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Still Syndrome: Causes, Symptoms and Treatment

Still syndrome is a rare inflammatory disease related to rheumatoid arthritis, affecting both children and adults. It is characterized by spiking fever, joint pain, and a salmon-colored skin rash.

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Things worth knowing about "Still syndrome"

Still syndrome is a rare inflammatory disease related to rheumatoid arthritis, affecting both children and adults. It is characterized by spiking fever, joint pain, and a salmon-colored skin rash.

Overview

Still syndrome, also known as Still disease, is a rare systemic inflammatory disorder belonging to the group of autoinflammatory diseases. It can affect both children and adults. In children, it is referred to as systemic juvenile idiopathic arthritis (sJIA), while in adults it is called adult-onset Still disease (AOSD). The condition is characterized by excessive activation of the innate immune system, leading to widespread inflammatory reactions throughout the body.

Causes

The exact cause of Still syndrome has not been fully established. It is believed that a combination of genetic predisposition and external triggers, such as infections, plays a role. Central to the disease is a dysregulation of the innate immune system, in which pro-inflammatory cytokines – particularly interleukin-1 (IL-1), interleukin-6 (IL-6), and interleukin-18 (IL-18) – are released in excessive amounts.

Symptoms

Still syndrome presents with a characteristic triad of:

High, spiking fever: Daily fever episodes, often exceeding 39 °C (102 °F), typically occurring in the evening or at night
Joint pain and arthritis: Multiple joints are often affected simultaneously (polyarthritis), leading to swelling and restricted movement
Salmon-colored skin rash: A transient, maculopapular rash that frequently appears in parallel with fever spikes

Additional possible symptoms include:

Enlarged lymph nodes (lymphadenopathy)
Enlargement of the liver and spleen (hepatosplenomegaly)
Inflammation of the pericardium (pericarditis) or the pleura (pleuritis)
Severe fatigue and general malaise
Sore throat

Complications

A feared and potentially life-threatening complication of Still syndrome is macrophage activation syndrome (MAS), also known as hemophagocytic lymphohistiocytosis. In this condition, immune cells are uncontrollably activated, leading to a severe inflammatory response throughout the body. Symptoms include persistent high fever, a decrease in blood cell counts, and organ damage.

Diagnosis

The diagnosis of Still syndrome is a diagnosis of exclusion, as there is no specific diagnostic test. Physicians rely on:

Clinical criteria (e.g., Yamaguchi criteria for adult-onset Still disease): fever, arthritis, rash, and elevated ferritin levels
Laboratory tests: Markedly elevated serum ferritin (often above 1,000 ng/ml) is a key indicator; additionally elevated inflammatory markers such as CRP and ESR, as well as leukocytosis
Imaging studies (X-ray, ultrasound, MRI) to exclude other conditions
Exclusion of infectious diseases, other rheumatic conditions, and malignancies

Treatment

Treatment of Still syndrome is guided by disease severity and response to therapy:

Medical Therapy

Nonsteroidal anti-inflammatory drugs (NSAIDs): First-line therapy in mild cases to relieve pain and fever
Corticosteroids (e.g., prednisolone): Used in moderate to severe cases for rapid suppression of inflammation
Conventional disease-modifying antirheumatic drugs (DMARDs) such as methotrexate: For long-term management and reducing corticosteroid dependence
Biologics: When conventional therapies are insufficient, IL-1 inhibitors (e.g., anakinra, canakinumab) or IL-6 inhibitors (e.g., tocilizumab) are employed

Course and Prognosis

The clinical course of Still syndrome is variable. Some patients experience a single self-limited episode, while others follow a chronic or relapsing pattern. With modern treatment options, particularly biologic therapies, good disease control can be achieved in the majority of patients.

References

Fautrel B. - Adult-onset Still disease. Best Practice & Research Clinical Rheumatology, 2008.
Ravelli A., Martini A. - Juvenile idiopathic arthritis. The Lancet, 2007.
Feist E., Mitrovic S., Fautrel B. - Mechanisms, biomarkers and targets for adult-onset Still's disease. Nature Reviews Rheumatology, 2018.

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