A Case of Simultaneous, Biopsy-Proven, Classic, ANCA-Positive Wegener's Granulomatosis and Anti-GBM Disease, but without Detectible Circulating Anti-GBM Antibodies
Wegener's granulomatosis (WG) is a systemic, necrotizing, granulomatous vasculitis of unknown etiology. Approximately 75% of cases present as classic WG with both pulmonary and renal involvement, while the remaining 25% of patients present with a limited form with either predominantly upper or...
Saved in:
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2010-01-01
|
| Series: | The Scientific World Journal |
| Online Access: | http://dx.doi.org/10.1100/tsw.2010.107 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832563110939983872 |
|---|---|
| author | Aleksandra Gmurczyk Shubhada N. Ahya Robert Goldschmidt George Kim L. Tammy Ho Kevin Nash |
| author_facet | Aleksandra Gmurczyk Shubhada N. Ahya Robert Goldschmidt George Kim L. Tammy Ho Kevin Nash |
| author_sort | Aleksandra Gmurczyk |
| collection | DOAJ |
| description | Wegener's granulomatosis (WG) is a systemic, necrotizing, granulomatous vasculitis of unknown etiology. Approximately 75% of cases present as classic WG with both pulmonary and renal involvement, while the remaining 25% of patients present with a limited form with either predominantly upper or lower respiratory tract symptoms. Ninety percent of WG patients have circulating anti–neutrophil cytoplasmic antibodies (ANCA), and approximately 10% have both circulating ANCA antibodies and concomitant anti–glomerular basement membrane (anti-GBM) disease on renal biopsy. Virtually all of these patients also have circulating anti-GBM antibodies. While it has been reported that some patients with ANCA vasculitis have circulating anti-GBM antibodies, and patients with anti-GBM disease may have positive ANCA, review of the literature does not demonstrate other cases of biopsy-proven, simultaneous, ANCA-associated vasculitis and anti-GBM disease. We report a case of simultaneous, biopsy-proven, classic, ANCA-positive WG and anti-GBM disease, but without detectible circulating anti-GBM antibodies. We present findings characteristic of both WG and linear IgG deposition along the GBM suggesting concurrent anti-GBM disease, in the absence of detectable circulating anti-GBM antibodies. Possible theories to explain the absence of these antibodies are discussed. |
| format | Article |
| id | doaj-art-aa32d025755345f38528ffd1efbb75ee |
| institution | Kabale University |
| issn | 1537-744X |
| language | English |
| publishDate | 2010-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | The Scientific World Journal |
| spelling | doaj-art-aa32d025755345f38528ffd1efbb75ee2025-02-03T01:20:57ZengWileyThe Scientific World Journal1537-744X2010-01-01101078108310.1100/tsw.2010.107A Case of Simultaneous, Biopsy-Proven, Classic, ANCA-Positive Wegener's Granulomatosis and Anti-GBM Disease, but without Detectible Circulating Anti-GBM AntibodiesAleksandra Gmurczyk0Shubhada N. Ahya1Robert Goldschmidt2George Kim3L. Tammy Ho4Kevin Nash5Evanston Northwestern Healthcare, Northwestern University Feinberg School of Medicine, Chicago, USAEvanston Northwestern Healthcare, Northwestern University Feinberg School of Medicine, Chicago, USAEvanston Northwestern Healthcare, Northwestern University Feinberg School of Medicine, Chicago, USAEvanston Northwestern Healthcare, Northwestern University Feinberg School of Medicine, Chicago, USAEvanston Northwestern Healthcare, Northwestern University Feinberg School of Medicine, Chicago, USAEvanston Northwestern Healthcare, Northwestern University Feinberg School of Medicine, Chicago, USAWegener's granulomatosis (WG) is a systemic, necrotizing, granulomatous vasculitis of unknown etiology. Approximately 75% of cases present as classic WG with both pulmonary and renal involvement, while the remaining 25% of patients present with a limited form with either predominantly upper or lower respiratory tract symptoms. Ninety percent of WG patients have circulating anti–neutrophil cytoplasmic antibodies (ANCA), and approximately 10% have both circulating ANCA antibodies and concomitant anti–glomerular basement membrane (anti-GBM) disease on renal biopsy. Virtually all of these patients also have circulating anti-GBM antibodies. While it has been reported that some patients with ANCA vasculitis have circulating anti-GBM antibodies, and patients with anti-GBM disease may have positive ANCA, review of the literature does not demonstrate other cases of biopsy-proven, simultaneous, ANCA-associated vasculitis and anti-GBM disease. We report a case of simultaneous, biopsy-proven, classic, ANCA-positive WG and anti-GBM disease, but without detectible circulating anti-GBM antibodies. We present findings characteristic of both WG and linear IgG deposition along the GBM suggesting concurrent anti-GBM disease, in the absence of detectable circulating anti-GBM antibodies. Possible theories to explain the absence of these antibodies are discussed.http://dx.doi.org/10.1100/tsw.2010.107 |
| spellingShingle | Aleksandra Gmurczyk Shubhada N. Ahya Robert Goldschmidt George Kim L. Tammy Ho Kevin Nash A Case of Simultaneous, Biopsy-Proven, Classic, ANCA-Positive Wegener's Granulomatosis and Anti-GBM Disease, but without Detectible Circulating Anti-GBM Antibodies The Scientific World Journal |
| title | A Case of Simultaneous, Biopsy-Proven, Classic, ANCA-Positive Wegener's Granulomatosis and Anti-GBM Disease, but without Detectible Circulating Anti-GBM Antibodies |
| title_full | A Case of Simultaneous, Biopsy-Proven, Classic, ANCA-Positive Wegener's Granulomatosis and Anti-GBM Disease, but without Detectible Circulating Anti-GBM Antibodies |
| title_fullStr | A Case of Simultaneous, Biopsy-Proven, Classic, ANCA-Positive Wegener's Granulomatosis and Anti-GBM Disease, but without Detectible Circulating Anti-GBM Antibodies |
| title_full_unstemmed | A Case of Simultaneous, Biopsy-Proven, Classic, ANCA-Positive Wegener's Granulomatosis and Anti-GBM Disease, but without Detectible Circulating Anti-GBM Antibodies |
| title_short | A Case of Simultaneous, Biopsy-Proven, Classic, ANCA-Positive Wegener's Granulomatosis and Anti-GBM Disease, but without Detectible Circulating Anti-GBM Antibodies |
| title_sort | case of simultaneous biopsy proven classic anca positive wegener s granulomatosis and anti gbm disease but without detectible circulating anti gbm antibodies |
| url | http://dx.doi.org/10.1100/tsw.2010.107 |
| work_keys_str_mv | AT aleksandragmurczyk acaseofsimultaneousbiopsyprovenclassicancapositivewegenersgranulomatosisandantigbmdiseasebutwithoutdetectiblecirculatingantigbmantibodies AT shubhadanahya acaseofsimultaneousbiopsyprovenclassicancapositivewegenersgranulomatosisandantigbmdiseasebutwithoutdetectiblecirculatingantigbmantibodies AT robertgoldschmidt acaseofsimultaneousbiopsyprovenclassicancapositivewegenersgranulomatosisandantigbmdiseasebutwithoutdetectiblecirculatingantigbmantibodies AT georgekim acaseofsimultaneousbiopsyprovenclassicancapositivewegenersgranulomatosisandantigbmdiseasebutwithoutdetectiblecirculatingantigbmantibodies AT ltammyho acaseofsimultaneousbiopsyprovenclassicancapositivewegenersgranulomatosisandantigbmdiseasebutwithoutdetectiblecirculatingantigbmantibodies AT kevinnash acaseofsimultaneousbiopsyprovenclassicancapositivewegenersgranulomatosisandantigbmdiseasebutwithoutdetectiblecirculatingantigbmantibodies AT aleksandragmurczyk caseofsimultaneousbiopsyprovenclassicancapositivewegenersgranulomatosisandantigbmdiseasebutwithoutdetectiblecirculatingantigbmantibodies AT shubhadanahya caseofsimultaneousbiopsyprovenclassicancapositivewegenersgranulomatosisandantigbmdiseasebutwithoutdetectiblecirculatingantigbmantibodies AT robertgoldschmidt caseofsimultaneousbiopsyprovenclassicancapositivewegenersgranulomatosisandantigbmdiseasebutwithoutdetectiblecirculatingantigbmantibodies AT georgekim caseofsimultaneousbiopsyprovenclassicancapositivewegenersgranulomatosisandantigbmdiseasebutwithoutdetectiblecirculatingantigbmantibodies AT ltammyho caseofsimultaneousbiopsyprovenclassicancapositivewegenersgranulomatosisandantigbmdiseasebutwithoutdetectiblecirculatingantigbmantibodies AT kevinnash caseofsimultaneousbiopsyprovenclassicancapositivewegenersgranulomatosisandantigbmdiseasebutwithoutdetectiblecirculatingantigbmantibodies |