Anti-glomerular basement membrane (GBM)-antibody-mediated disease with normal renal function.

Published on Apr 1, 1998in Nephrology Dialysis Transplantation4.531
· DOI :10.1093/NDT/13.4.935
C Ang1
Estimated H-index: 1
Judy Savige39
Estimated H-index: 39
+ 4 AuthorsR S Sinclair1
Estimated H-index: 1
Background. This study compared the clinical and laboratory characteristics of patients with anti-glomerular basement membrane (GBM) disease and normal renal function, with those of patients with anti-GBM disease where there was renal impairment. Methods. The medical records of the 14 patients who had presented with anti-GBM disease to our hospital in the past 20 years were reviewed. Results. Five (36%) had a normal serum creatinine or creatinine clearance at presentation. Other features were haemoptysis (2/5, 40%), macroscopic haematuria (2/5, 40%) or systemic symptoms (1/5, 20%). All five (100%) had some degree of haematuria, four (80%) had proteinuria of at least 1 g/day, and none was hypertensive. Anaemia, a raised WCC, or elevated ESR (>35 mm/h) occurred less often than in patients with impaired renal function (P 1 g/day in two (40%). Eight of the nine (89%) patients with impaired renal function survive, but all are currently being dialysed or have had a renal transplant. Conclusion. Patients with anti-GBM disease with normal renal function are not uncommon, and often have a good prognosis. There is less renal damage, possibly because of lower levels of circulating anti-GBM antibodies and less glomerular complement deposition.
📖 Papers frequently viewed together
422 Citations
210 Citations
4 Authors (Zhao Cui, ..., Hongya Wang)
36 Citations
Cited By40
#1Sandra Wymann (CSL Behring)H-Index: 10
#2Yun Dai (CSL Limited)
Last. Matthew P. Hardy (CSL Limited)H-Index: 10
view all 23 authors...
Human complement receptor 1 (HuCR1) is a pivotal regulator of complement activity, acting on all three complement pathways as a membrane-bound receptor of C3b/C4b, as a C3/C5 convertase decay accelerator, and as a co-factor for Factor I-mediated cleavage of C3b and C4b. In this study, we sought to identify a minimal soluble fragment of HuCR1 which retains the complement regulatory activity of the wild type protein. To this end, we generated recombinant, soluble, truncated versions of HuCR1 and c...
#1Dandan LiangH-Index: 2
#2Shaoshan Liang (NU: Nanjing University)H-Index: 14
Last. Caihong ZengH-Index: 35
view all 8 authors...
Aim To explore the clinicopathological characteristics of patients with anti-GBM antibody-negative anti-GBM disease. Methods The clinical and renal pathological findings were retrospectively studied in 19 patients. All patients met the following inclusion criteria: linear GBM IgG deposition on immunofluorescence(IF); and lack of serum anti-GBM antibodies by ELISA and indirect immunofluorescence assay. Results There were 17 male and two female patients, with a median age of 36 years (range 15–61 ...
6 CitationsSource
#1Vadim Pedchenko (VUMC: Vanderbilt University Medical Center)H-Index: 19
#2A. Richard Kitching (Monash University)H-Index: 56
Last. Billy G. Hudson (VUMC: Vanderbilt University Medical Center)H-Index: 77
view all 3 authors...
Abstract Goodpasture's (GP) disease is an autoimmune disorder characterized by the deposition of pathogenic autoantibodies in basement membranes of kidney and lung eliciting rapidly progressive glomerulonephritis and pulmonary hemorrhage. The principal autoantigen is the α345 network of collagen IV, which expression is restricted to target tissues. Recent discoveries include a key role of chloride and bromide for network assembly, a novel posttranslational modification of the antigen, a sulfilim...
14 CitationsSource
#1Stephen P. McAdoo (Imperial College London)H-Index: 18
#2Charles D. Pusey (Imperial College London)H-Index: 92
Antiglomerular basement membrane (anti-GBM) disease is a rare but life-threatening autoimmune vasculitis that is characterized by the development of pathogenic autoantibodies to type IV collagen antigens expressed in the glomerular and alveolar basement membranes. Once deposited in tissue, these autoantibodies incite a local capillaritis which manifests as rapidly progressive glomerulonephritis (GN) in 80 to 90% of patients, and with concurrent alveolar hemorrhage in ∼50%. A small proportion of ...
8 CitationsSource
#1Emily HoltH-Index: 1
#2Andrew GoughH-Index: 1
Last. Fahmid U. ChowdhuryH-Index: 18
view all 4 authors...
: A 30-year-old woman presented with lethargy, night sweats, and fever with raised inflammatory markers. Anti-neutrophil cytoplasmic antibody was negative. Abdominopelvic CT was unremarkable. Subsequently, she underwent FDG PET/CT showing globally enlarged kidneys with diffuse hypermetabolic activity within the renal parenchyma bilaterally. Renal biopsies showed morphologic features of an active necrotizing crescentic glomerulonephritis, which was confirmed clinically and treated. This case demo...
1 CitationsSource
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. Although rare, anti-GBM antibody disease should be considered in the differential diagnosis in patients with life-threatening pulmonary haemorrhage due to isolated diffuse alveolar haemorrhage. Enzyme-linked-immunosorbent assay (ELISA) testing for anti-GBM a...
5 CitationsSource
#1Mary H. Foster (Duke University)H-Index: 18
Abstract Basement membrane components are targets of autoimmune attack in diverse diseases that destroy kidneys, lungs, skin, mucous membranes, joints, and other organs in man. Epitopes on collagen and laminin, in particular, are targeted by autoantibodies and T cells in anti-glomerular basement membrane glomerulonephritis, Goodpasture's disease, rheumatoid arthritis, post-lung transplant bronchiolitis obliterans syndrome, and multiple autoimmune dermatoses. This review examines major diseases l...
15 CitationsSource