PMID- 8364753
OWN - NLM
STAT- MEDLINE
DCOM- 19931005
LR  - 20061115
IS  - 0001-4079 (Print)
IS  - 0001-4079 (Linking)
VI  - 177
IP  - 3
DP  - 1993 Mar
TI  - ["Idiopathic" extracapillary glomerulonephritides without immune deposits are 
      vascularitides: clinical and serologic analysis].
PG  - 481-94; discussion 494-8
AB  - To determine the spectrum of systemic diseases potentially associated with 
      pauci-immune rapidly progressive glomerulonephritis (GN), most of which being 
      considered as idiopathic, we have analyzed extra-renal manifestations, occurrence 
      of extra-glomerular vasculitis and incidence and specificity of ANCAs in 40 
      patients selected only on histological criteria. Extra-renal symptoms were 
      unexpectedly observed in all patients but one, and were suggestive of vasculitis 
      in 24 of them. Extra-glomerular vasculitis was evidenced in 18 kidney biopsies 
      and four biopsies from other organs. Among the 33 patients with suspected or 
      established vasculitis, 13 had presumed or biopsy-proven Wegener's granulomatosis 
      (WG), three had a macroscopic form of polyarteritis nodosa and 17 had a clinical 
      presentation compatible with the so-called microscopic polyarteritis previously 
      described in the british literature. An additional patient had clinical signs of 
      WG without clinical and histological evidence of vasculitis. ANCAs were detected 
      in 28/33 and 25/34 sera tested by IF and ELISA, respectively: 19 contained 
      anti-myeloperoxidase (MPO) antibodies and 6 had anti-proteinase 3 (Pr3) activity. 
      Anti-MPO and anti-Pr3 antibodies were present in all clinical subgroups but with 
      various incidences: anti-MPO antibodies were surprisingly more often detected 
      (6/12) than anti-Pr3 (4/12) in patients with suspected or histologically proven 
      WG but anti-Pr3 antibodies were nonetheless 3- to 4-fold more frequent in WG than 
      in non-WG systemic vasculitis (1/12) and necrotizing CGN without evidence of 
      extra-renal vasculitis (1/10). These results strongly suggest that pauci-immune 
      necrotizing CGNs belong to the broad spectrum of necrotizing vasculitides 
      affecting glomerular capillaries. This hypothesis is also supported by the good 
      response of patients to immunosuppressive treatments known for their efficacy in 
      vasculitides, whereas these treatments are usually less successful in severe 
      forms of extra-capillary GN with immune deposits.
FAU - Ronco, P
AU  - Ronco P
AD  - Service de Nephrologie, Hopital Tenon, Paris.
FAU - Mougenot, B
AU  - Mougenot B
FAU - Bindi, P
AU  - Bindi P
FAU - Noel, L H
AU  - Noel LH
FAU - Mignon, F
AU  - Mignon F
FAU - Lesavre, P
AU  - Lesavre P
LA  - fre
PT  - Comparative Study
PT  - English Abstract
PT  - Journal Article
TT  - Les glomerulonephrites extracapillaires "idiopathiques" sans depot immun sont des 
      vascularites: analyse clinique et serologique.
PL  - Netherlands
TA  - Bull Acad Natl Med
JT  - Bulletin de l'Academie nationale de medecine
JID - 7503383
SB  - IM
MH  - Glomerulonephritis/diagnosis/*immunology
MH  - Humans
MH  - Serologic Tests
MH  - Vasculitis/diagnosis/*immunology
EDAT- 1993/03/01 00:00
MHDA- 1993/03/01 00:01
CRDT- 1993/03/01 00:00
PHST- 1993/03/01 00:00 [pubmed]
PHST- 1993/03/01 00:01 [medline]
PHST- 1993/03/01 00:00 [entrez]
PST - ppublish
SO  - Bull Acad Natl Med. 1993 Mar;177(3):481-94; discussion 494-8.