Patients of Adult-Onset Still's Disease Association (PASDA)
The lowdown on Adult Onset Still’s Disease
There are many medical websites that can give you more detailed information about AOSD, but one of the reasons this organisation began was that we, the patients, felt that so much of this information can be very confusing to your average layman; we wanted to breakthrough the jargon.
So the information given below is NOT intended to replace or discredit any information that you may be given by your medically trained professionals, it simply aims to give you key information that will hopefully answer a few of the million questions you have when you are diagnosed. The answers have been written by patients based on their own experiences and those of others known to them, but also verified by our rheumatologists.
What are the main symptoms?
In the beginning, some patients experience a sudden and violent onset of symptoms whereas others experience them gradually more and more over time. Symptoms include (some more than others depending on the patient): extreme tiredness, spiking fevers, salmon pink rash (often at the same time as the fevers but not always), poor appetite, nausea, and weight loss, sore throat (or swelling of the lymph glands), enlargement of the spleen and liver, and finally swelling of the joints (usually involving many joints – “polyarticular” arthritis).
How long will it last?
Many cases burn out, but some are recurrent and a few are persistent. For most people with AOSD, the characteristic fever and rashes usually go away after the initial onset or after several months. The swelling of joints, however, can continue as a long-term condition. Both Still's Disease and AOSD can disappear on its own for 35% to 40% of people within ten years of onset. In other cases, patients can develop chronic arthritis.
You are not likely to die from AOSD, but there can be many problems that can arise from infections such as amyloid and macrophage. activationsyndrome (albeit these are extremely rare).
Can it be cured?
Not as such, but as noted above, most cases will burn out of their own accord eventually. The aim of treatment is to prevent the patient from getting worse and to prevent long-term joint damage. Treatment of the disease is therefore often targeted at specific areas of inflammation, which tend to be different for each patient. There are also drugs that can sometimes suppress the condition completely, which can be used in cases where the illness does not switch off by itself or with the help of the usual drugs (see below).
What treatments are used?
Many symptoms of AOSD can be controlled with anti-inflammatory drugs or nonsteroidal anti-inflammatory drugs (NSAIDs). Cortisone medications such as prednisone are also used to treat the more severe symptoms of the disease. As AOSD often affects internal organs, some people with the disease require heart and lung medication as well as medication for diabetes. Pain medication is often necessary and used as required depending on the severity of symptoms. Other medications include hydroxychloroquine, penicillamine, azathioprine, methotrexate, etanercept, anakinra, cyclophosphamide, adalimumab, rituximab, and infliximab. In addition tocizilumab seems to be particularly effective and can suppress the condition entirely.
So what is it?
Still’s Disease is a juvenile arthritic condition that affects children and teenagers. Still’s Disease accounts for 10-20% of cases of arthritis in this age group. However Adult-Onset Still’s Disease (AOSD) is the adult form of Still’s Disease, and it is much more rare. Only 1 in 100,000 adults develop AOSD. The symptoms usually start to occur between the ages of 20 and 35.
Most of the time AOSD is classed as an arthritic condition because of the pain and inflammation it causes in the joints. However, because it also affects the blood stream, causing the rash and fevers, it can sometimes be classed as a blood disorder. As it is so rare in adults, even many rheumatologists know very little about it. There is no test that proves its diagnosis and as such it often goes undiagnosed for weeks or even months. Plus, as AOSD or its treatments can lead to problems with the internal organs, often a patient is treated as if they have AOSD alongside something else.
How do you get it?
There are many different theories as to the cause of AOSD, but basically the cause is unknown. We don’t know the ultimate mechanism of AOSD,
but it involves the release of chemicals from white cells (“interleukins”), which create the inflammation.
What is its history?
Still’s Disease is named after Sir George Still (1868 – 1941), England’s first professor of childhood medicine. Interestingly, he was the house officer working in the evening, and so he saw the rash on the children when everyone else had gone home, and could therefore judge the disease to be different from other forms of arthritis. He published his results in his paper “On a form of chronic joint disease in Children” (1897). AOSD was first characterized by EG Bywaters in 1971.