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Effect of classification on the incidence of polyarteritis nodosa and microscopic polyangiitisComment onthe article by Watts et al.

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Vol. 40, No. 6, June 1097, pp 1183-1 188
0 1997, American College of Rheurnatologq
Effect of classification on the incidence of polyarteritis
nodosa and microscopic polyangiitis: comment on the
article by Watts et a1
To the Editor:
We read with interest the recent article by Watts et a1
(I), which compared the American College of Rheumatology
(ACR) 1990 classification criteria for polyarteritis nodosa
(PAN) with the Chapel Hill Consensus Conference (CHCC)
definitions for classic PAN and microscopic polyangiitis
(MPA), and which also aimed to estimate the annual incidence
of PAN and MPA. One aspect not reported in the paper which
may have a significant bearing on the estimated incidence of
MPA is the clinical and pathologic features of the 27 patients
in the Norfolk (UK) registry who were classified as having
Wegener’s granulomatosis (WG) by the ACR criteria (1). As
the authors themselves point out, there can be a significant
overlap between WG and MPA, both clinically and with
respect to antineutrophil cytoplasmic antibody specificity (1).
While upper respiratory tract and otolaryngologic involvement
tends to favor a diagnosis of WG, the “nasal or oral inflammation” defined by the ACR criteria is reported in up to 20%
of patients with MPA (2). The chief difficulty, however, arises
in patients who present with a pulmonary-renal syndrome (3).
Since the ACR criteria set does not recognize MPA as a
separate entity, patients with primary vasculitis presenting in
this way are classified as having WG, by the traditional format,
even without demonstration of granulomatous inflammation
(4). As a result, it is our contention that many cases of MPA
will be found within the ACR category of W G as well as in the
PAN group.
We recently studied a smaller cohort of 24 patients
with primary systemic vasculitis (5) and found that of 15 cases
classified as W G by the ACR criteria, only 5 satisfied the
CHCC definition of WG. Five others, 3 of whom presented
with pulmonary-renal manifestations without otolaryngologic
involvement, had small vessel vasculitis and no granulomatous
inflammation. These patients best satisfied the CHCC definition for MPA (5). Therefore, while we recognize that MPA
may evolve into WG, a careful description of the cases
classified as W G in the study by Watts et a1 would still have
been warranted in order to find other cases of MPA. Since the
WG category was much larger than the combined MPA-PAN
category (27 versus 13), this could significantly affect the
estimated prevalence of MPA reported.
Since these 2 nomenclature systems do not aim to
fulfill the same purpose (3), they have different strengths and
weaknesses which need to be recognized when attempting to
draw conclusions from studies in which they are applied. Areas
of potential overlap need to be paid particular attention.
Therefore, a broader look at all of the primary cases in the
Norfolk registry would have given a different and, in our view,
more precise estimation of the relative frequencies of the
particular conditions studied.
I. N. Bruce, MD, MRCP
The Toronto Hospital
Toronto, Ontario, Canada
A. L. Bell, MD, FRCP
Musgrave Park Hospital
Royal Victoria Hospital
The Queen’s University
Belfast, UK
1. Watts RA, Jolliffe VA, Carruthers DM, Lockwood M, Scott DGI:
Effect of classification on the incidence of polyarteritis nodosa and
microscopic polyangiitis. Arthritis Rheum 39:1208-1212, 1996
2. Savage COS, Winearls CG, Evans DJ, Rees AJ, Lockwood CM:
Microscopic polyarteritis: presentation, pathology and prognosis.
QJM 56~467-483, 1986
3. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL,
Hagen C, Hoffman GS, Hunder GG, Kallenberg CGM, McCluskey
RT, Sinico RA, Rees AJ, van Es LA, Waldherr R, Wiik A:
Nomenclature of systemic vascditides: proposal of an International
Consensus Conference. Arthritis Rheum 37:187-192, 1994
4. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend
WP, Calabrese LH, Fries JF, Lie JT, Lightfoot RW Jr, Masi AT,
McShane DJ, Mills JA, Stevens MB, Wallace SL, Zvaifler NJ: The
American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 33:l 101-1 107,
5. Bruce IN, Bell AL: A comparison of two nomenclature systems for
primary systemic vasculitis. Br J Rheumatol (in press)
Childhood-onset scleroderma from a dermatologist’s
perspective: comment on the article by Vancheeswaran
et a1
To the Editor:
The article by Vancheeswaran et a1 on childhood-onset
scleroderma (1) is of great interest because it facilitates the
sharing of information on the derrnatologic and rheumatologic
experience with this disease, which is seen much more often by
dermatologists than rheumatologists. This could account for
the authors’ statement that fewer than 150 cases of juvenile
scleroderma had been reported in the world literature. In fact,
the localized variant of scleroderma is a not-infrequent disease
in dermatologic practice. In their multicenter study, Vancheeswaran and colleagues identified 11 cases of diffuse cutaneous systemic sclerosis (dcSSc), which is extremely rare in
children, 14 cases of linear scleroderma, and 17 cases of
morphea. Since at our dermatology division we have a very
large cohort of patients with localized scleroderma who have
been followed up for many years, we would like to compare the
data reported by Vancheeswaran et a1 with our own experience
in this field. In our series of 552 cases of childhood scleroderma, linear scleroderma was found to be the most frequent
variant (301 patients; 54.5%). One hundred eighteen patients
(21.4%) had plaque and disseminated morphea, 7 (1.3%) had
generalized morphea, and the other 126 had atypical forms
(subcutaneous, nodular, primary atrophic of atrophoderma
Pasini-Pierini type, etc.).
The important difference from adult localized scleroderma is the significantly higher prevalence of linear variant,
including scleroderma en coup de sabre and facial hemiatro-
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effect, incidence, polyarteritis, articles, microscopy, classification, watt, nodosa, onthe, polyangiitiscomment
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