Adult Still’s Disease

What is Adult Still's Disease?

Adult-onset Still's disease is a rare inflammatory disorder marked by daily fever, inflammatory arthritis in several joints and a transient salmon-pink rash.^[1] It is the adult form of what children have as systemic juvenile idiopathic arthritis, and European experts now consider the two to be one disease.^[2]

Common symptoms

The classic symptoms are spiking fever, joint inflammation and a rash that appears and fades.^[3] Sore throat, enlarged liver, spleen or lymph nodes, and inflammation around the lungs or heart are also common.^[3]

People often feel very tired. The course varies: some have one episode, some have repeated flares with remissions, and some have persistent disease with chronic arthritis.^[3]

Causes and risk factors

The cause is unknown.^[3] Activation of macrophages and high levels of inflammatory signals called interleukin-1, interleukin-6 and interleukin-18 play a central role in how the disease develops.^[4] The way the disease begins is still not completely understood.^[4]

How it is diagnosed and treated conventionally

There is no single test, so diagnosis rests on the clinical picture after infections, cancers and other autoimmune diseases are excluded.^{[3, 4]} Blood tests typically show high ESR and C-reactive protein, and ferritin is usually higher than in other inflammatory, infectious or cancer conditions.^[3]

Corticosteroids are commonly the first treatment when whole-body symptoms predominate. Conventional DMARDs help the joints, and biologics that block interleukin-1 or interleukin-6 are widely used.^[4] The 2024 EULAR/PReS recommendations favor early use of an IL-1 or IL-6 inhibitor with a short course of glucocorticoids, aiming for inactive disease and remission.^[2]

Macrophage activation syndrome is the most severe complication, reported in up to 15% of patients and carrying a high death rate.^[3]

Why Adult Still's Disease calls for a whole-person approach

Adult Still's disease is a systemic inflammatory condition that needs specialist treatment, and no complementary therapy has been shown to control it. Living with flares, pain, fatigue and long-term medicines can still affect sleep, mood and daily function, which are areas where supportive care may be considered.^[3]

The factors below are the ones most relevant to everyday life with the disease.

Whole-body inflammation

Inflammation in Still's disease reaches beyond the joints and can involve the liver, spleen, lymph nodes and the lining of the heart and lungs.^[3] Because it is systemic, any new symptom, especially fever, should be reported to your rheumatologist before any supportive care is considered.

Joint pain and stiffness

Joint inflammation is one of the three main features of the disease.^[1] Joint pain is one area where supportive care may be considered, although the studies below come from other kinds of arthritis.

Medicine effects

Treatment often relies on corticosteroids and immunosuppressive drugs, which may raise the risk of infection and other side effects.^[3] Liver involvement can be part of the disease, which is relevant when herbs are considered.^{[3, 5]}

Fatigue and stress

Fatigue is a common feature, and living with a flaring condition can be stressful.^[3] We do not know of Still's-specific studies on stress or fatigue relief, so supportive care here is general and is not backed by trials in this condition.

Acupuncture as supportive care in Adult Still's Disease

What the research shows

No clinical trials of acupuncture for adult Still's disease were found in PubMed. Evidence for acupuncture in this condition is therefore none, and it is not an anti-inflammatory treatment for the disease.

Some information comes from rheumatoid arthritis, a different disease with a different cause. A network meta-analysis of 32 trials with 2,115 patients found that electroacupuncture, fire needle or moxibustion added to DMARDs improved disease activity scores, pain or blood inflammation markers more than DMARDs alone, but did not shorten morning stiffness.^[6] A separate analysis of seven systematic reviews containing 20 randomized trials concluded that acupuncture probably has little or no impact on rheumatoid arthritis.^[7] The findings are mixed and cannot be assumed to apply to Still's disease.

How acupuncture may work

How acupuncture might act in Still's disease has not been studied. We do not claim that it changes the interleukin-driven inflammation of the disease.

What treatment involves

Treatment uses fine, sterile, single-use needles, usually left in place for about 20 to 40 minutes, over a short course of visits with reassessment. Sessions are aimed at comfort, relaxation and sleep, and we avoid inflamed or swollen joints and any skin rash area.

Serious problems from acupuncture are rare. In prospective studies, about 9 in 100 patients had a minor reaction such as bleeding, soreness or a flare at a needle site, and serious events occurred in about 1 in 10,000 patients.^[8] Because Still's disease is often treated with medicines that weaken the immune system, tell the practitioner about all medicines, any fever or infection, and whether you take blood thinners. Mention pregnancy or a pacemaker as well (relevant to electroacupuncture). Treatment should wait during a fever or flare until you have had medical advice.

Ayurvedic medicine as supportive care in Adult Still's Disease

The Ayurvedic view

Ayurveda has no classical name that matches Still's disease. Practitioners may place fever with joint pain and rash among conditions of aggravated Pitta, and joint disease in the Vata-Kapha group, with ama (undigested metabolic residue) described as a contributing factor. This is a traditional framework and not a biomedical diagnosis.

Therapies that may be used

Care is individual and chosen only after your medical treatment is established. It may include:

• A regular, easily digested diet and daily routine
• Gentle oil massage away from inflamed joints during a flare
• Individually prescribed herbs, after review of your medicines and liver tests

Panchakarma is not appropriate during pregnancy, acute illness or frailty, and it is not suitable while you have fever, an active flare or are taking immunosuppressive drugs, because of the risk of infection and dehydration.

What the research shows

We found no studies of Ayurvedic treatment in Still's disease, so evidence is none.

Turmeric (curcumin) has been studied in rheumatoid arthritis. A meta-analysis of six publications covering 539 patients found lower disease activity, swollen and tender joint counts and ESR with curcumin, and the authors called for larger trials.^[9] That is a different disease, and we do not know whether it applies to Still's disease. Herbal and dietary supplements are a recognized cause of liver injury, so any supplement, curcumin included, should be cleared with your rheumatologist.^[5]

Herbal medicine as supportive care in Adult Still's Disease

Herbs and formulas that have been studied

Two published case reports describe Chinese herbal formulas in adult Still's disease. One used a granule formula based on Xiaochaihu decoction and Yinqiao powder, and the other used Zhu Ye Shi Gao decoction added to a corticosteroid.^{[10, 11]} Formulas are prescribed individually by a qualified practitioner.

Tripterygium wilfordii (lei gong teng) is a Chinese herb studied for rheumatoid arthritis, not Still's disease.^[12]

What the research shows

The evidence is very limited. Each case report describes one 28-year-old patient who improved with the herbal treatment, one for 15 months without relapse after six weeks of treatment, and the other after a corticosteroid had given only partial relief.^{[10, 11]} A single case cannot show that a herb caused the improvement, and the first report calls for prospective studies. In the second, the patient was also given liver-protecting medicines alongside the corticosteroid.

For Tripterygium, an overview of 11 systematic reviews in rheumatoid arthritis found improvements in joint counts and inflammation markers, with quality of evidence rated low to moderate and gonad toxicity as a main concern.^[12] This does not show that it is appropriate in Still's disease.

Safety and herb-drug interactions

Herbs are active substances and can interact with medicines. Reviews describe bleeding when warfarin is combined with some herbs, and other clinically important interactions with a range of drugs.^{[13, 14]} Anything that affects the immune system needs review by your rheumatologist, because you may be taking steroids, methotrexate or biologics.

Herbal and dietary supplements are a recognized cause of liver injury, and about one fifth of Ayurvedic products bought online contained detectable lead, mercury or arsenic.^{[5, 15]} Because Still's disease and some of its treatments can affect the liver, we ask for recent liver tests. Herbs should come from tested sources and be prescribed individually. Tell us if you are pregnant or breastfeeding or have kidney disease.

Translating traditional medicine into modern biological language

This is interpretation, not equivalence. Chinese medicine may describe the fever, rash and thirst of an active flare as heat or toxic heat, and the fatigue between flares as deficiency of qi and body fluids, which is how one published case was framed.^[11] Ayurveda may describe aggravated Pitta or ama.

Modern researchers describe Still's disease as macrophage activation with high interleukin-1, interleukin-6 and interleukin-18 signaling.^[4] No study has shown that heat, qi or Pitta corresponds to these processes. The traditional terms may help a practitioner organize questions about energy, digestion and sleep, but they do not describe the disease mechanism.

How this care fits with your medical care

Complementary care for adult Still's disease is supportive only. For a condition like this, we ask that you are under a physician's care for it and that your treating team knows you are receiving complementary care. Keep your rheumatologist, take your corticosteroids, DMARDs and biologics as directed, and do not stop or change them without your prescriber.

We do not diagnose or manage Still's disease. Please bring your diagnosis, recent blood tests including liver tests and ferritin, a full list of medicines and supplements, and any recent infections or hospital stays.

When to seek urgent medical care

Still's disease can become serious quickly, and certain symptoms need prompt medical assessment.^[3]

• A new or persistent high fever, particularly with feeling very unwell, confusion or a rapidly worsening rash, since macrophage activation syndrome is a life-threatening complication
• Chest pain, shortness of breath or a racing heart
• Fever or signs of infection while on immunosuppressive medicines
• Yellow skin or eyes, or severe abdominal pain
• Unexplained bleeding or bruising
• Severe joint swelling that prevents movement

For emergencies, call 911 or go to the nearest emergency department.