scholarly journals 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

2021 ◽  
Vol 12 (10) ◽  
pp. 405-410
Author(s):  
Vanessa Palha ◽  
Ana Sa ◽  
Teresa Pimentel ◽  
Narciso Oliveira ◽  
Sofia Rocha ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Rare Case ◽  
Proteinase 3 ◽  
Pulmonary Renal Syndrome

scholarly journals Atypical anti-glomerular basement membrane disease with anti-GBM antibody negativity and ANCA positivity: a case report

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Na Guo ◽  
Qinghua Yin ◽  
Song Lei ◽  
Yanjun He ◽  
Ping Fu
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Rare Case ◽  
Test Results ◽  
Serum Samples ◽  
Tissue Samples ◽  
Case Presentation ◽  
Severe Renal Insufficiency ◽  
Laboratory Test Results ◽  
Organ Specific

Abstract Background Anti-glomerular basement membrane (anti-GBM) disease is an organ-specific autoimmune disease that involves the lung and kidneys and leads to rapid glomerulonephritis progression, with or without diffuse alveolar hemorrhage, and even respiratory failure. Classic cases of anti-GBM disease are diagnosed based on the presence of the anti-GBM antibody in serum samples and kidney or lung biopsy tissue samples. However, atypical cases of anti-GBM disease are also seen in clinical practice. Case presentation We herein report the rare case of a patient with atypical anti-GBM disease whose serum was negative for the anti-GBM antibody but positive for the myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody (p-ANCA) and another atypical ANCA. Laboratory test results showed severe renal insufficiency with a creatinine level of 385 μmol/L. Renal biopsy specimen analysis revealed 100% glomeruli with crescents; immunofluorescence showed immunoglobulin G (IgG) linearly deposited alongside the GBM. Finally, the patient was discharged successfully after treatment with plasmapheresis, methylprednisolone and prednisone. Conclusion This patient, whose serum was negative for the anti-GBM antibody but positive for p-ANCA and another atypical ANCA, had a rare case of anti-GBM disease. Insights from this unusual case might help physicians diagnose rare forms of glomerulonephritis and treat affected patients in a timely manner.

scholarly journals Rare case of atypical crescentic glomerulonephritis and interstitial lung disease with negative anti-GBM antibody and positive anti-MPO antibody

2019 ◽  
Vol 12 (8) ◽  
pp. e229256 ◽  
Author(s):  
Alexander Hanna ◽  
Jenny Ross ◽  
Fernanda Heitor
Keyword(s):  
Hepatitis B ◽  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Rare Case ◽  
Crescentic Glomerulonephritis ◽  
Kidney Biopsy ◽  
Flank Pain ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Hepatitis B Infection ◽  
Double Positive

A 70-year-old man presented with 1 month of haematuria and mild right-sided flank pain with no other symptoms. Diagnostic workup included serum studies which showed the presence of antimyeloperoxidase antibodies, a kidney biopsy which demonstrated necrotising crescentic glomerulonephritis with linear immunofluorescence of the basement membrane, and electron microscopy which exhibited thickening of the glomerular basement membrane. Incidentally, the patient was discovered to have a latent hepatitis B infection, which complicated immunosuppressive therapy. He was treated with a course of plasmapheresis and methylprednisolone, followed by entecavir for hepatitis B prophylaxis, and finally by rituximab. This case of glomerulonephritis was notable for its resemblance to the better known Goodpasture’s disease. Typically, Goodpasture’s syndrome exists on a spectrum from seronegative disease to double-positive disease that presents with both anti–glomerular basement membrane (anti-GBM) and cytoplasmic-antineutrophil cytoplasmic antibodies/antiproteinase 3 antibodies (c-ANCA/anti-PR3). However, this patient’s glomerulonephritis was unique because he presented negative for anti-GBM antibodies and positive for perinuclear-antineutrophil cytoplasmic antibodies/antimyeloperoxidase antibodies (p-ANCA/anti-MPO).

scholarly journals Successful Treatment of Dual-Positive Anti-Myeloperoxidase and Anti-Glomerular Basement Membrane Antibody Vasculitis with Pulmonary-Renal Syndrome

2016 ◽  
Vol 6 (1) ◽  
pp. 1-7 ◽  
Author(s):  
Jinxian Huang ◽  
Ling Wu ◽  
Xiaoyan Huang ◽  
Yan Xie ◽  
Jinquan Yu ◽  
...  
Keyword(s):  
Pulmonary Fibrosis ◽  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Poor Prognosis ◽  
Mediastinal Mass ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Rapid Progression ◽  
Aggressive Treatment ◽  
Anca Associated Vasculitis ◽  
Pulmonary Renal Syndrome

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and anti-glomerular basement membrane (GBM) disease are two separate diseases, while sometimes they can coexist together. The exact mechanisms are not clear, but due to the rapid progression and poor prognosis, prompt and aggressive treatment is usually required. We treated with steroids combined with cyclophosphamide and rituximab an 84-year-old man with ANCA-associated vasculitis and anti-GBM disease who had prior pulmonary fibrosis and a coexisting anterosuperior mediastinal mass. Conventional therapy including steroids, plasmapheresis and cyclophosphamide failed to attenuate the anti-GBM disease, yet he responded to an alternative treatment of rituximab. This case suggests the efficacy of steroids and immunosuppressant for the treatment of a dual-positive case with an anterosuperior mediastinal mass.

scholarly journals Antigen-specific radioimmunoassays for anti-neutrophil cytoplasmic antibodies in the diagnosis of rapidly progressive glomerulonephritis.

1991 ◽  
Vol 2 (1) ◽  
pp. 27-36
Author(s):  
J L Niles ◽  
G L Pan ◽  
A B Collins ◽  
T Shannon ◽  
S Skates ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Renal Disease ◽  
Glomerular Basement Membrane ◽  
Crescentic Glomerulonephritis ◽  
Rapidly Progressive Glomerulonephritis ◽  
Diagnostic Value ◽  
Proteinase 3 ◽  
Serum Samples ◽  
Additional Control ◽  
Pathologic Findings

Circulating anti-neutrophil cytoplasmic antibodies (ANCA) have been described in most patients with "pauci-immune" necrotizing and crescentic glomerulonephritis. A 29-kDa serine protease (p29 or proteinase 3) and myeloperoxidase are the two best characterized antigens recognized by ANCA. The study presented here was conducted to define the diagnostic value of assays for antibodies against these two antigens in rapidly progressive glomerulonephritis. Radioimmunoassays were developed for anti-p29 and anti-myeloperoxidase antibodies, with purified antigens, and the results of the radioimmunoassays were compared with those obtained by immunofluorescence tests for ANCA. We performed assays on serum samples from 123 patients with the syndrome of rapidly progressive glomerulonephritis, as well as from 200 blood bank donors and from 717 additional control patients. Without knowledge of the results of ANCA tests, the renal pathologic findings in the 123 patients with rapidly progressive glomerulonephritis were analyzed, and 42 were classified as pauci-immune necrotizing and crescentic glomerulonephritis, 18 were classified as anti-glomerular basement membrane nephritis and 63 were classified as other forms of renal disease. We found radioimmunoassays to be more reliable in the diagnosis of pauci-immune necrotizing and crescentic glomerulonephritis than immunofluorescence testing. By radioimmunoassay, ANCA were found in 40 of 42 patients (95% sensitivity) with pauci-immune necrotizing and crescentic glomerulonephritis (14 with anti-p29 and 26 with anti-myeloperoxidase antibodies). The tests for antibodies to p29 and myeloperoxidase were 99.9 and 99.5% specific for pauci-immune necrotizing and crescentic glomerulonephritis, respectively. In the setting of rapidly progressive glomerulonephritis, a positive radioimmunoassay for anti-p29 or anti-myeloperoxidase antibodies (together with a negative test for anti-GBM antibodies) gives a probability of pauci-immune necrotizing and crescentic glomerulonephritis of over 99%.

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