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Hypocomplementemia is associated with worse renal survival in ANCA-positive
granulomatosis with polyangiitis and microscopic polyangiitis
#MMPMID29621352
Deshayes S
; Aouba A
; Khoy K
; Mariotte D
; Lobbedez T
; Martin Silva N
PLoS One
2018[]; 13
(4
): e0195680
PMID29621352
show ga
Recent data suggest the existence of a complement alternative pathway activation
in the pathogenesis of antineutrophilic cytoplasmic antibody (ANCA)-associated
vasculitis (AAV), a condition that remains poorly understood. This study aims to
assess the clinical characteristics and outcomes of granulomatosis with
polyangiitis (GPA) and microscopic polyangiitis (MPA) patients with regard to
their plasma complement levels at diagnosis. A retrospective monocentric study
carried out at Caen University Hospital led to the identification of
proteinase-3- or myeloperoxidase-ANCA-positive GPA and MPA patients from January
2000 to June 2016 and from September 2011 to June 2016, respectively. All
patients with available C3 and C4 levels at diagnosis were included. Patients
were categorized in the hypocomplementemia group if their C3 and/or C4 levels at
diagnosis were below the lower limit of the normal range. Among the 76 AAV
patients (43 GPA, 33 MPA), 4 (5%) had hypocomplementemia, and the 72 remaining
patients exhibited normal plasma complement levels. All 4 hypocomplementemia
patients had renal involvement. Hypocomplementemia was followed in 1 patient
whose post-treatment complement level normalized within 1 month. Among all
clinical and ANCA specificity, including relapse-free survival (p = 0.093), only
overall and renal survival rates were significantly lower in the
hypocomplementemia group (p = 0.0011 and p<0.001, respectively).
Hypocomplementemia with low C3 and/or C4 levels at GPA or MPA diagnosis may be
responsible for worse survival and renal prognosis. These results argue for
larger and prospective studies to better determine the epidemiology of the
disease and to assess complement-targeting therapy in these patients.
|Aged
[MESH]
|Autoantibodies/metabolism
[MESH]
|Biomarkers/blood
[MESH]
|Complement C3/*metabolism
[MESH]
|Complement C4/*metabolism
[MESH]
|Female
[MESH]
|Granulomatosis with Polyangiitis/complications/*physiopathology/therapy
[MESH]
free
free
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