Biological treatment in resistant adult-onset Still’s disease: A single-center, retrospective cohort study

Abstract

Objectives: The aim of this study was to assess the demographic and clinical characteristics of patients with adult-onset Still’s disease (AOSD) under biological treatment. Patients and methods: This retrospective cohort study included a total of 19 AOSD patients (13 males, 6 females; median age: 37 years; range, 28 to 52 years) who received biological drugs due to refractory disease between January 2008 and January 2020. The data of the patients were obtained from the patient files. The response to the treatment was evaluated based on clinical and laboratory assessments at third and sixth follow-up visits. Results: Interleukin (IL)-1 inhibitor was prescribed for 13 (68.4%) patients and IL-6 inhibitor prescribed for six (31.6%) patients. Seventeen (89.5%) patients experienced clinical remission. Conclusion: Biological drugs seem to be effective for AOSD patients who are resistant to conventional therapies. Due to the administration methods and the high costs of these drugs, however, tapering the treatment should be considered, after remission is achieved.

Systematic Review and Metaanalysis of Pharmacological Interventions in Adult-Onset Still Disease and the Role of Biologic Disease-Modifying Antirheumatic Drugs

“…42 The 44 studies were conducted across a range of countries across Europe and Asia, and in the USA. 4,26,27,33, Seventeen 26,33,43,44,46,47,49,52,57,59,62,63,66,69,[71][72][73] of the 44 studies reported that included patients had to be refractory to conventional treatment including CS and csDMARDs. In other studies, patients had undergone previous treatment, but failure on all conventional management was not an inclusion criterion.…”

“…For the case series, 23 were judged to be at a low ROB 4,42-46,48-50,52,53,55-57,59,61-63,65,66,68-70 and 17 at a high ROB. 26,27,47,51,54,67,[71][72][73][74][75][76][77][78][79][80][81] Reporting was often not clear or complete. Ten of the case series showed high ROB in 1 or 2 fields, most commonly relating to poor or nonexistent reporting of patient baseline characteristics or outcomes.…”

“…Ten of the case series showed high ROB in 1 or 2 fields, most commonly relating to poor or nonexistent reporting of patient baseline characteristics or outcomes. 51,69,71,72,[74][75][76][77]79,81 This was often due to the case series being reported in abstracts rather than full papers, with 7 abstracts 47,72,[75][76][77][78]80 flagged as having a high ROB.…”

“…Complete intervention-free remission. Data on the proportion of patients experiencing complete intervention-free remission were available for TCZ (6 studies including 97 patients 50,55,57,62,71,74 ), ANK (4 studies including 208 patients 42,53,62,71 ), and CNK (1 study of 11 patients 76 ; Table 2). A large range of results was observed.…”

“…A large range of results was observed. Nonetheless, between 14% and 73% of patients (across 6 studies 4,55,57,62,71,74 ) achieved complete intervention-free remission with TCZ. The 4 ANK studies 42,53,62,71 reported between 2% and 39% of patients with intervention-free remission, whereas the single CNK study 76 reported 9%.…”