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Treatment Options for Still's Disease

Jul 22, 2022 11:31 am

Begin – are you treating “systemic” or “articular” (arthritis) Still’s disease? Most Still’s patients have a dominance of one or the other. With certainty, the right therapy for the right symptoms can be chosen.

  1. Systemic disease: key symptoms/findings include high daily fevers (>102oF), rashes, pleuritis or pericarditis, enlarged liver/spleen/lymph nodes, wt. loss
    • Labs: active disease is indicated by very high ESR, CRP, WBC, ferritin levels, aldolase, increased LFTs; with anemia (low Hgb/Hct), low albumin
  2. Articular disease: key symptoms are multiple painful, swollen joints (usually hands, wrists, knees, ankles, feet, shoulders), often with fatigue & stiffness
    • Labs: active disease usually has normal WBC, mild anemia, increased ESR or CRP, negative tests for ANA & RF. (Normal: ferritin, albumin, LFTs)

Sequential Drug Use – The goal is to rapidly control of pain and inflammation – this is starts with using “1st Line Therapy”.

  • 1st Line Therapy: start with drugs that act quickly (hours, days). The potential down side of fast-acting drugs may be long-term side effects; this is the case with NSAIDs and steroids; see Best Drugs Table below. With time 1st line drugs are replaced by more long-lasting therapies – “2nd Line Therapy”.
  • 2nd Line Therapy: these drugs are referred to as Disease Modifying Anti-Rheumatic Drugs (DMARDs). DMARDs may be oral pills or injectables and are typically a) slow-acting (weeks to months), b) given chronically (often called “steroid-sparing drugs”), and c) are given to change the underlying disease. “Biologic” DMARDs (injectable or parenteral) are similar to your body’s proteins, and are used to specifically regulate an overactive immune system.

When Not Responding? While most patients respond well to the treatments noted below, some patients may have an incomplete or no response, or become unresponsive to a drug that once worked well. Three key questions need to be answered when the patient is not responding (or refractory): (1) Is this the right diagnosis? (2) Is this Systemic or Articular disease? (3) I there another problem (new diagnosis/complication or medication side effect developing)? Off-Label Use: When poorly responsive or refractory to standard DMARD or biologic therapy, numerous options can be considered. All of these are considered “off-label”, as these treatment options are not FDA approved for use in Still’s disease. Nearly all treatments used in Still’s disease are “off-label” because Still’s is a very rare, for which there are few studies that might lead to FDA approved drugs. Only tocilizumab & canakinumab are FDA approved for use in children (sJIA) and only canakinumab is FDA approved for adult-onset Still’s disease (AOSD). MAS (Macrophage Activation Syndrome): MAS is a severe complication of “systemic” Still’s disease, where uncontrolled, hyper-inflammation can lead to organ damage. MAS is associated with a 10-35% risk of death. MAS should be considered with very high fever, hypotension, altered mental status, very high levels of CRP, liver enzymes, and ferritin (often >10,000), along with a sudden drop in blood counts (leukopenia, lymphopenia, thrombocytopenia). Drugs used to treat MAS include high dose steroids, inhibitors of IL-1 or IL-6, emapalumab, cyclosporine, etoposide or JAK inhibitors. 

TREATMENT OPTIONS IN STILL’S DISEASE

Treatment Stage

Systemic Disease

Articular Disease

1st Line Therapy

Prednisone 60 mg/day (or >1mg/kg/day) (Or high doses of IV methylprednisolone)

NSAIDs Low dose Prednisone 5-10 mg/day

2nd Line Therapy

IL-1 inhibitors SC IL-6 inhibitors SC or IV (Methotrexate may be added to above)

Methotrexate or Leflunomide TNF inhibitors SC or IV Canakinumab SC Tocilizumab SC or IV

Refractory Disease Drugs

Methotrexate JAK inhibitors IVIG

JAK inhibitors, Rituximab, Abatacept, Azathioprine Hydroxychloroquine, cyclosporine A

Best Drugs Table

Generic name

Example

Type of Drug

How given

Usual dose

Used to treat:

NSAIDS

Naproxen Ibuprofen

Meloxicam

Antiinflammatory

Oral pills

Naproxen: 500mg Ibuprofen 600-800mg

Meloxicam 7.5, 15 mg 

Joint pain, stiffness, aching, swelling

Prednisone

Low dose Prednisone

High dose Prednisone

Corticosteroid

Antiinflammatory

Oral pills

Low dose 2.5-10 mg/d

High dose 60-80 mg/d

Articular Still’s

Systemic Still’s

Methylprednisolone

Solumedrol

Corticosteroid

Antiinflammatory

Intravenous

1-2 mg/kg/d

Systemic Still’s

MAS

Methotrexate

Rheumatrex

DMARD

Antiinflammatory

Oral pills or 

Subcutaneous injection

7.5-25 mg

(Kids: 0.3-0.6 mg/kg)

Articular Still’s

Systemic Still’s

Leflunomide

Arava

DMARD

Antiinflammatory

Oral Pills

10-20 mg

Articular Still’s

Azathioprine

Imuran

DMARD

Immunosuppressive

Oral Pills

50-200 mg

(1-2 mg/kg)

Articular Still’s

Systemic Still’s

Hydroxychloroquine

Plaquenil

DMARD

Oral pills

200-400 mg

(< 5.5 mg/kg)

Articular Still’s

JAK inhibitors

Xeljanz, Olumiant, Rinvoq

DMARD

Oral pills

Xeljanz 11 mg; Rinvoq 15mg; Olumiant 2mg

Articular Still’s

Canakinumab

Ilaris

Interleukin-1 inhibitor

Subcutaneous injection

150-300 mg

(4 mg/kg)

Systemic Still’s Articular Still’s

Anakinra

Kineret

Interleukin-1 inhibitor

Subcutaneous injection

100 mg (some 200mg)

(1-2 mg/kg)

Systemic Still’s Articular Still’s

Tocilizumab

Actemra

Interleukin-6 inhibitor

Subcutaneous or Intravenous

SC: 162 mg

IV: 4-12 mg/kg

Systemic Still’s Articular Still’s

Infliximab

Remicade

TNF inhibitor

Intravenous infusion

3-10 mg/kg

Articular Still’s

Adalimumab

Humira

TNF inhibitor

Subcutaneous injection

40 mg

Articular Still’s

Etanercept

Enbrel

TNF inhibitor

Subcutaneous injection

25 or 50 mg

Articular Still’s

Certolizumab

Cimzia

TNF inhibitor

Subcutaneous injection

200 mg

Articular Still’s

IVIG

(IV gammaglobulin)

Gammagard (many brands available)

Immunoglobulins Immunosuppressive

Subcutaneous or Intravenous

1-2 g/kg.

Systemic Still’s

Rituximab 

Rituxan

B-cell inhibitor

Biologic DMARD

Intravenous infusion

1000 mg

Articular Still’s

Abatacept 

Orencia

T-cell inhibitor

Biologic DMARD

Subcutaneous or Intravenous

SC: 125 mg

IV: 500, 750, 1000mf

Articular Still’s

(Abbreviations: SC-subcutaneous; IV-intravenous; NSAID-nonsteroidal anti-inflammatory drugs; IL-interleukin; LFTs-liver function tests; TB-tuberculosis; BP-blood pressure)

 

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