Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose We previously described clinical characteristics and evolution of 142 patients with relapsing polychondritis (RP) followed in a single center and seen at least once since 2000 (1). A cluster analysis was performed with the aim to identify different subtypes of RP.
Methods A cluster analysis using a k-mean clustering method preceded by a multiple correspondence analysis was performed on 142 patients with RP according to Michet’s criteria.
Results We identified 3 clusters corresponding to 3 distinct clinical phenotypes (Table 1). Cluster 1 (n=12, 8%) corresponded to the more severe phenotype, with a mortality rate of 58% and intensive care unit (ICU) admission rate of 50%. This cluster mainly included men (83%), older at diagnosis, with myelodysplasia (83%), cutaneous (92%) and cardiac (58%) involvement, but with rare tracheobronchial involvement. They were more frequently treated with biologics (58%) than with immunosuppressive agents (33%).
Cluster 2 (n=37, 26%) was characterised by patients with predominant tracheobronchial (76%) involvement and abnormal functional respiratory test results (57%). None had myelodysplasia, and cardiac involvement was less frequent (24%). The prognosis was intermediate: mortality was 14%, but these patients with high infection rate (35%) were frequently admitted to ICU (27%). They frequently received immunosuppressive agents (84%).
Cluster 3 (n=93, 65%), the largest and less severe, was mainly composed of women (68%) with infrequent tracheobronchial (3%) and hematological involvement (2% had myelodysplasia and 5% another hematological disease). Few patients died (4%) or were admitted to ICU (2%). All patients with long-lasting remission (n=15) were in this group.
Conclusion Using cluster analysis, we were able to distinguish three distinct subgroups of RP. Cluster 1 and 2 had the worst prognosis: older men with myelodysplasia were more likely to have a fatal issue and patients with a respiratory tract involvement were more likely to be admitted to intensive care and had an intermediate survival. By contrast, the last group, mainly composed of patients without hematological or respiratory involvement, had a good prognosis. These results need to be confirm in further studies.
1. Dion J, Costedoat-Chalumeau N, Sène D, Piette JC. Description of 142 cases of relapsing polychondritis followed in a single center since 2000 (abstract). ArthritisRheum 2013;10(supplement):S868.
Table 1: Cluster analysis of 142 patients with relapsing polychondritis
|
|
Overall series N=142 |
Cluster 1 n=12 (8%) |
Cluster 2 n=37 (26%) |
Cluster 3 n=93 (65%) |
p value |
|
Demographical data |
|
|
|
|
|
|
Women (%) |
86 (61) |
2 (17) |
21 (57) |
63 (68) |
0.003 |
|
Men |
56 (39) |
10 (83) |
16 (43) |
30 (32) |
|
|
Age at onset |
|
|
|
|
0.02 |
|
≤55 years |
109 (77) |
5 (42) |
33 (89) |
75 (81) |
|
|
>55 years |
33 (23) |
7 (58) |
4 (11) |
18 (19) |
|
|
Clinical phenotype |
|
|
|
|
|
|
Laryngeal |
61 (43) |
2 (17) |
25 (68) |
34 (37) |
0.001 |
|
Tracheobronchial |
32 (22) |
1 (8) |
28 (76) |
3 (3) |
<0.0001 |
|
Audiovestibular |
48 (34) |
6 (50) |
15 (41) |
27 (29) |
0.21 |
|
Ophthalmological |
80 (56) |
11 (92) |
18 (49) |
51 (55) |
0.029 |
|
Cutaneous |
40 (29) |
11 (92) |
6 (16) |
23 (25) |
<0.0001 |
|
Deep vein thrombosis |
15 (11) |
3 (25) |
6 (16) |
6 (6) |
0.06 |
|
Myelodysplasia |
12 (8) |
10 (83) |
0 (0) |
2 (2) |
<0.0001 |
|
Other hematological disease |
6 (4) |
0 (0) |
1 (3) |
5 (5) |
0.59 |
|
Cardiac |
38 (27) |
7 (58) |
9 (24) |
22 (24) |
0.03 |
|
Abnormal functional respiratory test result |
29 (20) |
2 (17) |
21 (57) |
6 (6) |
<0.001 |
|
Treatments |
|
|
|
|
|
|
Steroids |
133 (94) |
12 (100) |
37 (100) |
84 (90) |
0.08 |
|
Biological agents |
22 (15) |
7 (58) |
12 (32) |
3 (3) |
<0.0001 |
|
Immunosuppressive agents |
56 (39) |
4 (33) |
31 (84) |
21 (23) |
<0.0001 |
|
Disease evolution |
|
|
|
|
|
|
Death |
16 (11) |
7 (58) |
5 (14) |
4 (4) |
<0.0001 |
|
Serious infection |
26 (18) |
7 (58) |
13 (35) |
6 (6) |
<0.0001 |
|
ICU admission |
18 (13) |
6 (50) |
10 (27) |
2 (2) |
<0.0001 |
|
Long lasting remission |
15 (11) |
0 (0) |
0 (0) |
15 (16) |
0.02 |
Disclosure:
J. Dion,
None;
N. Costedoat-Chalumeau,
None;
D. Sène,
None;
J. Cohen-Bittan,
None;
G. Leroux,
None;
C. Dion,
None;
C. Francès,
None;
J. C. Piette,
None.
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