PULMONARY RENAL SYNDROME SEROPOSITIVE FOR BOTH ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODIES AND ANTI-GLOMERULAR BASEMENT MEMBRANE AUTOANTIBODIES

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.

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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

Journal of Medical Cases ◽

10.14740/jmc3742 ◽

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

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Electron microscopic radioautographic studies on the incorporation of 3H proline in the glomerular basement membrane (GBM) in antistreptococcal-cell-membrane (SCM) injected mice

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100111057 ◽

1984 ◽

Vol 42 ◽

pp. 188-189

Author(s):

R.P. Nayyar ◽

C.F. Lange ◽

J. L. Borke

Keyword(s):

Body Weight ◽

Cell Membrane ◽

Basement Membrane ◽

Glomerular Basement Membrane ◽

Mesangial Matrix ◽

Electron Microscopic ◽

Group A ◽

Injection Protocol

Streptococcal cell membrane (SCM) antiserum injected mice show a significant thickening of glomerular basement membrane (GBM) and an increase in mesangial matrix within 4 to 24 hours of antiserum administration (1,2,3). This study was undertaken to evaluate the incorporation of 3H proline into glomerular cells and GBM under normal and anti-SCM induced conditions. Mice were administered, intraperitoneally, 0.1 ml of normal or anti-SCM serum followed by a 10 µC/g body weight injection of 3H proline. Details of the preparation of anti-SCM (Group A type 12 streptococcal pyogenes) and other sera and injection protocol have been described elsewhere (2). After 15 minutes of isotope injection a chase of cold proline was given and animal sacrificed at 20 minutes, 1,2,4,8,24 and 48 hours. One of the removed kidneys was processed for immunofluorescence, light and electron microscopic radioautographic studies; second kidney was used for GBM isolation and aminoacid analysis.

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