Anti-hLAMP2-antibodies and dual positivity for anti-GBM and MPO-ANCA in a patient with relapsing pulmonary-renal syndrome
<p>Abstract</p> <p>Background</p> <p>Pulmonary-renal syndrome associated with anti-glomerular basement membrane (GBM) antibodies, also known as Goodpasture's syndrome, is a rare but acute and life-threatening condition. One third of patients presenting as anti-GBM...
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doaj-14468afcc18e4075b58c2b149871fbf42020-11-24T22:22:23ZengBMCBMC Nephrology1471-23692011-06-011212610.1186/1471-2369-12-26Anti-hLAMP2-antibodies and dual positivity for anti-GBM and MPO-ANCA in a patient with relapsing pulmonary-renal syndromeKistler ThomasWüthrich Rudolf PRegenass StephanGaspert ArianaEtter ChristophKain RenateCohen Clemens D<p>Abstract</p> <p>Background</p> <p>Pulmonary-renal syndrome associated with anti-glomerular basement membrane (GBM) antibodies, also known as Goodpasture's syndrome, is a rare but acute and life-threatening condition. One third of patients presenting as anti-GBM antibody positive pulmonary-renal syndrome or rapidly progressive glomerulonephritis are also tested positive for anti-neutrophil cytoplasmic antibodies (ANCA). Whilst anti-GBM disease is considered a non-relapsing condition, the long-term course of double-positive patients is less predictable.</p> <p>Case Presentation</p> <p>We report a patient with such dual positivity, who presented with pulmonary hemorrhage, crescentic glomerulonephritis and membranous nephropathy. Plasmapheresis in combination with immunosuppresive therapy led to a rapid remission but the disease relapsed after two years. The serum of the patient was tested positive for antibodies to human lysosomal membrane protein 2 (hLAMP2), a novel autoantigen in patients with active small-vessel vasculitis (SVV). The anti-hLAMP2 antibody levels correlated positively with clinical disease activity in this patient.</p> <p>Conclusion</p> <p>We hypothesize that this antibody may indicate a clinical course similar to ANCA-associated vasculitis in double-positive patients. However, this needs to be confirmed on comprehensive patient cohorts.</p> http://www.biomedcentral.com/1471-2369/12/26 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Kistler Thomas Wüthrich Rudolf P Regenass Stephan Gaspert Ariana Etter Christoph Kain Renate Cohen Clemens D |
spellingShingle |
Kistler Thomas Wüthrich Rudolf P Regenass Stephan Gaspert Ariana Etter Christoph Kain Renate Cohen Clemens D Anti-hLAMP2-antibodies and dual positivity for anti-GBM and MPO-ANCA in a patient with relapsing pulmonary-renal syndrome BMC Nephrology |
author_facet |
Kistler Thomas Wüthrich Rudolf P Regenass Stephan Gaspert Ariana Etter Christoph Kain Renate Cohen Clemens D |
author_sort |
Kistler Thomas |
title |
Anti-hLAMP2-antibodies and dual positivity for anti-GBM and MPO-ANCA in a patient with relapsing pulmonary-renal syndrome |
title_short |
Anti-hLAMP2-antibodies and dual positivity for anti-GBM and MPO-ANCA in a patient with relapsing pulmonary-renal syndrome |
title_full |
Anti-hLAMP2-antibodies and dual positivity for anti-GBM and MPO-ANCA in a patient with relapsing pulmonary-renal syndrome |
title_fullStr |
Anti-hLAMP2-antibodies and dual positivity for anti-GBM and MPO-ANCA in a patient with relapsing pulmonary-renal syndrome |
title_full_unstemmed |
Anti-hLAMP2-antibodies and dual positivity for anti-GBM and MPO-ANCA in a patient with relapsing pulmonary-renal syndrome |
title_sort |
anti-hlamp2-antibodies and dual positivity for anti-gbm and mpo-anca in a patient with relapsing pulmonary-renal syndrome |
publisher |
BMC |
series |
BMC Nephrology |
issn |
1471-2369 |
publishDate |
2011-06-01 |
description |
<p>Abstract</p> <p>Background</p> <p>Pulmonary-renal syndrome associated with anti-glomerular basement membrane (GBM) antibodies, also known as Goodpasture's syndrome, is a rare but acute and life-threatening condition. One third of patients presenting as anti-GBM antibody positive pulmonary-renal syndrome or rapidly progressive glomerulonephritis are also tested positive for anti-neutrophil cytoplasmic antibodies (ANCA). Whilst anti-GBM disease is considered a non-relapsing condition, the long-term course of double-positive patients is less predictable.</p> <p>Case Presentation</p> <p>We report a patient with such dual positivity, who presented with pulmonary hemorrhage, crescentic glomerulonephritis and membranous nephropathy. Plasmapheresis in combination with immunosuppresive therapy led to a rapid remission but the disease relapsed after two years. The serum of the patient was tested positive for antibodies to human lysosomal membrane protein 2 (hLAMP2), a novel autoantigen in patients with active small-vessel vasculitis (SVV). The anti-hLAMP2 antibody levels correlated positively with clinical disease activity in this patient.</p> <p>Conclusion</p> <p>We hypothesize that this antibody may indicate a clinical course similar to ANCA-associated vasculitis in double-positive patients. However, this needs to be confirmed on comprehensive patient cohorts.</p> |
url |
http://www.biomedcentral.com/1471-2369/12/26 |
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