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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Main Authors: Kistler Thomas, Wüthrich Rudolf P, Regenass Stephan, Gaspert Ariana, Etter Christoph, Kain Renate, Cohen Clemens D
Format: Article
Language:English
Published: BMC 2011-06-01
Series:BMC Nephrology
Online Access:http://www.biomedcentral.com/1471-2369/12/26
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spelling 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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