Goodpasture's syndrome (Anti GBM disease)

Related Subjects: Granulomatosis with Polyangiitis GPA (Wegener's) | Goodpasture's syndrome (Anti GBM disease) | Respiratory Failure | Acute Kidney Injury |

Prognosis is very much related to the speed of initiation of treatment. Many labs can do a quick test for ANCA/Anti-GBM. Speak to renal unit as soon as suspected.

About

• Very rare with 1 case per million per year
• Induces a rapidly progressive Glomerulonephritis (GN)
• Diffuse proliferative GN with crescent formation
• Inflammatory response leads to collagen damage

Classification

• Goodpasture's affects Kidneys and Lungs.
• If kidney/lungs alone called Anti GBM disease

Aetiology

• Production of an antibody against Alpha 3 chain of Type 4 collagen
• Found in lungs and GBM

Clinical

• Lung haemorrhage - dyspnoea, haemoptysis
• Haematuria, Proteinuria
• Rarely and acute nephritic syndrome and RPGN
• Progressive renal failure over weeks

Investigations

• FBC: raised WCC, anaemia, elevated ESR/CRP
• U&E: AKI picture with Urea and Creatinine
• Urine: protein, blood, red cell casts.
• CXR : alveolar shadowing from haemorrhage <
• Check Anti-GBM antibody and ANCA
• Renal biopsy for Anti-GBM nephritis with Immunofluorescence shows linear ribbon-like deposition of IgG along GBM. Diffuse crescentic GN.

Differentials

• Wegener's Granulomatosis / Granulomatosis with Polyangiitis

Management (Urgent early renal consult)

• Treatment: steroids, cyclophosphamide and plasma exchange
• Urgent Plasmapheresis / Prednisolone 1 mg/kg, Cyclophosphamide, Azathioprine
• Renal transplant in end-stage renal disease (recurrence in graft possible but unusual except for Alport's syndrome. Anti GBM antibodies need to be reduced to prevent disease in a transplant kidney.