Anti-glomerular basement membrane disease due to monoclonal IgG Lambda antibodies: a very rare case of monoclonal gammopathy of renal significance

Peña, Camila; Valjalo, Ricardo; Rocca, Ximena; Roa, Macarena; Morales, Alejandra; Méndez, Gonzalo

doi:10.1007/s00277-018-3513-4

Cited by 3 publications

(1 citation statement)

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“…There are also rare cases in which IgG4 subclass and IgA (18,19), or monoclonal immunoglobulins (20,21), have been described. Anti-GBM antibodies may also target other alpha chains.…”

Section: Pathogenesismentioning

confidence: 99%

Goodpasture syndrome and anti-glomerular basement membrane disease

Reggiani

L’Imperio

Calatroni

et al. 2023

Clinical and Experimental Rheumatology

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Anti-glomerular basement membrane (anti-GBM) disease is a rare life-threatening small vessel vasculitis that typically affects the capillaries of kidneys and lungs, with most of patients developing rapidly progressive crescentic glomerulonephritis, and 40%-60% concomitant alveolar haemorrhage. It is caused by the deposition in alveolar and glomerular basement membrane of circulating autoantibodies directed against antigens intrinsic to the basement membrane. The exact mechanism that induces the formation of autoantibodies is unknown, but probably environmental factors, infections or direct damage to kidneys and lungs may trigger the autoimmune response in genetically susceptible individuals. Initial therapy includes corticosteroids and cyclophosphamide to prevent autoantibodies production, and plasmapheresis to remove the circulating autoantibodies. Good renal outcomes may be achieved by a prompt treatment initiation. However, when patients present with severe renal failure requiring dialysis or with a high proportion of glomerular crescents at biopsy, renal outcomes are bad. Relapses are rare and when renal involvement is present, the suspect of concomitant diseases, such as ANCA-associated vasculitis and membranous nephropathy, should be raised. Imlifidase is showing promising results, which if confirmed will cause a paradigm shift in the treatment of this disease.

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“…There are also rare cases in which IgG4 subclass and IgA (18,19), or monoclonal immunoglobulins (20,21), have been described. Anti-GBM antibodies may also target other alpha chains.…”

Section: Pathogenesismentioning

confidence: 99%