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

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-labe...

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Published in:Medicine (Baltimore) 2015-09, Vol.94 (39), p.e1554-e1554
Main Authors: Ortiz-Sanjuán, Francisco, Blanco, Ricardo, Riancho-Zarrabeitia, Leyre, Castañeda, Santos, Olivé, Alejandro, Riveros, Anne, Velloso-Feijoo, María L, Narváez, Javier, Jiménez-Moleón, Inmaculada, Maiz-Alonso, Olga, Ordóñez, Carmen, Bernal, José A, Hernández, María V, Sifuentes-Giraldo, Walter A, Gómez-Arango, Catalina, Galíndez-Agirregoikoa, Eva, Blanco-Madrigal, Juan, Ortiz-Santamaria, Vera, Del Blanco-Barnusell, Jordi, De Dios, Juan R, Moreno, Mireia, Fiter, Jordi, Riscos, Marina de Los, Carreira, Patricia, Rodriguez-Valls, María J, González-Vela, M Carmen, Calvo-Río, Vanesa, Loricera, Javier, Palmou-Fontana, Natalia, Pina, Trinitario, Llorca, Javier, González-Gay, Miguel A
Format: Article
Language:English
Subjects:
Adult
Case studies
Drug Therapy, Combination
Drugs
Estudi de casos
Female
Humans
Immunosuppressive Agents - administration & dosage
Immunosuppressive Agents - therapeutic use
Interleukin 1 Receptor Antagonist Protein - administration & dosage
Interleukin 1 Receptor Antagonist Protein - therapeutic use
Malalties rares
Male
Medicaments
Middle Aged
Observational Study
Prednisone - administration & dosage
Rare diseases
Retrospective Studies
Reumatologia
Rheumatology
Still's Disease, Adult-Onset - drug therapy
Therapeutic use
Ús terapèutic
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Summary: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.
ISSN:0025-7974
1536-5964
DOI:10.1097/MD.0000000000001554