Juvenile Arthritis with Systemic Onset (Stills Disease)
Short summary
50-year-old male was diagnosed with Juvenile arthritis with systemic onset (Stills disease) after having symptoms of high fever, rashes and pain. He was treated with high doses of corticosteroids, but had two exacerbations in the last 2 years. In the current time he suffers of swelling of knee joints and hand joints, and occasionally a feeling of burning feet or cold feet. In the expert's opinion, the clinical description fit well with the diagnosis of adult onset systemic arthritis which manifestations are similar to the juvenile systemic arthritis. The treatment of choice is non-steroidal anti-inflammatory agents for very mild cases and steroids in more severe cases. Methotrexate is recommended to reduce steroid toxicity.
Patient's questions
1. Is there any possibility to treat his disease?
2. Are there any other medicines (except corticosteroids) available that have less side effects?
3. Are there any biological medicines? What are the risks of this treatment? What is the cost of this treatment?
Medical Background
Male, born in 1960
Diagnose: M08.2 Juvenile arthritis with systemic onset (Stills disease)
He had begun to feel ill at the age of 44, in 2004, with a fever (40 degrees Celsius), rashes and pain all over the body.
Treatment: Initially, he was treated with high doses of corticosteroids (methylprednisolone, 110 mg daily). The dose was gradually lowered to 4 mg daily. In the last 2 years he had two exacerbations of the disease which were successfully treated by the increased dose of methylprednisolone (10 days, 32 mg), which was later gradually lowered to 4 mg again.
Current physical status: Swelling of knee joints and small joints in hands. Occasionally he has a feeling of burning feet and at other times, a feeling of cold feet.
Medical opinion
Male, D.O.B. 1960
The clinical description provided may fit well with the diagnosis of adult onset systemic arthritis. Although uncommon in adults, manifestations are very similar to the juvenile systemic arthritis. While there is no specific serological marker in this disease, elevated sedimentation rate, increased CRP and very high ferritin levels are common. Liver enzymes may also be elevated and should be monitored especially if certain medication such as Methotrexate are used. ANA and Rheumatoid factor are usually negative in this condition.
X-rays of the joints and chest as well as echo of the heart (pericardium) may be helpful in identifying serositis (inflammation and fluids around the lungs and the heart).
The treatment of choice is non-steroidal anti-inflammatory agents such as ibuprofen or naproxen for very mild cases and steroids in more severe cases. The patient seems to have responded well to steroids which is a good sign. He seems to have had a recurrence of symptoms which is common in this condition. The decision to treat him again with steroids was the right choice according to the limited amount of information provided.
The problem with steroids is there adverse events, if they are given for long periods of time. Methotrexate is currently used quite successfully as a “steroid-sparing drug”. Others have used Cyclosporine A with some success.
Biologic response modifies such as TNF inhibitors have also been used in extreme cases. It’s not clear whether the effects are durable while the side effects associated with this treatment could be grave, including cancer. I would not recommend this treatment in this case unless all other treatments have failed.
In summary, systemic rheumatoid arthritis is an incurable autoimmune disorder. However, appropriate treatment can control symptoms and allow for a good quality of life. To reduce steroid toxicity, I would recommend trying to add Methotrexate.
