Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose: Adult-onset Still’s disease (AOSD) is frequently refractory to standard therapy. Anakinra (ANK), an interleukin-1 (IL-1) receptor antagonist, has demonstrated efficacy in single cases or in small series of AOSD. We assessed the efficacy of ANK in a large series of AOSD patients.
Methods: Multicenter retrospective open-label study of 41 patients with AOSD from 19 hospitals. ANK was used due to lack of efficacy to standard synthetic immunosuppressive drugs and some cases also due to lack of adequate response to at least 1 biologic agent.
Results: 41 Patients (26 women/15 men) had a mean age of 34.4±14 years and a median [interquartile range- IQR] AOSD duration of 2.2 [1-24] years before ANK onset.
Besides oral steroids, patients had previously received the following drugs: Methotrexate (32 patients), Leflunomide (7), Etanercept (10), Infliximab (9) and Adalimumab (6). ANK standard dose was 100 mg/sc/day.
At ANK onset, the most frequent clinical manifestations were joint manifestations (n=36), fever (n=32) and cutaneous manifestations (n=24). Abnormality of laboratory parameters was generally observed at that time: high C reactive protein (CRP) (n=37), high erythrocyte sedimentation rate (ESR) (n=32), leukocytosis (n=27) or anemia (n=23). ANK yielded rapid and maintained clinical and laboratory improvement (TABLE). After one year of ANK therapy, joint manifestations had decreased from 87.8% to 29.6%, fever from 78% to 7.4%, cutaneous manifestations from 58.5% to 3.7%, anemia from 56.1% to 0%, hepatomegaly and/or splenomegaly from 31.7% to 3.7% and lymphadenopathy from 26.8% to 0%. Also, a dramatic reduction of laboratory markers of inflammation including CRP, ESR and ferritin was achieved. The median [IQR] dose of prednisone was also reduced from 20 [0-100] mg/day at ANK onset to 2.5 [0-40] at 12 months. After a median [IQR] follow-up of 15.5 [1-206] months, the most important side effects were cutaneous rash (n=8), mild leukopenia (n=3), myopathy with elevation of muscle enzymes (n=1), respiratory infection by Pseudomonas Aeruginosa and gluteal abscess (n=1), herpes zoster (n=1), phalanx osteomyelitis (n=1) and urinary tract infection (n=2).
Conclusion: ANK is associated with rapid and maintained clinical and laboratory improvement, even in cases that are refractory to other biologic agents. However, joint manifestations seem to be more refractory than systemic manifestations.
TABLE
|
Baseline N=41 |
Month 1 N=41 |
Month 3 N=37 |
Month 6 N=32 |
Month 12 N=27 |
|
|
Patients with joint manifestations, %
|
87.8 |
48.7 |
34.1 |
28.1 |
29.6 |
|
Patients with fever, %
|
78 |
17.1 |
10.8 |
0 |
7.4 |
|
Patients with cutaneous manifestations , %
|
58.5
|
9.8
|
10.8
|
0
|
3.7
|
|
Leukocytosis/mm3, mean ± StD
|
15120.7±7752.3 |
8100.5±3655.1 |
7807.6±3001.7 |
7843.9±2297 |
7842.9±2702.6 |
|
ESR (mm/1st hour), median [IQR]
|
60.5 [1-137] |
16.5 [1-95] |
16 [1-120] |
8 [1-75] |
6 [1-104] |
|
CRP (mg/dL), median [IQR]
|
8.9 [0.1-37.7]
|
1.1 [0-14] |
0.6 [0-31.5] |
0.5 [0-17] |
0.3 [0-22] |
|
Prednisone dosage, median [IQR]
|
20 [0-100] |
15 [0-100] |
8.75 [0-60] |
5 [0-15] |
2.5 [0-40] |
Disclosure:
L. Riancho-Zarrabeitia,
None;
R. Blanco,
None;
A. Olivé,
None;
A. Riveros-Frutos,
None;
S. Castañeda,
None;
M. L. Velloso Feijoo,
None;
J. Narváez,
None;
I. Jiménez-Moleón,
None;
O. Maiz-Alonso,
None;
M. C. Ordóñez,
None;
J. A. Bernal,
None;
M. V. Hernández,
None;
A. Sifuentes Giraldo,
None;
C. Gomez Arango,
None;
E. Galindez-Agirregoikoa,
None;
V. Ortiz-Santamaría,
None;
J. del Blanco,
None;
J. R. De Dios,
None;
M. Moreno,
None;
J. Fiter,
None;
M. de los Riscos,
None;
P. Carreira,
None;
M. J. Rodríguez Valls Sr.,
None;
F. Ortiz-Sanjuán,
None;
T. Pina Murcia,
None;
M. Santos-Gómez,
None;
M. A. González-Gay,
None.
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