Antiglomerular basement membrane (anti-GBM) disease with clinical and histological features that bridge the typical to atypical spectrum

Abstract

We describe antiglomerular basement membrane (anti-GBM) disease with rapidly progressive glomerulonephritis and concurrent parainfluenza pneumonia. Circulating anti-GBM antibodies were barely detectable and disappeared rapidly following corticosteroids, cyclophosphamide and plasma exchange. Kidney biopsy demonstrated strong linear GBM staining for IgG and IgG4 and unusually prominent endocapillary hypercellularity, suggesting ‘atypical anti-GBM disease’, although glomerular necrosis and crescents were also seen. When kidney function deteriorated further, despite persistently absent circulating anti-GBM antibodies, a repeat kidney biopsy was performed, showing crescents in 100% of glomeruli with ongoing endocapillary hypercellularity and strong IgG and IgG4 GBM staining. This case highlights complexities in the diagnosis of anti-GBM disease, with clinical and histological features bridging the atypical to typical anti-GBM disease spectrum. We hypothesise that these findings might be explained by the presence of IgG4 (rather than traditional IgG1 or IgG3) autoantibodies. To our knowledge, this is also the first report of parainfluenza associated with anti-GBM disease.

A case report of atypical antiglomerular basement membrane nephritis associated with Mycobacterium Avium

“…To the best of our knowledge, this is the first case report of atypical anti-GBM nephritis associated with NTM. Atypical anti-GBM nephritis has been described in association with other infectious agents (for example Staphylococcus aureus [ 5 ], bacterial pneumonia [ 6 ], parainfluenza [ 7 ], and possibly also cytomegalovirus [ 8 ]). In the largest published cohort of atypical anti-GBM nephritis, ~ 13% of cases were associated with a prodromal infection [ 9 ].…”

“…In one of the previously reported cases of infection-related atypical anti-GBM nephritis (suspected S. aureus (not confirmed by culture or PCR) and no biopsy of lung or kidney tissue) [ 5 ], clinical remission was achieved after 8 weeks of antibiotic treatment without the use of immunosuppressive therapy. In the remaining cases of atypical anti-GBM nephritis associated with bacterial pneumonia [ 6 ], parainfluenza [ 7 ], and cytomegalovirus [ 8 ], kidney function declined despite concomitant treatment with antibiotics/antivirals, immunosuppression, and plasmapheresis. However, given the slow replication rate of NTM and long duration of antibiotic treatment necessary to treat the infection (12 months after culture conversion [ 10 ]), it is possible that regardless of concurrent immunosuppressive treatment, the infection in our case report could not be cleared fast enough to allow for immunological quiescence and recovery of kidney function.…”

Great prognosis of concurrent anti-GBM disease and IgA nephropathy in a young woman: A case report

“…Anti-glomerular basement membrane (anti-GBM) disease is a rare autoimmune condition characterized by linear deposition of immunoglobulin G along the GBM. [ 1 ] Anti-GBM disease is mediated by circulating autoantibodies and its main autoantigen is the NC1 domain of the α3 chains of collagen IV on GBM. [ 2 ] Clinically, it classically presents as rapidly progressive glomerulonephritis (RPGN), and histologically, it is closely associated with crescentic glomerulonephritis.…”