a 6-year-old boy
Immunoglobulin A nephropathy (IgAN) alongside PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, and adenitis)
He did not respond well to cimetidine, so at age 7 his doctors removed his tonsils (palatine tonsillectomy). No extra immune-suppressing drugs were needed. After the surgery his fever episodes stopped, his blood in the urine ceased, and a repeat biopsy showed the kidney tissue had improved a lot.
In one 6-year-old boy who had both PFAPA syndrome and IgA nephropathy, removing his tonsils alone stopped the fevers and the blood in his urine, and a follow-up biopsy at 11 months showed his kidney tissue had improved a lot, without any immune-suppressing drugs.
- This boy had two conditions at once, PFAPA and IgA nephropathy, which the paper says both involve immune trouble tied to the tonsils.
- His blood in the urine showed up together with his fever episodes, and a kidney biopsy confirmed IgA nephropathy.
- The medicine cimetidine did not work well for him, so at age 7 his doctors removed his palatine tonsils.
- After the surgery his fever episodes fully stopped and the blood in his urine went away, with no extra immune-suppressing drugs added.
- A repeat kidney biopsy 11 months after surgery showed clear improvement, with the earlier mesangial overgrowth gone and less endocapillary proliferation.
Why it might work
The paper's idea is that both PFAPA and IgA nephropathy in this boy came from the same source: an overactive immune reaction in the tonsils. When the tonsils are misfiring, they can drive the fever attacks and also push the immune system to make faulty IgA that lands in the kidney and inflames it. By taking the tonsils out, the doctors removed that shared trigger, so both the fevers and the kidney inflammation calmed down at the same time. The fact that the fevers stopped, the urine cleared, and the biopsy improved all together is what the authors point to as support for that one-shared-cause explanation.
The honest limits
- This is a single patient (n=1), and the paper calls it the first pediatric case of its kind, so it cannot tell us how often this works for other people.
- The abstract reports biopsy findings and symptoms but does not give any kidney function numbers like eGFR or protein in the urine, so we cannot see the full picture of kidney health.
- Follow-up was only 11 months, so we do not know if the improvement lasts for years.
- The abstract does not state whether the authors disclosed any conflicts of interest.
- My IgA nephropathy does not come with PFAPA or tonsil-related fevers, so would tonsillectomy be expected to help me at all?
- What kidney function tests, like eGFR and urine protein, would you track to judge whether any treatment is actually working for me?
- For an adult with stage 3 kidney disease, what treatments have the strongest evidence, given that this report is a single child's case?