Scleroderma
and the Kidney: Not just Renal Crisis
Not all renal
failure in scleroderma is HTN related renal crisis. While renal TMA and
endothelial damage is a common cause of AKI in scleroderma, other interesting
cause to keep in mind is vasculitis.
Of all the
vasculitides, small vessel ANCA vasculitis is noted to be seen with
scleroderma.
A
study by Rho
et al. found 31 reports containing 63 cases of ANCA vasculitis with scleroderma up to 1994. Fifty of the 63 cases provided sufficient
clinical and laboratory information and were included in the analysis.
Eighty-four percent were women with a mean age of 57.1 years. Over 70% had ANA positive and 70% with
anti-Scl-70 antibody, and 72% with positive anti-MPO antibody. The most common
end-organ involvement included kidneys (82%) and lungs (70% had pulmonary
fibrosis). Mortality was highest in the first year.
A more recent review has
11 more cases that also were reported.
Most were females, all had anti MPO titers and 9 /11 cases had renal
involvement with crescentic GN.
These findings highlight the importance of considering
crescentic GN related to ANCA vasculitis as a potential cause of renal insufficiency
in scleroderma. Classically, scleroderma renal crisis occurs in up to 20% of
patients with diffuse scleroderma, and renal involvement manifesting as
hypertension, proteinuria, or azotemia can be found in 45–60%. However, causes
other than scleroderma renal crisis should be considered as a differential
diagnosis, especially in settings of normal blood pressure or ANCA positivity.
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