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But even still outcomes are not great.

🏥In the Columbia series, the treatment group had similar dialysis outcomes as those not treated. About 1/3 with Hz-vasculitis stay on dialysis.

This is in line with previous cohorts.
PMIDs 31497427, 29657868, 1310098, 18676500

Understanding who is at risk and clinical sx are important when considering outcomes.

🏥The Columbia series suggest treatment with immunosuppression results in similar outcomes to ANCA vasculitis, but few received additional cytotoxic therapy.

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The most common Caucasian haplotype DR4 carries a null allele for C4B and they are located in close proximity to HLA-B and HLA-DR.

The similar rates of Hz-lupus in those with HLA-DR4 and null C4 linkage disequilibrium may be important in the pathogenesis

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But others feel perhaps 🧬 HLA is more important regarding risk than acetylator phenotype.

Of patients with Hz-lupus, the RR of Hz-lupus is 5.58 if HLA-DR4 is present at 12 months
-compared to control (RR5.58)
-compared to slow acetylators (RR8.14)

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🧬 Genetics may also play a role to determining who is at risk.

Hz is made inactive through acetylation. There are three acetylator phenotypes: slow, intermediate, fast

🐢 Slow acetylators appear to be at higher risk of Hz-lupus

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81% with Hz-vasculitis will have renal involvement, whereas only 13% of Hz-lupus will involve the kidneys. But there can be features of both on pathology.

The rate of renal involvement is not true for all drug induced vasculitis.

PMIDs 26092827, 20107567, 19424772, 19424772

Patients with Hz-Lupus had an incidence of 5-10% (older series 5-7%) with peak incident rate at 📆 3 years of therapy.

♀️ Women on high doses are at especially high risk!

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Patients with Hz-vasculitis had a
💊mean daily dose of 142 mg
📆mean exposure time of 4.7 years.

The higher the dose, the higher the incidence.

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🔎 Another clue may be presence of ⬆️ VERY high ANCA titers

Of cases of high MPO ANCA titers
- 33% had Hz exposure

A series of 12 patients with Hz-V
- median MPO titer 1:2560
- median ANA titer 1:1280

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What other #stoneconundrums do you have?

Uric acid as a nidus for calcium stone formation is a common pearl but the data on irate lowering therapies to prevent calcium stones is sparse. @kireports #nephsky #nephtwitter #kidneystones

https://buff.ly/40IdWot