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3. Shimizu J, Oka H, Yamano Y, Yudoh K, Suzuki N. Cardiac
involvement in relapsing polychondritis in Japan. Rheumatology
(Oxford) 2016;55:583–4.
4. Shimizu J, Oka H, Yamano Y, Yudoh K, Suzuki N. Cutaneous
manifestations of patients with relapsing polychondritis: an associa-
tion with extracutaneous complications. Clin Rheumatol 2016;
35:781–3.
5. Dion J, Costedoat-Chalumeau N, S
ene D, Cohen-Bittan J, Leroux
G, Dion C, et al. Relapsing polychondritis can be characterized by
three different clinical phenotypes: analysis of a recent series of
142 patients. Arthritis Rheumatol 2016;68:2992–3001.
DOI 10.1002/art.40329
Reply
To the Editor:
Shimizu et al report that, using correlation matrix
analysis in a study of 239 Japanese patients with RP, they found
that airway involvement (laryngeal and tracheobronchial) was
strongly associated with nasal chondritis whereas there was an
inverse relationship between airway involvement and external
ear chondritis, suggesting that nasal and auricular chondritis
define 2 subgroups of patients with RP. Our study included 142
patients from a single specialty center. Using multiple corre-
spondence analysis followed by cluster analysis, we identified 3
RP subgroups: patients with MDS, patients with tracheo-
bronchial involvement, and patients with none of these features
(who had a milder form of the disease).
We have now conducted an additional analysis of our
data and, in keeping with the results described by Shimizu
et al, found a negative correlation between tracheobronchial
and auricular involvements (r =0.245, P=0.003). However,
this correlation was not significant for airway involvement
overall. We did not find any correlation between nasal and
tracheobronchial chondritis (r =0.08, P=0.34).
There could be several explanations for the partial
discrepancy between the findings demonstrated in our addi-
tional analysis and those in Shimizu and colleagues’research.
First, patient characteristics were quite different between the
2 studies (Table 1). In the Japanese study, auricular chondritis
was absent in 22% of patients. MDS, a very specific feature
of RP within connective tissue disorders, was rare although
the mean age at RP onset was approximately 9 years older
than in our study. Alternatively, tracheobronchial involvement
was more frequent and severe in the Japanese study and
renal manifestations were present in 6.7% of the patients
(Oka H, Yamano Y, Shimizu J, Yudoh K, Suzuki N. A large-
scale survey of patients with relapsing polychondritis in Japan.
Inflamm Regen 2014;34:149–56).
In RP patients described in the literature, renal
involvement usually consists of necrotizing glomerulonephri-
tis, therefore raising the question of granulomatosis with
polyangiitis (Wegener’s), as we have emphasized. In this set-
ting, it would be interesting to know if the patients from the
2009 Japanese study were tested for anti–proteinase 3 anti-
bodies and if they underwent renal biopsy.
Interestingly, the association between nasal and respi-
ratory involvement could have a pathophysiologic explanation.
In a rat model of RP induced by immunization against matri-
lin-1, animals developed severe respiratory involvement with
nasal chondritis but did not develop either auricular chondritis
or arthritis (Hansson AS, Heineg
ard D, Holmdahl R. A new
animal model for relapsing polychondritis, induced by cartilage
matrix protein [matrilin-1]. J Clin Invest 1999;104:589–98).
In conclusion, the description of distinct subgroups of
RP with different prognosis should be taken into account for
future randomized therapeutic trials, which have never been
carried out to date in this rare, but not too uncommon, disease.
J
er
emie Dion, MD
Nathalie Costedoat-Chalumeau, MD, PhD
Universit
e Ren
e Descartes Paris V
H^
opital Cochin
Jean-Charles Piette, MD
Universit
e Pierre et Marie Curie Paris VI
Paris, France
DOI 10.1002/art.40335
Does the revised definition of eosinophilic
granulomatosis with polyangiitis (Churg-Strauss)
indicate the need for a new treatment? Comment on
the article by Pu
echal et al
To the Editor:
The recently published CHUSPAN 2 study addresses
the important issue of the optimum treatment for nonsevere
systemic necrotizing vasculitis (SNV) that poses no immediate
threat to life or essential-organ function (1). That study, con-
ducted by the French Vasculitis Study Group, contributed
much to our understanding of systemic vasculitis. All
patients with SNVs have a poor prognosis and require
prompt immunosuppressive therapy. Nevertheless, it is clear
today that less aggressive and less toxic immunosuppressive
regimens (i.e., intravenous instead of oral cyclophosphamide
for induction of remission, azathioprine (AZA) with
Table 1. Comparison of patient characteristics between 2 recent
series of patients with relapsing polychondritis*
Dion et al,
France
(n =142)
Oka et al,
Japan
(n =239)
Female sex 61 46
Age at disease onset, mean years 43.5 52.7
Auricular chondritis 89 78
Nasal chondritis 63 39
Arthritis 33†39
Laryngeal involvement 43 20
Tracheobronchial involvement 22 41
Audiovestibular involvement 34 27
Ocular manifestations 56 46
Cutaneous manifestations 28 13
Myelodysplasia 8 1.7
Neurologic manifestations 11 9.6
Cardiac manifestations 27 7.1
Renal manifestations 0 6.7
* Except where indicated otherwise, values are the percent of
patients.
†Sixty-six percent had arthralgia.
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