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Berger disease: Henoch-Schönlein syndrome without the rash
Abstract:
Identical 7-year-old twin boys each had a proved adenovirus infection at the same time. A few days later one developed florid Henoch-Schönlein purpura, severe alimentary tract symptoms, and transient joint symptoms. He had an acute nephritic syndrome, which progressed to nephrotic syndrome and renal insufficiency. Biopsy showed severe proliferative glomerulonephritis with crescents and marked deposition of IgA, IgG, C3, and fibrin. The second twin had hematuria and abdominal pain but no rash. Biopsy showed mesangial proliferative glomerulonephritis with mesangial deposits of IgA and, to a lesser extent, IgG and C3. The appearance was characteristic of Berger disease, and the subsequent clinical course has been that of symptomless microscopic hematuria and recurrent macroscopic hematuria with normal renal function. Immunologic studies have not revealed why these identical twins responded differently to the same provocation. Perhaps Berger disease may be considered a variant of Henoch-Schönlein nephritis.
Insights
Identical twins exposed to adenovirus developed different kidney diseases. One had Henoch-Schönlein purpura nephritis, while the other developed Berger disease, suggesting a potential link.
Area of Science:
- Nephrology
- Immunology
- Pediatrics
Background:
- Adenovirus infections can trigger various post-infectious glomerulonephritis syndromes.
- Henoch-Schönlein purpura nephritis and Berger disease are IgA-mediated kidney diseases with overlapping features.
Observation:
- Identical twins simultaneously contracted adenovirus.
- One twin developed Henoch-Schönlein purpura with severe systemic and renal involvement, including crescentic glomerulonephritis.
- The other twin presented with milder symptoms, diagnosed as Berger disease (IgA nephropathy) based on biopsy and clinical course.
Findings:
- Renal biopsy in the first twin revealed severe proliferative glomerulonephritis with crescents and IgA, IgG, C3, and fibrin deposition.
- Renal biopsy in the second twin showed mesangial proliferative glomerulonephritis with IgA, IgG, and C3 deposits, characteristic of Berger disease.
- Despite identical genetic background and similar adenovirus provocation, the twins exhibited distinct clinical and pathological renal outcomes.
Implications:
- This case highlights the variable presentations of post-infectious glomerulonephritis even in genetically identical individuals.
- It raises questions about the specific immunopathogenesis and potential environmental or subtle genetic factors influencing disease manifestation.
- Berger disease may represent a milder or variant form of Henoch-Schönlein nephritis, particularly in the context of specific triggers like adenovirus.