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Polyarteritis nodosa when applying the Chapel Hill nomenclature--a descriptive study on ten patients.

Selga, Daina LU ; Mohammad, Aladdin LU ; Sturfelt, Gunnar LU and Segelmark, Mårten LU (2006) In Rheumatology 45(10). p.1276-1281
Abstract
Objectives. Polyarteritis nodosa (PAN) is a term that has been used to describe a wide variety of vasculitic conditions. In 1994, the Chapel Hill Consensus Conference on the nomenclature of systemic vasculitides proposed that the name classical PAN should be restricted to diseases where there is arteritis in small and medium-sized arteries without the involvement of smaller vessels. Our aim was to describe the symptoms and course of disease in PAN when the microscopic forms are excluded. Methods. All patients with a diagnosis of PAN treated in our departments during the period 1990-2002 were eligible for this study. The diagnosis had to be confirmed by biopsy, angiography or electromyography. Results. Ten patients were eligible for the... (More)
Objectives. Polyarteritis nodosa (PAN) is a term that has been used to describe a wide variety of vasculitic conditions. In 1994, the Chapel Hill Consensus Conference on the nomenclature of systemic vasculitides proposed that the name classical PAN should be restricted to diseases where there is arteritis in small and medium-sized arteries without the involvement of smaller vessels. Our aim was to describe the symptoms and course of disease in PAN when the microscopic forms are excluded. Methods. All patients with a diagnosis of PAN treated in our departments during the period 1990-2002 were eligible for this study. The diagnosis had to be confirmed by biopsy, angiography or electromyography. Results. Ten patients were eligible for the study. The median age was 46 yrs. Renal involvement was seen in 70% at diagnosis. After 5 yrs, 57% had experienced a relapse, which is equivalent to the relapse rate seen in microscopic polyangiitis. Organ damage was assessed by the Vasculitis Damage Index (VDI) and after 5 yrs cardiovascular and neuropsychiatric damage dominated, followed by renal damage. During the follow-up, two patients developed end-stage renal disease. The annual incidence of PAN in our local catchment area was estimated to 1.6 per million and year. Conclusions. When applying the Chapel Hill nomenclature, PAN is a rare but severe disease with a high incidence of renal involvement and frequent relapses. (Less)
Please use this url to cite or link to this publication:
author
; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
outcome, vasculitis, polyarteritis nodosa, PAN, incidence, relapses
in
Rheumatology
volume
45
issue
10
pages
1276 - 1281
publisher
Oxford University Press
external identifiers
  • wos:000240927100017
  • scopus:33749636518
  • pmid:16595516
ISSN
1462-0332
DOI
10.1093/rheumatology/kel091
language
English
LU publication?
yes
id
7e911dc1-aebb-4830-bc82-88ef6b45644b (old id 156037)
alternative location
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16595516&dopt=Abstract
date added to LUP
2016-04-01 17:07:58
date last changed
2021-09-22 02:54:57
@article{7e911dc1-aebb-4830-bc82-88ef6b45644b,
  abstract     = {Objectives. Polyarteritis nodosa (PAN) is a term that has been used to describe a wide variety of vasculitic conditions. In 1994, the Chapel Hill Consensus Conference on the nomenclature of systemic vasculitides proposed that the name classical PAN should be restricted to diseases where there is arteritis in small and medium-sized arteries without the involvement of smaller vessels. Our aim was to describe the symptoms and course of disease in PAN when the microscopic forms are excluded. Methods. All patients with a diagnosis of PAN treated in our departments during the period 1990-2002 were eligible for this study. The diagnosis had to be confirmed by biopsy, angiography or electromyography. Results. Ten patients were eligible for the study. The median age was 46 yrs. Renal involvement was seen in 70% at diagnosis. After 5 yrs, 57% had experienced a relapse, which is equivalent to the relapse rate seen in microscopic polyangiitis. Organ damage was assessed by the Vasculitis Damage Index (VDI) and after 5 yrs cardiovascular and neuropsychiatric damage dominated, followed by renal damage. During the follow-up, two patients developed end-stage renal disease. The annual incidence of PAN in our local catchment area was estimated to 1.6 per million and year. Conclusions. When applying the Chapel Hill nomenclature, PAN is a rare but severe disease with a high incidence of renal involvement and frequent relapses.},
  author       = {Selga, Daina and Mohammad, Aladdin and Sturfelt, Gunnar and Segelmark, Mårten},
  issn         = {1462-0332},
  language     = {eng},
  number       = {10},
  pages        = {1276--1281},
  publisher    = {Oxford University Press},
  series       = {Rheumatology},
  title        = {Polyarteritis nodosa when applying the Chapel Hill nomenclature--a descriptive study on ten patients.},
  url          = {http://dx.doi.org/10.1093/rheumatology/kel091},
  doi          = {10.1093/rheumatology/kel091},
  volume       = {45},
  year         = {2006},
}