Clinical scenario: hemoptysis | breathlessness | cola-colored urine| anti-GBM antibodies
A 15-year-old boy presents with a 1-week history of hemoptysis, progressive breathlessness, and cola-colored urine. Examination reveals pallor, bilateral basal crackles, and hypertension (150/95 mmHg). Investigations show:
- Hemoglobin: 8.5 g/dL
- Serum creatinine: 3.4 mg/dL
- Urinalysis: 3+ blood, 2+ protein
- Urine microscopy: Red cell casts
- Chest radiograph: Diffuse bilateral alveolar infiltrates
Serological testing reveals positive anti-glomerular basement membrane (anti-GBM) antibodies.
What is the most appropriate initial treatment?
A. High-dose corticosteroids alone
B. High-dose corticosteroids and cyclophosphamide
C. High-dose corticosteroids, cyclophosphamide, and plasmapheresis
D. Rituximab alone
E. Hemodialysis only
Correct answer & Explanation:
Correct Answer: C. High-dose corticosteroids, cyclophosphamide, and plasmapheresis
.Explanation
The standard initial treatment of anti-GBM disease consists of:
- High-dose corticosteroids to suppress inflammation.
- Cyclophosphamide to stop further antibody production.
- Urgent plasmapheresis to remove circulating anti-GBM antibodies.
This combination has been the standard of care for decades and is recommended by KDIGO.
Why the other options are incorrect
- A. Corticosteroids alone: Insufficient because antibodies continue to be produced.
- B. Corticosteroids + cyclophosphamide: Important, but plasmapheresis is essential to rapidly remove circulating antibodies.
- D. Rituximab alone: May be used in selected cases when cyclophosphamide is contraindicated, but it is not first-line initial therapy.
- E. Hemodialysis only: Supports renal failure but does not treat the underlying autoimmune disease.
