Still’s disease treatment has been revolutionized by the use of biologic drugs capable of targeting specific inflammatory molecules. Evidence and clinical practice suggests early and aggressive treatment of Still’s disease with appropriate biologic agents may prevent the development of the core disease process and associated comorbidities.
This treatment page is directed toward patient use but may also be of significance for providers. Below are the categories of front line and commonly used therapeutics for Still’s disease.
The following information is based off U.S. FDA Approved treatments only. Drug approvals may differ in other jurisdictions.
Biologics are targeted immunology therapies that use or mimic molecules that already exist in the body. They are used to treat a wide variety of diseases, from genetic deficiencies and cancers to autoinflammatory and autoimmune diseases.
Front Line Biologics IL-1 & IL-6
Current evidence-based research and clinical practice points to IL-1 and IL-6 therapies as the most efficacious front line biologics for treating the primary underlying systemic inflammation of Still’s disease.
Not included in this list is rilonocept (Arcalyst) due to no current FDA approval for SJIA/AOSD (Still’s) or RA (common indications for approval of Still’s therapeutics).
|anakinra (Kineret)||IL-1 receptor antagonist (inhibitor)|
|canakinumab (Ilaris)||IL-1 receptor antagonist (inhibitor)|
|sarilumab (KEVZARA)||anti-IL-6 monoclonal antibody|
|tocilizumab (Actemra)||IL-6 receptor antagonist (inhibitor)|
Short-acting IL-1 inhibitor used for both Still’s and Macrophage Activation Syndrome (MAS).
Provider note: Approved FDA indications-NOMID, CAPS, (SJIA-is approved-but not listed as indication). List indication under SJIA.
Patient Assistance Contact Information: Kineret On Track
Kineret On Track Phone #: 866-547-0644
Provider Note: Kineret on Track should be your provider’s FIRST step. Download the start or referral form on website and fill out ALL sections with doctor’s signature and submit via fax back to Kineret on Track. Call in Prior Authorization (PA) and actual prescription to the patient’s insurance and provide hard copy and verbal PA to Biologics specialty pharmacy (SP). Kineret on Track will assign the patient a case manager based on his/her region once in the system. The case manager will guide the patient through the insurance authorization and procurement of the Kineret. Case managers will also facilitate bridge (free drug) medication during insurance PA and denial/appeals process. All Care SP is the bridge supply pharmacy.
Provider Note: FDA Approved Indications-NOMID, CAPS
**However, insurance approves SJIA-put indication under SJIA
Kineret is available as an infusion but generally not recommended as safety profiles favor the injection route. Infusions are utilized for severe MAS episodes.
Biologics SP#: 800-850-4306
**Available in limited distribution in the U.S. by Biologics SP only.
All Care SP #: 844-581-7454
** Free Bridge Supply SP via drug manufacturer
Provider Note: You will be required to also call in a verbal prescription to All Care with refills. Providing refills will augment weekly need to call in continued bridge supplies until the medication is approved for shipment. Bridge supplies are only authorized every 7 days by Kineret On Track if the patient’s PA is pending or any ongoing insurance issue (commercial insurance only). Patient Assistance Program (PAP) for uninsured or under-insured and government (TRICARE, Medicaid, Medicare-including dual eligible SSI/disability) are subject to different benefits.
Commercial maximum patient responsibility per shipment: $25
Long-acting IL-1(b) inhibitor. Can be used to control MAS episodes as well.
Provider note: Approved FDA Indications-SJIA, AOSD (and other indications). List indication under either SJIA or AOSD.
Patient Assistance Contact Information: Ilaris Support Program
Ilaris Support Program Phone #: 866-972-8315
All Care SP# 844-581-7454
**If your insurance is delaying your first shipment 2 wks or more your first shipment is free and your provider must call All Care SP to provide a prescription verbally over the phone to ship to you.
Commercial maximum patient responsibility per shipment: $30
Provider Note: Novartis recently received FDA approval to expand the indications of Ilaris to include AOSD. Please do check your dosing is correct and is based off the correct indication and kg/mg, not the SJIA standard 150mg/1ml dosing which is not appropriate for adult dosing. Ilaris is not a pre-filled injection. You may request either a nurse to come to the patient’s home for home injection training (must select this on the start form). You may also give the injection in office. The patient may give the injection at home going forward once shown how to draw up and give the injection.
IL-6 inhibitor. Generally prescribed for those Still’s patients with more arthritis symptoms.
Provider Note: FDA approved indications-RA. Must list indication under RA for approval.
Manufacturer: Sanofi and Regeneron Pharmaceuticals
Patient Assistance Contact Information: Kevzara Connect
Kevzara Connect Phone# 844-538-9272
Commercial maximum patient responsibility per shipment: *$0
*as low as $0 for most commercial insurance providers.
IL-6 inhibitor. Generally prescribed for the Still’s patients with more arthritis symptoms.
Provider Note: FDA Approved indications-PJIA, SJIA and RA. May prescribe under any of the above three.
Manufacturer: Genentech (Roche)
Patient Assistance Contact Information: Actemra & You
Website: actemrahcp.com (you must access the healthcare provider site)
Actemra & You phone # 800-228-3672
Commercial maximum patient responsibility per shipment: $5
|TNFa Inhibitors||adalimumab (Humira), etanercept (Enbrel), infliximab (Remicade)|
|JAK Inhibitors||golimumab (RINVOQ), tofacitinib (Xeljanz)|
|Others||abatacept (Orencia), rituximab (Rituxan)|
Patient assistance information: See IL-1 and IL-6 front line biologic information above. Other biologics have individual drug manufacturer patient assistance programs which operate similarly as above. You will find your specific therapeutic agent under its [drugname].com (e.g. ilaris.com) like above.
Front line biologic agent in RA. Often used in Still’s to treat joint (arthritic) involvement, but does not generally treat systemic issues.
Evidence suggests that TNFa treatment is less efficacious than IL-1 or IL-6 treatment in controlling Still’s disease. TNFa’s do have higher safety profile concerns if used with increased frequency and duration. TNFa’s have a generally efficacy rate of about 30%, thus providing sub-optimal outcomes. Insurance may require use of TNFa’s prior to front line Still’s biologics (IL-1 or IL-6) listed above.
There are now a wide number of TNFa inhibitors on the market, so the specific agent your provider elects to prescribe may not be listed.
Unlike any other type of biologic, JAK Inhibitors can be prescribed orally.
The JAK/STAT system act as key inhibitors as part of IL-6 inflammatory cascade.
**There is a current patient trial evaluating the efficacy of JAK inhibitors in the SJIA-MAS population. The study is being conducted at Cincinnati Children’s Hospital.
There are a variety of other biologics on the market with various mechanisms of action (MOA). Selecting the most efficacious therapy is based off clinical findings, personal history and responses, genetics, and any specific symptoms/comorbidities for the patient.
Corticosteroids are considered the traditional gold standard therapeutic for most autoinflammatory and autoimmune diseases. Steroids are utilized for most any inflammation. Sterioids are utilized as both first line and adjunctive therapy for Still’s disease treatment to reduce chronic systemic inflammation and as anti-pyretics for fevers.
However, long term use of steroids is associated with an array of complications. Thus, biologics came onto the market as a steroid sparing therapeutic effects.
|corticosteroids (prednisone, prednisolone, IV Solumedrol, others)||Anti-inflammatory|
Disease Modifying Anti-Rheumatic Drugs. DMARDS are often used in conjunction with biologics to more effectively suppress the immune system.
Step therapy approaches often require use of DMARDs before biologics will be approved. Some state medical laws have circumnavigated the step edit requirement with PA’s and make it easier to get a biologic as front line therapy.
|leflunomide (Arava)||Pyrimidine synthesis inhibitor|
|methotrexate (MTX)||Purine metabolism inhibitor|
|sulfasalazine (SSZ)||Suppression of IL-1 & TNF-alpha, induce apoptosis of inflammatory cells and increase chemotactic factors|
Used when more general immunosuppression is needed.
|mycophenolate (CellCept)||T-lymphocytic suppression|
|tacrolimus (Prograf)||CD4 & T helper cell inhibition by binding to cellular receptor FK506-binding protein|
|azathioprine (Imuran)||Purine synthesis/nucleic acid inhibitor|
|ciclosporin (Cyclosporin A)||Calcineurin inhibitor|