Enfermedad de Still
Nuevo término general para la enfermedad de Still de inicio en la edad adulta (AOSD, por sus siglas en inglés) y la artritis idiopática juvenil sistémica (JIA, por sus siglas en inglés).
Still’s Disease is a rare, multi-systemic (whole body) autoinflammatory condition that can vary from person to person. Because of a similar pathogenesis, broadly overlapping symptoms, and organ involvement, Still’s Disease represents a disease continuum that includes both Systemic Juvenile Idiopathic Arthritis (SJIA) and Adult-Onset Still’s Disease (AOSD).
Those with onset
> 16 years of age are categorized with AOSD, while those originally diagnosed with SJIA carry this diagnosis through adulthood. Onset can occur at any age, even presenting after birth. In some individuals, Still’s Disease develops suddenly, disappears almost as quickly, and may not return for many years, if ever. In others, it is a chronic condition.
In 2017, the International Still's Disease Foundation (ISDF) dissolved and, as a trusted collaborator over the years, requested that our organization house any existing resources. Read the original dissolution announcement letter.
Symptoms of Still's Disease
Still's Disease symptoms and progression vary per individual. Some patients will have many of these symptoms, some only a few. This makes detecting, diagnosing, and treating Still’s Disease difficult. Severity and frequency will vary in degree based on the individual. Please view our resources, including our Still's Disease brochures and emergency cards, to learn more.
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ErupciónElemento de la lista 1
Most commonly a salmon colored rash appearing on the upper anterior torso, arms, and legs that may quickly fade. Typically does not itch or mild itch, however patients reported a rash that can itch, be raised, appear as a number of small, flat red spots, and may not quickly fade away. May not be visible if covered with hair or if it fades very quickly. (2)
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FeverElemento de la lista 2
High and spiking, rising to 104 degrees F/41 degrees C or higher and can rapidly return to normal levels or below. Time of day can vary, but evening and nighttime reported more often. May come and go, sometimes twice a daily, but may last a week or longer. Could cause for patient-reported excessive sweating/night sweats. (3)
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FatigueElemento de la lista 3
Reported in > 90%*; common in all autoinflammatory diseases, not unique to Still’s.
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MyalgiaElemento de la lista 4
Reported in > 75%* of patients, some described similar to flu-like weakness.
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Sore ThroatTodos los grupos de edad, con una incidencia aún mayor en niños. Algunos reportan dificultad para tragar.
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Artritis inflamatoriaElemento de la lista 1
Historically arthritis, or joint involvement, was considered one of the major Still's Disease symptoms. However, recent research suggests about 25% of patients may not have arthritis at or near onset - some may not develop it for years, if ever. In others, the arthritis is severe. Swelling, redness, warmth, and joint erosion is possible. Can cause severe stiffness after rest.
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Organ InvolvementElemento de la lista 2
Generalized lymph nodes/glandular swelling. Enlargement of the liver and/or spleen (4). Inflammation of lungs (pleuritis), the saclike covering of your heart (pericarditis), the muscular portion of your heart (myocarditis) (5), eyes, serous tissues (6). Organ involvement may be present at onset.
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GastrointestinalElemento de la lista 3
Nausea, weight loss, poor appetite, canker sores in mouth.
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Central Nervous System ManifestationsElemento de la lista 4
Including seizures, meningismus.
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Cognitive Dysfunction aka “Brain Fog”*
May be associated with inflammation in the brain caused by the immune response (7).
Diagnosing Still's Disease
La enfermedad de Still es un diagnóstico de exclusión, que requiere descartar primero todas las demás afecciones. El diagnóstico tardío es frecuente, ya que la enfermedad de Still a menudo se confunde con una infección. Si bien la investigación actual está en desarrollo, no existen pruebas ni biomarcadores específicos (9).
Las anomalías de laboratorio más comunes en pacientes con enfermedad de Still incluyen:
- Velocidad de sedimentación globular (VSG) elevada
- Aumento de leucocitos, especialmente neutrófilos (glóbulos blancos).
- Trombocitosis (recuento elevado de plaquetas)
- Niveles elevados de ferritina (a veces de forma drástica) Prueba de factor reumatoide (FR) negativa (10)
- Prueba de anticuerpos antinucleares (ANA) negativa
- Anemia (<10 g/dl)
- Hipoalbuminemia (niveles bajos de albúmina < 3 g/dl)
- Elevación leve de las enzimas hepáticas
Cause of Still's Disease
Some researchers suspect the condition might be triggered by a viral or bacterial infection or genetic mutations. While Still's is an autoinflammatory disease (of the innate side of the immune system), research suggests both sides of the immune system may be triggered, causing autoimmune (of the acquired side of the immune system) features as well. Learn about the sides of the immune system.
*These statistics and experiences are the result of our internal research, then medically reviewed by the Center for Autoinflammatory Diseases. Therefore, the percentages may vary from research conducted elsewhere.
Otras complicaciones asociadas con la enfermedad de Still
Macrophage Activation Syndrome (MAS) - a massive inflammatory response that overwhelms the whole body, including organs. MAS is more common in SJIA and may be present at onset (8). Check out our MAS page for information, resources, and patient stories.
Mental Health
Mental health - A quarter of patients surveyed reported mental health issues including: irritability, low self esteem, and difficulty coping with stress, anxiety, isolation, frustration, and depression.
Still's Disease Resources & Support
At AiArthritis, we place community voice at the heart of everything we do. Our Still’s Disease resource hub was created through collaboration with patients and clinicians to develop materials that reflect lived experiences and medical expertise. Here you can explore brochures, educational tools, awareness materials, and clinician resources designed to educate, empower, and support personalized care.
The dragon shown is the original logo designed by the International Still's Disease Foundation, who dissolved into AiArthritis in 2017 and is replicated in the Still's Disease brochures and emergency cards.
International Still's Disease Support Center
Un recurso AiLa artritis se apoderó tras la disolución de la Fundación Internacional para la Enfermedad de Still (ISDF). El grupo de apoyo para la enfermedad de Still en Facebook es donde las personas afectadas por esta enfermedad pueden hacer preguntas y obtener apoyo de otras personas como ellas. Si usted es una persona que vive con la enfermedad de Still (paciente o familiar/pareja), ¡únase hoy mismo!
Folletos y tarjetas de emergencia sobre la enfermedad de Still reportada por el paciente
Our #MyStills Still’s Disease brochures and folding information cards are available as free, downloadable PDFs in English, Spanish, and French. The brochures were co-created with Still's patients and health professionals. Brochures provide a clear overview of the disease, while the compact emergency/new health professional folding cards highlight key symptoms, important testing, and space for patients to write personalized alerts.
Macrophage Activation Syndrome (MAS)
MAS is a condition that occurs when there is an extreme excess of inflammation caused by an overactive immune system. This
causes uncontrolled, extreme activation of macrophages and
T-Cells (cytokine storm) that then attack and cause damage to any healthy cells and tissue. It is emergent, life-threatening, and
requires immediate medical attention.
Quick Still's Disease Symptom Sheet
Este recurso se desarrolló a partir de la investigación sobre la experiencia de la enfermedad reportada por los pacientes, realizada por nuestra organización, combinando los resultados de nuestros Síntomas Tempranos. AiEstudio sobre artritis (2013-2015) y datos aportados por pares.
Concientización sobre la enfermedad de Still
Still’s Disease Awareness shines a light on this rare autoinflammatory condition, helping to educate the public, accelerate diagnosis, and empower patients living with systemic juvenile idiopathic arthritis and adult-onset Still’s disease.
September 7th is Still's Disease Awareness Day
Describe some quality or feature of the company. Write a short paragraph about it and choose an appropriate icon.
#MyStills Awareness and Educational Posters
Describe some quality or feature of the company. Write a short paragraph about it and choose an appropriate icon.
#MyStills Campaign and Patient Experience Videos
By collecting these stories, we hope to create more awareness and education about Still's Disease for patients, their support network, the public, and the medical community.
References
1) Research based on 2015
Early Symptoms Study (ESS) combined with 2019-2020 focus groups and survey participation, both conducted by the International Foundation for Autoimmune & Autoinflammatory Arthritis.
2) There is increasing evidence of atypical rashes: raised, red or purple plaques; itchy; bumps that closely resemble eczema or urticaria. Atypical rashes, in conjunction with other typical Still’s symptoms, should be tested to rule out other causes, such as urticarial vasculitis.
3) Some patients reported feeling extremely hot and flushed, without registering a fever.
4) May be cause of abdominal pain.
5) May be responsible for patient-reported “shallow breathing” or “chest pain”.
6) Serositis refers to inflammation of the serous tissues of the body, the tissues lining the lungs, heart, and the inner lining of the abdomen and organs within.
7) Medical reviewers could not confirm this symptom as related directly to Still’s Disease.
8) Haemophagocytic lymphohistiocytosis (HLH) is termed macrophage activation syndrome (MAS) when associated with rheumatic disease (secondary HLH) and triggers including malignancy and infection.
9) Some studies suggest certain cytokine profiles (interleukins 1, 18 and IL18BP) and human leukocyte antigen (HLA) genotypes may provide diagnostic clues, but no consistent results are clinically validated.
10) Rheumatoid factor can be positive in healthy people, so having a positive RF should not dictate diagnosis.