Abstract
Background
Despite the previous reports of patients with combined anti-glomerular basement membrane (anti-GBM) disease occurring sequentially or simultaneously with other nephropathies, most of them have been reported seperately. The complication of these diseases is rare and the mechanism is not clear, and their immuno-antibodies, microscopic picture, clinical presentation, treatment and prognosis are different, therefore, we presented the collection of anti-GBM with combined disease such as membranous nephropathy (MN), anti-neutrophil cytoplasmic antibody (ANCA), IgA nephropathy and atypical anti-GBM to systematically characterized the epidemiological features, clinical manifestations, pathological features and herapeutic outcomes through a summative review.
Method
We retrospectively a case series of 39 anti-GBM diseases from a single center in Northwest China from 2011–2023.
Results
A total of 39 patients with anti-GBM disease including 19 males and 20 females were collected with a mean age of 50.0 ± 14.6 years. Among them there were 22 cases (22/39,56.4%) of anti-GBM alone, 6 cases (6/39,15.4%) of combined ANCA, 6 cases (6/39,15.4%) of combined MN, 2 cases (2/39,5.1%) of combined IgAN, and 3 cases (3/39,7.7%) of atypical anti-GBM nephropathy. The mean duration of the disease was 2.6 ± 6.2 months. Clinical symptoms were dominated by fever 68.2% (15/22), oliguria/anuria 63.6% (14/22), and microscopic haematuria 88.9% (16/22,) in the anti-GBM alone group, and nausea and vomiting 50% (3/6) in the anti-GBM + MN group, and edema 83.3% (5/6) in the anti-GBM + MN group. The proportion of patients requiring hemodialysis (HD) at the first visit was 79.5% (31/39) in all patients, 56.4% (22/39) in the anti-GBM alone group, 66.7% (4/6) in the anti-GBM + ANCA group, 66.7% (4/6) in the anti-GBM + MN group, and 100% (2/2) in the anti-GBM + IgAN group. Among them, 56.4% had more than 85% glomerular involvement. The proportion of sclerotic glomeruli was higher in the anti-GBM and anti-GBM + ANCA groups. The highest percentage of cellular crescents was found in the anti-GBM group as long with fibrous crescents in the anti-GBM + ANCA group. Immunofluorescence staining demonstrated positive IgG and C3 staining in all subgroups.
Conclusions
We concluded that the complication of other nephritis is another potential risk factor for anti-GBM, which is directly attributable to the adverse effects of the different immune depositions and pathological features on renal function, as timely intervention in patients with different pathological features is crucial.