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Efficacy of Anakinra in Refractory Adult-Onset Still's Disease: Multicenter Study of 41 Patients and Literature Review

Abstract: Adult-onset Still's disease (AOSD) is often refractory to standard therapy. Anakinra (ANK), an interleukin-1 receptor antagonist, has demonstrated efficacy in single cases and small series of AOSD. We assessed the efficacy of ANK in a series of AOSD patients. Multicenter retrospective open-label study. ANK was used due to lack of efficacy to standard synthetic immunosuppressive drugs and in some cases also to at least 1 biologic agent. Forty-one patients (26 women/15 men) were recruited. They had a mean age of 34.4 ± 14 years and a median [interquartile range (IQR)] AOSD duration of 3.5 [2-6] years before ANK onset. At that time the most common clinical features were joint manifestations 87.8%, fever 78%, and cutaneous rash 58.5%. ANK yielded rapid and maintained clinical and laboratory improvement. After 1 year of therapy, the frequency of joint and cutaneous manifestations had decreased to 41.5% and to 7.3% respectively, fever from 78% to 14.6%, anemia from 56.1% to 9.8%, and lymphadenopathy from 26.8% to 4.9%. A dramatic improvement of laboratory parameters was also achieved. The median [IQR] prednisone dose was also reduced from 20 [11.3-47.5] mg/day at ANK onset to 5 [0-10] at 12 months. After a median [IQR] follow-up of 16 [5-50] months, the most important side effects were cutaneous manifestations (n = 8), mild leukopenia (n = 3), myopathy (n = 1), and infections (n = 5). ANK is associated with rapid and maintained clinical and laboratory improvement, even in nonresponders to other biologic agents. However, joint manifestations are more refractory than the systemic manifestations.

Otras publicaciones de la misma revista o congreso con autores/as de la Universidad de Cantabria

 Fuente: Medicine (Baltimore) 2015 Sep;94(39):e1554

 Año de publicación: 2015

Nº de páginas: 8

Tipo de publicación: Artículo de Revista

 DOI: 10.1097/MD.0000000000001554

ISSN: 0025-7974,1536-5964

Autores/as

ORTÍZ SANJUÁN, F

BLANCO, R

RIANCHO ZARRABEITIA, L

CASTAÑEDA, S

OLIVÉ, A

RIVEROS, A

VELLOSO FEIJOO, ML

NARVÁEZ, J

JIMÉNEZ MOLEÓN, I

MAIZ ALONSO, O

ORDOÑEZ, C

BERNAL, JA

HERNÁNDEZ, MV

SIFUENTES GIRALDO, WA

GÓMEZ ARANGO, C

GALÍNDEZ AGIRREGOIKOA, E

BLANCO MADRIGAL, J

ORTÍZ SANTAMARÍA, V

BLANCO BARNUSELL, J DEL

DIOS, JR DE

MORENO, M

FITER, J

RISCOS, M DE LOS

CARREIRA, P

RODRÍGUEZ VALLS, MJ

GONZÁLEZ VELA, MC

CALVO RIO, V

LORICERA, J

PALMOU FONTANA, N

PINA, T