A Case of Simultaneous, Biopsy-Proven, Classic, ANCA-Positive Wegener's Granulomatosis and Anti-GBM Disease, but without Detectible Circulating Anti-GBM Antibodies

Autor: Shubhada N. Ahya, Robert A. Goldschmidt, Aleksandra Gmurczyk, Kevin Nash, George Kim, L. Tammy Ho
Rok vydání: 2010
Předmět:
Pathology
medicine.medical_specialty
Biopsy
lcsh:Medicine
Enzyme-Linked Immunosorbent Assay
Disease
urologic and male genital diseases
lcsh:Technology
General Biochemistry
Genetics and Molecular Biology
Antibodies
Antineutrophil Cytoplasmic
Glomerular Basement Membrane
medicine
Humans
anti-GBM antibody
lcsh:Science
Aged
Autoantibodies
General Environmental Science
Case Study
medicine.diagnostic_test
biology
lcsh:T
ANCA
urogenital system
business.industry
lcsh:R
Granulomatosis with Polyangiitis
Wegener’s granulomatosis
General Medicine
medicine.disease
female genital diseases and pregnancy complications
nervous system diseases
medicine.anatomical_structure
Concomitant
Immunology
kidney injury
biology.protein
Etiology
lcsh:Q
Female
Renal biopsy
Antibody
Vasculitis
business
Respiratory tract
Zdroj: The Scientific World Journal
The Scientific World Journal, Vol 10, Pp 1078-1083 (2010)
ISSN: 1537-744X
DOI: 10.1100/tsw.2010.107
Popis: 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.
Databáze: OpenAIRE
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