remission of Antineutrophil Cytoplasmic Antibody-Mediated Glomerulonephritis and Nephrotic Syndrome in Mixed Connective Tissue Disease

Rare Case of Renal Limited Dual Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: Double Trouble!

Dual anti-neutrophil cytoplasmic antibody-related pauci-immune crescentic glomerulonephritis in a patient with Sjögren’s syndrome

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