Abstract
‘Double-positive’ with anti-glomerular basement membrane (anti-GBM) antibody and anti-neutrophil cytoplasmic antibody (ANCA) is an extremely rare cause of small vessel vasculitis in children. Studies have reported a distinct hybrid phenotype in double-positive patients which requires an aggressive treatment approach. The data on double positive children are scarce with few case reports available with varied outcomes. A case of 9-year-old girl from India who had double-positive anti-GBM disease is reported here. The patient presented with complaints of edema, oliguria, gross hematuria with rising creatine and was diagnosed as rapidly progressive glomerulonephritis (RPGN). Histological examinations of linear immunoglobulin G deposits along with glomerular capillaries were suggestive of anti-GBM disease. The anti-GBM and p-ANCA antibody titers were also high, and the aforementioned findings led to a diagnosis of ‘double-positive’ anti-GBM with RPGN. She was treated with standard plasma exchange therapy along with pulse methyl prednisolone (3 doses of 30 mg/kg/day on Days 1 to 3), oral prednisolone (1.5 mg/kg on Day 4), single intravenous injection of cyclophosphamide (500 mg/m2 on Day 5) and mycophenolate mofetil (MMF; 1000 mg/m2 on Day 6) followed by maintenance treatment with oral prednisolone (1 mg/kg/day) and MMF (800 mg/m2/day) from Day 7 onwards. Overall, the anti-GBM and p-ANCA levels declined throughout the treatment period but the patient progressed towards end stage renal disease.
Highlights
The anti-glomerular basement membrane disease was first described in a patient by Goodpasture as “Goodpasture syndrome” almost a century ago (1919), [1] and different clinical phenotypes have been described since [2]
Only 7 double-positive children aged
We report the first case of a “double-positive” anti-GBM disease with crescentic
Summary
The anti-glomerular basement membrane (anti-GBM) disease was first described in a patient by Goodpasture as “Goodpasture syndrome” almost a century ago (1919), [1] and different clinical phenotypes have been described since [2]. The presentation of anti-GBM antibody and anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV), termed as ‘double-positive’ is a rare phenomenon [3, 4]. An estimated 50% patients with anti-GBM disease have AAV whereas an estimated 10% patients with AAV present with anti-GBM antibody [3]. Studies have reported poor prognosis in the double-positive cases versus as reported for individual AAV or anti-GBM diseases [3]. Anti-GBM disease is uncommon in adults and less reported in the pediatric age group. Double-positive cases are extremely rare in children. Only 7 double-positive children aged
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