Physician practices for withdrawal of medications in inactive systemic juvenile arthritis, Childhood Arthritis and Rheumatology Research Alliance (CARRA) survey

Abstract

Background We describe a Childhood Arthritis and Rheumatology Research Alliance (CARRA) survey of North American pediatric rheumatologists that assesses physician attitudes on withdrawal of medications in systemic juvenile idiopathic arthritis (SJIA). Methods A REDCap anonymous electronic survey was distributed to 100 random CARRA JIA workgroup physician-voting members. The survey had three broad sections including: A) demographic information; B) physicians’ opinions on clinical inactive disease (CID) in SJIA and C) existing practices for withdrawing medications in SJIA. Results The survey had an 86% response rate. 88 and 93% of participants agreed with the current criteria for CID and clinical remission on medications (CRM) respectively. 78% thought it necessary to meet CRM before tapering medications except steroids. 76% use CARRA SJIA consensus treatment plans always or the majority of the time. All participants weaned steroids first in SJIA patients on combination therapy, 47% waited > 6 months before tapering additional medications. 35% each tapered methotrexate over > 6 months and 2–6 months; however, 39% preferred tapering anakinra, canakinumab and tocilizumab more quickly over 2–6 months and favored spacing the dosing interval for canakinumab and tocilizumab. When patients are on combination therapy with methotrexate and biologics, 58% preferred tapering methotrexate first while others considered patient/family preference and adverse effects to guide their choice. Conclusion Most CARRA members surveyed use published consensus treatment plans for SJIA and agree with validated definitions of CID and CRM. There was agreement with tapering steroids first in SJIA. There was considerable variability with tapering decisions of all other medications. Electronic supplementary material The online version of this article (10.1186/s12969-019-0342-5) contains supplementary material, which is available to authorized users.

Current Review of Systemic Juvenile Idiopathic Arthritis: What Do Paediatricians Need to Know?

“…A recent survey was conducted and showed that most rheumatologist participants would agree with Wallace criteria as the matrix to define clinically inactive disease in sJIA. Tapering medications should be decided on the basis of patients reaching clinical remission on medication (CRM), defined as 3 to 6 consecutive months or more of inactive disease while on medication [98]. However, a few rheumatologists understandably would like a more stringent approach, with 1 year of clinical remission while pateints are on medication [98].…”

“…Tapering medications should be decided on the basis of patients reaching clinical remission on medication (CRM), defined as 3 to 6 consecutive months or more of inactive disease while on medication [98]. However, a few rheumatologists understandably would like a more stringent approach, with 1 year of clinical remission while pateints are on medication [98]. It is advisable to taper steroids early and first because of their side effects within the first 6 months, even before patients reach full CRM [34] [98].…”

“…However, a few rheumatologists understandably would like a more stringent approach, with 1 year of clinical remission while pateints are on medication [98]. It is advisable to taper steroids early and first because of their side effects within the first 6 months, even before patients reach full CRM [34] [98].…”

“…How should patients be weaned off the medications used to treat sJIA?  Steroids should be tapered gradually from a high dose to lower doses very early, within 1 -3 weeks, if systemic inflammatory symptoms have subsided [98]. Then, weaning off of a steroid gradually should be planned within the first 6 months [34].…”

“…Then, weaning off of a steroid gradually should be planned within the first 6 months [34].  Past failure to taper, intolerance or toxicity of medications, previous flares and MAS were important factors in surveyed rheumatologists' decisions on when to taper medications [98].  Weaning patients off of anakinra is usually accomplished by using an alternate-day regimen, which means using it every other day for a month and then stopping its use if the child continues to be in remission clinically and biochemically [5] [21].…”