Still’s Disease is a Systemic form of Rheumatoid Arthritis. The term ‘systemic’ here is an important one and means that, alongside the joint inflammation that people associate with Arthritis, there are also body-wide symptoms such as fever, rash, swollen glands and internal organ involvement.
Still’s Disease was first diagnosed as a childhood disease, although in recent years doctors have favoured the term ‘Systemic Juvenile Idiopathic Arthritis’ or JIA when referring to the disease in children. Similarly, in cases including adults, the disease can be referred to as Adult Onset Still’s Disease or AOSD. I have also seen mention of it as Wissler-Fanconi Syndrome, although there seems to be some confusion as to whether this is a separate (but similar) condition.
Still’s Disease is often described as an Auto-Immune Disease, which means that the immune system becomes overactive and destroys healthy cells, rather than just those invading the body. This is why the majority of treatments for Still’s Disease are classed as Immuno-Suppressants; working to stop this destruction.
The reason for autoimmunity in itself could be triggered by a number of factors including infection, allergy, trauma or hypersensitivity – the jury is still out on that one. You will come across many different theories if you look into it; my own feelings are that certain vaccinations triggered my symptoms, but I’m not a doctor and of course it could be different for everyone.
Patients with Still’s Disease typically present with symptoms that are initially systemic in nature but this is eventually followed by joint involvement. These initial symptoms include:
- Fever of 39° or greater. This often fluctuates throughout the day, reaching ‘peaks’ at the same time each day and sometimes coinciding with the rash.
- A faint salmon coloured rash that usually appears on the torso and limbs and does not itch. It seems to be more noticeable under fluorescent lighting and during times of fever.
- Fatigue and weakness, which does not improve with sleep.
- Sore throat, described as a ‘burning’ pain.
- Swollen glands and lymph nodes.
- Loss of appetite and general malaise.
- Enlargement of the Spleen and Liver, often accompanied by abdominal discomfort.
- Muscle pain and tenderness.
- Then of course the joint swelling and inflammation, with wrist involvement being typical.
Still’s Disease can also lead to inflammation of the eye, called Iritis. You can read more about this connection here.
As well as presenting with the above symptoms, there are also some clinical signs that point to Still’s Disease that are found in your blood work. These include:
- Increased number of White Blood cells (WBCs) and Neutrophils. This can lead doctors to suspect a serious infection but usually none is found.
- Elevated ESR (Erythrocyte Sedimentation Rate), also known as ‘Sed Rate’ and CRP (C-Reactive Protein) levels; both of these are classed as inflammation markers – the higher the level, the more inflammation and disease activity there tends to be.
- High Ferritin Levels – this seems to be particular to Still’s Disease.
- Reduced Red Blood Cell (RBC) count, leading to Anaemia.
The blood tests used to diagnose Rheumatoid Arthritis (Rheumatoid Factor) and Systemic Lupus Erythematosus (AntiNuclear Antibodies or ANA) are almost always negative.
This Glossary is useful when trying to understand your own results
Different treatments work for different people, which means that it can take a while to find the right one for you. There does seem to be a pattern in their usage though, which I’ll try and highlight, but this is only taken from my experience and limited research. Eventually, I’ll put a more in-depth section on treatment up.
STAGE 1: Doctors may try and control mild symptoms using drugs call Non-Steroidal Anti-Inflammatory Drugs or NSAIDs. These include:
There are also a group of drugs called Cox-2 inhibitors, but I think these are used more sparingly now since Celebrex was withdrawn, due to their side effects.
If symptoms are more severe or NSAIDs do not work, your doctor may try a drug called Prednisolone, which is a Corticosteroid. Most people have a love/hate relationship with Prednisolone – it can work wonders in bringing relief and getting symptoms to ease quite quickly but also has serious side effects and so is usually only a short term option. This means it is useful in flareups and acute disease activity but should never be depended upon in the longterm. If a particular joint is troubling you, the doctor may decide to treat it directly, by injecting an equivalent Hydrocortisone into the joint.
STAGE 2: If the NSAIDs alone do not work or initial symptoms get worse, doctors will usually then turn their attention to finding a Disease Modifying Anti-Rheumatic Drug or DMARD. These drugs ease symptoms by blocking the mechanisms that cause anti-bodies to attack joints etc, therefore slowing down disease progression. I think most, if not all, are also classed as immuno-suppressant drugs and include:
Cellcept (Mycophenolate Motefil)
Immunoglobulin Therapy (Iv Ig)
Sometimes you may have to try a few of these options before discovering which one works best for you or is tolerated best; the drugs in bold are the ones I have tried over the years. They all come with their own list of side effects and monitoring requirements.
STAGE 3: Sometimes a DMARD may not work well enough on its own, or may become less effective over time. In which case your doctor may move onto the newest line of treatment called Biologic Drugs. These are administered via injection or infusion and the mechanisms of each can be different; often, they are taken alongside Methotrexate or a similar DMARD. Biologic Drugs include:
Enbrel (Etanercept) – via injection
Kineret (Anakinra) – a noteable drug in the treatment of Still’s Disease – via injection
Humira (Adalimumab) – via injection
Cimzia (Certolizumab pegol) – via injection
Simponi (Golimumab) – via injection
MabThera (Rituximab) – via IV infusion
Remicade (Infliximab) – via IV infusion
Orencia (Abatacept) – via IV infusion
RoActemra (Tocilzumab) – another promising drug for Still’s Disease – via IV infusion
As I said previously, these are the newest line of drugs used to treat Still’s and other auto-immune diseases and there is ongoing research and development into them; new options seem to be appearing all the time.
OTHER DRUGS: There are also a number of other medications that you may require in managing Still’s Disease. The main one is probably Pain medication, which can include Paracetamol, Ibuprofen, Codeine /Co-codamol, Tramadol, Pain killing patches and Oramorph, just to name a few. Some people may require anti-depressants and sleeping tablets to cope with the pain and emotional aspects of living with a chronic illness, but I will have to look into these further before commenting since I am not familiar with them. Supplements such as Iron tablets, Calcium and Vitamin D, Folic Acid, Cod Liver Oil, Glucosamine are often necessary or desirable too.
To Be Continued…
There is also a post entitled ‘Confused About Stills Disease?‘ that you might find worth reading, even if some of the information is repeated.