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
Aleksandra Gmurczyk +5 more
doaj +2 more sources
Characterization of anti-GBM antibodies involved in Goodpasture's syndrome
Goodpasture's syndrome is a life threatening autoimmune kidney disease. The patients have autoantibodies to the glomerular basement membrane, which are specific for the C-terminal domain of type IV collagen (NC1). The major antigen has been localized to the alpha 3 (IV)-chain. We have investigated sera from 44 patients with anti-NC1 antibodies.
Hellmark, Thomas +2 more
core +4 more sources
Comparison of anti-GBM antibodies in sera with or without ANCA. [PDF]
An appreciable percentage of patients with serum anti-glomerular basement membrane (anti-GBM) antibodies also have antineutrophil cytoplasmic antibodies (ANCA), against either myeloperoxidase (MPO-ANCA), or proteinase 3 (PR3-ANCA). In sera without ANCA, the anti-GBM antibodies have been shown to react mainly with the noncollagenous domain (NC1) of Type
T, Hellmark +4 more
openaire +3 more sources
A case of aggressive atypical anti-GBM disease complicated by CMV pneumonitis
Background Anti-glomerular basement membrane (anti-GBM) disease is characterized by circulating IgG glomerular basement membrane antibodies and is clinically expressed as a rapidly progressive crescentic glomerulonephritis (GN), with 30–60% of patients ...
Barbora Sporinova +6 more
doaj +2 more sources
Goodpasture’s Syndrome with Negative Anti-glomerular Basement Membrane Antibodies [PDF]
A young male patient with rapidly progressive and life-threatening pulmonary haemorrhage due to anti-glomerular basement membrane (anti-GBM) antibody disease without renal involvement repeatedly tested negative for serum anti-GBM antibodies.
Tjitske Berends-De Vries +5 more
doaj +2 more sources
A 69 year old male developed rapid deterioration of kidney function and developed uremic symptoms. Creatinine was 486.2 µmol/L (5.5 mg/dl) on presentation. Anti-MPO was positive (titer>8 units, normal
Gaurang P Mavani +4 more
doaj +3 more sources
Plasma exchange in focal necrotizing glomerulonephritis without anti-GBM antibodies
To determine whether plasma exchange was of additional benefit in patients treated with oral immunosuppressive drugs for focal necrotizing glomerulonephritis (without anti-GBM antibodies), we performed a randomized controlled trial with stratification for renal function on entry.
Pusey, Charles D. +4 more
openaire +3 more sources
Anti-hLAMP2-antibodies and dual positivity for anti-GBM and MPO-ANCA in a patient with relapsing pulmonary-renal syndrome [PDF]
Background 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.
Kistler Thomas +6 more
doaj +2 more sources
Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients [PDF]
Stephen P Mcadoo +2 more
exaly +2 more sources
Effective management of necrotizing crescentic glomerulonephritis using an aggressive combination therapy including avacopan in a patient double-seropositive for anti-GBM antibodies and ANCA: a case report [PDF]
Atsuro Kawaji +2 more
exaly +2 more sources

