(anti-GBM antibody disease)

Incidence is about 1/106/ year. Men are more frequently affected than women (2 to 9 : 1). It is also more frequent in Causasian population and Maori's in New-Zealand. Dysimmune disease caused by the presence of IgG1 antibodies directed against the NC1 domain of the alpha-3 chain of type IV collagen. This channel is mainly located in the renal glomeruli and in the basal membrane of the pulmonary alveoli. HLA DRB1 * 1501 and DRB1 * 1502 groups are found in 70-80 % of patients. Some environmental factors can increase the risk: solvents (wed killers), cocaine, smoking, antilymphocytic treatment, some viral and bacterial infections.

The classical form combines:

-        a rapidly progressive extra-capillary proliferative glomeruronephritis:  micro- or macroscopic hematuria, non nephrotic proteinuria

-        an intra-alveolar hemorrhage: hemoptysis (48%), yspnea (35%), cough

-   the presence of serum autoantibodies; ANCA antibodies (anti-cytoplasm of anti-myeloperoxidase type polymorphonuclear neutrophils) may be associated with worse prognosis

An isolated involvement of the lungs or the kidneys is possible.

This syndrome is part of the pneumorenal syndromes as the Wegener granulomatosis, the polyangeitis, the Church-Strauss syndrome some types of lupus (see these terms).

Treatment: corticosteroids, plasma exchange and immunosuppression (cyclophosphamide).

Rituximab is used in case of poor response to those treatments.

It is recommended to wait for one year long extrarenal remission before considering kidney transplantation because there is a risk of post-transplantion recurrence.

Anesthetic implications:

check renal function, total blood count and SpO2 at room air; adapt perioperative fluid replacement to renal function.

References :

-                Dorval G, Guérin S, Berteloot L, Krid S, Salomon R, Galmiche-Roland L, Boyer O.
Syndrome de Goodpasture et maladie des anticorps anti-membrane basale chez l’enfant : revue de la littérature.
Arch Pédiatr 2017 ; 27 : 1019-28.

Updated: May 2019