Who manages Goodpasture syndrome?

Management of Goodpasture Syndrome

Goodpasture syndrome requires immediate management by a multidisciplinary team centered around nephrologists with expertise in rapidly progressive glomerulonephritis, supported by pulmonologists for pulmonary hemorrhage, and intensivists when critical care is needed. 1

Primary Management Team

Nephrology as the Core Specialty

• Nephrologists should lead the management of Goodpasture syndrome given the predominant renal involvement with rapidly progressive glomerulonephritis and the need for urgent decisions regarding plasmapheresis, immunosuppression, and dialysis 1, 2
• The nephrologist coordinates diagnostic kidney biopsy, which typically shows necrotizing and crescentic glomerulonephritis with linear IgG staining on the glomerular basement membrane 1, 3
• Nephrology expertise is critical for determining prognosis based on serum creatinine levels (particularly whether >500 μmol/L or approximately 5.7 mg/dL), percentage of crescents on biopsy, and dialysis dependence at presentation 1, 2, 4

Pulmonology Involvement

• Pulmonologists must be involved when pulmonary hemorrhage is present, which occurs in the majority of cases presenting as pulmonary-renal syndrome 1, 5, 4
• They manage hemoptysis, dyspnea, and monitor for diffuse alveolar hemorrhage through bronchoscopy and imaging 3, 6
• Pulmonary involvement requires urgent assessment as it can be life-threatening and influences the decision to proceed with aggressive treatment even in dialysis-dependent patients 1

Specialized Treatment Services

Apheresis/Plasmapheresis Services

• Immediate access to plasmapheresis is essential as treatment should begin without delay once diagnosis is confirmed or highly suspected 1, 2
• The typical regimen involves 200-250 mL/kg over five sessions during 1-2 weeks, or a median of 13 sessions based on clinical response 2
• Plasmapheresis services must be available urgently, as delays in treatment significantly worsen renal outcomes 1, 5

Nephrology Intensive Care or Critical Care

• Intensive care management is often required for patients with severe pulmonary hemorrhage, respiratory failure, or hemodynamic instability from dysautonomia 4, 6
• Critical care teams manage mechanical ventilation when needed for pulmonary hemorrhage and monitor for complications of aggressive immunosuppression 2, 4

Supporting Specialists

Rheumatology or Immunology Consultation

• Rheumatologists or immunologists may assist with immunosuppressive regimen management, particularly cyclophosphamide dosing (oral versus intravenous) and monitoring for treatment complications 2
• They help distinguish Goodpasture syndrome from other pulmonary-renal syndromes, particularly in the 30% of patients who are double-positive for anti-GBM antibodies and ANCA 1, 4

Infectious Disease

• Infectious disease consultation becomes critical as severe infection is a leading cause of mortality (7 of 16 deaths in one large cohort) in patients receiving aggressive immunosuppression 2
• They guide antimicrobial prophylaxis and manage infections that arise during cyclophosphamide and high-dose corticosteroid therapy 2

Transplant Nephrology (Long-term)

• Transplant nephrologists manage patients who progress to end-stage renal disease and require kidney transplantation 1
• Transplantation must be deferred until anti-GBM antibodies have been undetectable for a minimum of 6 months to prevent recurrence in the allograft 1

Organizational Structure

Specialized Center Requirements

• Patients should be managed at centers with experience in rapidly progressive glomerulonephritis and immediate access to plasmapheresis, similar to the organizational structure recommended for complex conditions requiring multidisciplinary care 7
• The center must have 24/7 availability for urgent kidney biopsy, plasmapheresis initiation, and dialysis 1
• Dedicated hospital beds under nephrology service are essential for initial aggressive treatment and managing complications 7

Coordination and Follow-up

• A designated coordinator (typically a specialized nurse) should manage the complex treatment schedule involving plasmapheresis sessions, immunosuppression administration, and monitoring 7
• Long-term monitoring for at least 2 years is necessary to detect rare relapses, though recurrence occurs in only 2-5% of patients 1, 5

Treatment Decision Algorithm

Immediate Treatment Candidates

Aggressive treatment with cyclophosphamide, corticosteroids, and plasmapheresis should be initiated immediately for all patients EXCEPT those meeting ALL three criteria: (1) dialysis-dependent at presentation, (2) 100% crescents or >50% global glomerulosclerosis on biopsy, and (3) no pulmonary hemorrhage 1

Prognostic Stratification

• Patients with serum creatinine <500 μmol/L at presentation have significantly better renal survival and should receive full aggressive therapy 2
• Age <60 years correlates with improved overall survival 2
• The presence of pulmonary hemorrhage mandates aggressive treatment regardless of renal status 1

Common Pitfalls

• Delaying treatment while awaiting anti-GBM antibody results: Approximately 10% of cases are seronegative, and treatment should begin based on clinical suspicion and proceed with kidney biopsy confirmation 1, 3
• Underestimating infection risk: Severe infections are the leading cause of death in treated patients, requiring vigilant monitoring and low threshold for antimicrobial therapy 2
• Failing to recognize double-positive (anti-GBM and ANCA) patients who may require modified treatment approaches 1, 4