How does Still's disease relate to juvenile idiopathic arthritis?
Still's disease is one type of juvenile idiopathic arthritis (JIA) and is also known as systemic-onset JIA. It is referred to as “juvenile” because it typically affects children under 16 years of age. By systemic, it is meant that along with joint inflammation it typically begins with symptoms and signs of systemic (body-wide) illness, such as high fevers, gland swelling, and internal organ involvement. By idiopathic, it is meant that the disease has no known cause. Systemic-onset JIA was formerly known as systemic-onset juvenile rheumatoid arthritis (JRA) and is the same disease. Several years ago, the naming system for all types of JRA changed, and JRA is now called juvenile idiopathic arthritis or JIA.
What are symptoms and signs of Still's disease?
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Still's disease usually begins with systemic (body-wide) symptoms. Extreme fatigue can accompany waves of high fevers that rise daily to 102 F (39 C) or even higher and rapidly return to normal levels or below. Fever spikes often occur at approximately the same time every day. A faint salmon-colored skin rash characteristically comes and goes and does not itch.
Poor appetite, nausea, and weight loss are common. There is also commonly swelling of the lymph glands, enlargement of the spleen and liver, and sore throat. Some patients develop inflammation around the heart (pericarditis) and lungs (pleuritis), with occasional fluid accumulation around heart (pericardial effusion) and lungs (pleural effusion). Arthritis, with joint swelling, often occurs after rash and fevers have been present for some time. Although the arthritis may initially be overlooked because of the impressive nature of the systemic symptoms, everyone with Still's disease eventually develops joint pain and swollen joints. This usually involves many joints (polyarticular arthritis). Any joint can be affected, although there are preferential patterns of joint involvement in Still's disease
Still's disease is diagnosed purely on the basis of the typical clinical features of the illness. These features include high intermittent fever, joint inflammation and pain, muscle pain, faint salmon-colored skin rash, swelling of the lymph glands or enlargement of the spleen and liver, and inflammation around the heart (pericarditis) or lungs (pleuritis). Persistent arthritis (arthritis lasting at least six weeks) is required to make a firm diagnosis of Still's disease. Other diseases (especially infections, cancers, and other types of arthritis) must be excluded.
Many patients with Still's disease develop markedly elevated white blood cell counts, as if they have a serious infection, but no infection is found. Low red blood counts (anemia) and elevated blood tests that indicate inflammation (such as erythrocyte sedimentation rate or ESR, C-reactive protein, and ferritin) are common. However, the classic blood tests for rheumatoid arthritis (rheumatoid factor) and systemiclupus erythematosus (antinuclear antibodies) are usually negative.
Of all patients with Still's disease, 100% have high intermittent fever, and 100% have joint inflammation and pain, muscle pain with fevers, and develop persistent chronic arthritis. Approximately 95% of Still's disease patients have the faint salmon-colored skin rash, 85% have swelling of the lymph glands or enlargement of the spleen and liver, 85% have a marked increase in the white blood cell count, 60% have inflammation of the lungs (pleuritis) or around the heart (pericarditis), 40% have severe anemia, and 20% have abdominal pain.
Spot is still there and now a second popped up. So very painful.
. I'm hoping for big changes with Remi. I hope it works fast!
