adult-onset Still's disease

What is Adult-Onset Still's Disease?

Adult-onset Still's disease (AOSD) is a rare and complex inflammatory disorder that affects multiple organs in the body. It is characterized by high fevers, joint pain, and a distinctive salmon-colored rash.

Key Features of AOSD:

• Age: AOSD typically affects young adults under the age of 35.
• Symptoms: High fevers, joint pain (polyarthritis), and a transient salmon-pink maculopapular rash are common symptoms. [10][11]
• Complications: If left untreated, AOSD can lead to long-term arthritis, organ damage, and other complications. [8]

Causes and Risk Factors:

While the exact cause of AOSD is unknown, it is believed to be an autoimmune disorder, where the body's immune system mistakenly attacks its own tissues. Age is a significant risk factor, with most cases occurring in young adults. Males and females are equally at risk. [1]

Diagnosis and Treatment:

AOSD can be challenging to diagnose due to its rarity and similarity to other conditions. However, diagnosis is often made based on clinical presentation, laboratory tests (e.g., elevated ferritin levels), and exclusion of other possible causes. Treatment typically involves anti-inflammatory medications, corticosteroids, and immunosuppressive agents to manage symptoms and prevent complications.

References:

• [1] Adult-onset Still disease (AOSD) is a rare systemic inflammatory disorder characterized by daily fever, inflammatory polyarthritis, and a transient salmon-pink maculopapular rash. Initially documented in children by George Still in 1896...
• [8] Adult Still disease (ASD) is a rare illness that causes high fevers, rash, and joint pain. It may lead to long-term (chronic) arthritis.
• [10] Adult-onset Still disease (AOSD) is a rare systemic inflammatory disorder characterized by daily fever, inflammatory polyarthritis, and a transient salmon-pink maculopapular rash. Initially documented in children by George Still in 1896...
• [11] Adult-onset Still's disease (AOSD) is a form of Still's disease, a rare systemic autoinflammatory disease characterized by the classic triad of fevers, joint pain, and a distinctive salmon-colored bumpy rash...

Adult-onset Still's disease (AOSD) is a rare inflammatory disorder that can cause a variety of systemic symptoms. The most common signs and symptoms of AOSD include:

• Fever: High, spiking fevers are a hallmark symptom of AOSD, often reaching temperatures of at least 102 degrees Fahrenheit (38.9 degrees Celsius). These fevers may spike once or twice a day for a week or longer [1].
• Rash: A pink or salmon-colored rash is common in people with AOSD, typically appearing on the trunk, arms, or legs [2-4]. The rash can be transient and may not always be present.
• Joint pain: Joint pain and inflammation are common symptoms of AOSD, often affecting multiple joints at once [5-7].
• Muscle pain: Muscle pain is another frequent symptom of AOSD, which can range from mild to severe [8].
• Sore throat: Many people with AOSD experience a sore throat, which can be accompanied by other symptoms like fever and rash [9].
• Enlarged spleen and lymph nodes: In some cases, AOSD can cause the spleen and lymph nodes to become enlarged, leading to additional symptoms like fatigue and weight loss [10-12].

It's worth noting that not everyone with AOSD will experience all of these symptoms, and the severity of each symptom can vary widely from person to person. If you're experiencing any of these symptoms, it's essential to consult a healthcare professional for proper diagnosis and treatment.

References: [1] Macovei LA, et al. Adult-onset Still's Disease: A complex disease, a challenging treatment. International Journal of Rheumatic Diseases. [2] Adult-onset Still’s disease is a rare type of inflammatory arthritis that develops unexpectedly in early adulthood. It can cause a variety of systemic symptoms, most often muscle pain, a fever and a rash. [3] Adult-onset Still’s disease (AOSD) is a rare inflammatory disorder that can affect the entire body (systemic disease). The cause of the disorder is unknown (idiopathic). [4] Almost all people with the disease will have fever, joint pain, sore throat, and a rash. Joint pain, warmth, and swelling are common. [5] Symptoms usually begin with a high fever that spikes once or twice a day and a salmon-pink rash on the trunk, arms or legs. Other symptoms include sore throat ... [6] Both the adult form and the juvenile form of Still's disease are marked by fatigue, fever, joint pain, and high levels of ferritin. [7] Adult-onset Still disease (AOSD) is an inflammatory condition that affects multiple organs. The most common symptoms are high fevers, skin rash, arthritis, and high levels of ferritin. [8] Adult-onset Still’s disease is an inflammatory condition with swelling of joints, organs, and lymph nodes. Learn about symptoms, risk factors, and treatments. [9] Adult-onset Still disease (AOSD) is a rare systemic inflammatory disorder characterized by daily fever, inflammatory polyarthritis, and a transient salmon-pink maculopapular rash. [10] Adult-onset Still’s disease (AOSD) is a rare multi-system autoinflammatory disorder that typically affects young adults. The clinical course varies and can be monocyclic, polycyclic, or chronic with either systemic or articular symptoms predominant. [11] Adult-onset Still disease (AOSD) is an inflammatory condition that affects multiple organs. The most common symptoms are high fevers, skin rash, arthritis, and high levels of ferritin. [12] by P Efthimiou · 2006 · Cited by 643 — Clinical manifestations · AOSD typically manifests as a triad of symptoms that include high‐spiking fevers, a characteristic rash, and arthritis/arthralgias.

Diagnostic Tests for Adult-Onset Still's Disease

Adult-onset Still's disease (AOSD) is a rare and complex condition that can be challenging to diagnose. While there is no single test that can definitively diagnose AOSD, various diagnostic tests can help rule out other conditions with similar symptoms.

• Imaging Tests: Imaging tests such as X-rays, CT scans, or MRI can reveal damage caused by the disease, but they are not specific for AOSD [1].
• Blood Tests: Blood tests can help identify inflammation and rule out other conditions. The following blood tests may be helpful in diagnosing AOSD:
  • Complete Blood Count (CBC): May show a high number of white blood cells [6].
  • Erythrocyte Sedimentation Rate (ESR) and C-reactive protein (CRP): Typically show high levels of inflammation [8, 9].
• Diagnostic Criteria: The diagnostic criteria for AOSD include:
  • Fever ≥39°C lasting ≥1 week
  • Arthralgias or arthritis lasting ≥2 weeks
  • Nonpruritic macular or papular rash [7]
• Serum Ferritin: Serum ferritin has gained attention as both a diagnostic test for AOSD and a marker of disease activity. In AOSD, ferritin is an acute phase reactant that can be elevated [12].

It's essential to note that the diagnosis of AOSD requires a combination of clinical features, laboratory findings, and exclusion of other conditions with similar symptoms. There is no definitive test for AOSD, and the diagnosis is often challenging [11, 15].

Treatment Options for Adult-Onset Still's Disease

Adult-onset Still's disease (AOSD) is a rare and complex condition that requires prompt and effective treatment to manage its symptoms and prevent complications. While there is no cure for AOSD, various medications can help alleviate the symptoms and improve quality of life.

First-Line Treatment

The first line of treatment for AOSD typically involves:

• Glucocorticoids (GCs): Steroids such as prednisolone are commonly used to reduce inflammation and manage symptoms. They may be given in high doses, known as pulses, for more severe cases.
• Non-steroidal anti-inflammatory drugs (NSAIDs): Medications like ibuprofen or naproxen can help alleviate pain, fever, and inflammation.

Second-Line Treatment

For patients who do not respond to first-line treatment or experience a relapse, second-line medications may be considered:

• Biologics: Infliximab, etanercept, and other biologic agents have been used to treat AOSD in some cases. These medications can help reduce inflammation and prevent complications.
• Canakinumab (Ilaris): This medication has been FDA-approved for the treatment of active Still's disease, including AOSD.

Treatment Goals

The primary goals of treating AOSD are to:

• Manage symptoms and improve quality of life
• Prevent complications such as osteoporosis, diabetes, and infections
• Reduce inflammation and prevent organ damage

Individualized Treatment Plans

It is essential to note that each patient's response to treatment may vary. Treatment plans should be tailored to individual needs and may involve a combination of medications.

References:

[3] The U.S. Food and Drug Administration today approved Ilaris (canakinumab) injection for the treatment of Active Still’s disease, including Adult-Onset Still’s Disease (AOSD).

[5] Anti-inflammatory drugs form the cornerstone of treatment of AOSD. Treatment is often based on expert opinion through the lack of international guidelines.

[12] Glucocorticoids and non-steroidal anti-inflammatory drugs (NSAIDs) represent the first line of therapy for AOSD, with add-on therapy with second-line drug reserved to steroid-dependent patients.

[13] ILARIS is a once-a-month treatment for a rare autoinflammatory condition called Still’s disease, which is referred to as Adult-Onset Still’s disease (AOSD) in adults and Systemic Juvenile Idiopathic Arthritis (SJIA) in children.

Adult-Onset Still's Disease (AOSD) Differential Diagnosis

Adult-onset Still's disease (AOSD) is a rare multisystem inflammatory disorder that can be challenging to diagnose due to its rarity and overlapping symptoms with other diseases. The differential diagnosis of AOSD encompasses various conditions, including:

• Infections: Endocarditis, occult infections, secondary syphilis, viral rash [3]
• Malignancies: Lymphoma [3]
• Systemic autoimmune rheumatic diseases: Other fevers of 'unknown origin' [6]
• Other differential diagnoses: Such as drug reactions and systemic autoimmune rheumatic diseases [2]

Key Diagnostic Features

To differentiate AOSD from other conditions, the following features are crucial:

• Fever: Fever ≥39°C lasting ≥1 week [7]
• Arthralgias or arthritis: Lasting ≥2 weeks [7]
• Nonpruritic macular or maculopapular skin rash: Salmon-colored in some cases [7]

Diagnostic Tools

Various diagnostic tools can aid in the differential diagnosis of AOSD, including:

• FDG PET/CT: Used for differential diagnosis of Adult-onset Still's disease and other connective tissue diseases [9]
• Laboratory tests: Such as neutrophilic leukocytosis (> 80% polymorphonuclear cells) to differentiate AOSD from other fevers of 'unknown origin' [6]

References

[1] by J Bhargava · 2022 · Cited by 9 [2] Sep 3, 2024 [3] Differential diagnoses include infections (endocarditis, occult infections, secondary syphilis, viral rash), malignancies (lymphoma) or autoimmune diseases ( ... [6] by M Gerfaud-Valentin · 2014 · Cited by 626 [7] 1.Fever ≥39°C lasting ≥1 week? · 2.Arthralgias or arthritis lasting ≥2 weeks? · 3.A nonpruritic macular or maculopapular skin rash that is salmon-colored in ... [8] Jun 24, 2020 [9] by X Zhou · 2019