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Distinct characteristics and outcomes in elderly-onset IgA vasculitis
(Henoch-Schönlein purpura) with nephritis: Nationwide cohort study of data from
the Japan Renal Biopsy Registry (J-RBR)
#MMPMID29738576
Komatsu H
; Fujimoto S
; Maruyama S
; Mukoyama M
; Sugiyama H
; Tsuruya K
; Sato H
; Soma J
; Yano J
; Itano S
; Nishino T
; Sato T
; Narita I
; Yokoyama H
PLoS One
2018[]; 13
(5
): e0196955
PMID29738576
show ga
BACKGROUND: The clinical presentation and prognosis of adult and elderly patients
with IgA vasculitis (Henoch-Schönlein purpura) accompanied by nephritis (IgAV-N)
have not been investigated in detail. We therefore surveyed the features and
outcomes of IgAV-N based on nationwide data derived from the Japan Renal Biopsy
Registry (J-RBR). METHODS: This multi-center cohort study compared the
clinicopathological parameters at diagnosis, initial therapies and outcomes
between 106 adult (age 19-64 years) and 46 elderly (?65 years) patients with
IgAV-N who were registered in the J-RBR between 2007 and 2012. The primary
end-points comprised a 50% increase in serum creatinine (sCr) values or end-stage
kidney disease. Factors affecting a decrease in renal function were assessed
using Cox proportional hazards models. RESULTS: Rates of hypertension, impaired
renal function, hypoalbuminemia and crescentic glomerulonephritis were
significantly higher among the elderly, than the adult patients. About 80% and
60% of the patients in both groups were respectively treated with corticosteroid
and a renin-angiotensin system (RAS) blockade. Both groups had favorable renal
survival rates for nine years (93.6% and 91.4% of the adult and elderly patients,
respectively). Significantly more elderly than adult patients developed a 50%
increase in sCr during a mean observation period of 3.9 years (21.7% vs. 4.7%, p
= 0.012), and significantly fewer elderly, than adult patients achieved clinical
remission (23.9% vs. 46.2%, p = 0.016). Multivariate analysis selected advanced
age (?65 years) and lower serum albumin values as independent prognostic factors
for a decline in renal function, whereas steroid pulse therapy helped to preserve
renal function. CONCLUSIONS: The renal prognosis of adult and elderly patients
with IgAV-N was favorable when treated aggressively with corticosteroid and RAS
blockade. However, the course of renal function should be carefully monitored in
patients aged over 65 years and those with hypoalbuminemia.
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