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2015 ; 54
(6
): 1025-32
Nephropedia Template TP
gab.com Text
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English Wikipedia
Necrotizing and crescentic glomerulonephritis presenting with preserved renal
function in patients with underlying multisystem autoimmune disease: a
retrospective case series
#MMPMID25431483
McAdoo SP
; Tanna A
; Randone O
; Tam FW
; Tarzi RM
; Levy JB
; Griffith M
; Lightstone L
; Cook HT
; Cairns T
; Pusey CD
Rheumatology (Oxford)
2015[Jun]; 54
(6
): 1025-32
PMID25431483
show ga
OBJECTIVE: Necrotizing and crescentic GN usually presents with rapidly declining
renal function, often in association with multisystem autoimmune disease, with a
poor outcome if left untreated. We aimed to describe the features of patients who
have presented with these histopathological findings but minimal disturbance of
renal function. METHODS: We conducted a retrospective review (1995-2011) of all
adult patients with native renal biopsy-proven necrotizing or crescentic GN and
normal serum creatinine (<120 ?mol/l) at our centre. RESULTS: Thirty-eight
patients were identified. The median creatinine at presentation was 84 ?mol/l and
the median proportion of glomeruli affected by necrosis or crescents was 32%.
Clinicopathological diagnoses were ANCA-associated GN (74%), LN (18%), anti-GBM
disease (5%) and HScP (3%). Only 18% of cases had pre-existing diagnoses of
underlying multisystem autoimmune disease, although the majority (89%) had
extra-renal manifestations accompanying the renal diagnosis. All patients
received immunosuppression and most had good long-term renal outcomes (median
duration of follow-up 50 months), although two progressed to end-stage renal
disease within 3 years. We estimate that renal biopsy had an important influence
on treatment decisions in 82% of cases. CONCLUSION: Necrotizing and crescentic GN
may present in patients with no or only minor disturbance of renal function. This
often occurs in patients with underlying systemic autoimmune disease; early
referral for biopsy may affect management and improve long-term outcomes in these
cases.