7 Key Symptoms of Adult Still’s Disease You Should Know

Adult Still’s disease is a rare inflammatory disorder characterized by distinctive symptoms that can initially be mistaken for other conditions. Named after English physician George Still, who first described this condition in children, Adult Still’s disease affects individuals typically between ages 16 and 35, though it can occur at any age. Understanding the characteristic symptoms is crucial for early diagnosis and proper management of this complex condition.

This systemic inflammatory disorder can affect multiple organs and systems in the body, making it challenging to diagnose. The symptoms often appear suddenly and can vary significantly from person to person. Below, we explore the seven key symptoms that characterize Adult Still’s disease.

1. High Spiking Fever

One of the most distinctive and consistent symptoms of Adult Still’s disease is a characteristic fever pattern. This isn’t just any fever—it has specific features that help distinguish it from other conditions.

Key characteristics of the fever include:

• Daily temperature spikes typically reaching 102°F (39°C) or higher
• Usually occurs once or twice daily, most commonly in the late afternoon or evening
• Temperature returns to normal or below normal between spikes
• Fever pattern typically lasts for at least two weeks
• May persist for months if left untreated

The fever often appears suddenly and can be accompanied by chills and sweating. Many patients report feeling relatively well between fever episodes, which is an important distinguishing feature. This cyclical pattern is so characteristic that it’s considered one of the major diagnostic criteria for Adult Still’s disease.

2. Salmon-Pink Rash

A distinctive skin rash is another hallmark symptom of Adult Still’s disease, appearing in approximately 85% of patients. This rash has unique characteristics that differentiate it from other skin conditions.

Distinctive features of the rash:

• Salmon-pink or peachy-orange colored flat spots or slightly raised bumps
• Typically appears on the trunk, arms, and legs
• Usually comes and goes in conjunction with fever spikes
• May be itchy but often isn’t
• Can be brought out by heat, rubbing the skin, or stress
• Individual lesions are typically transient, lasting only hours

The rash may be barely noticeable when the fever is absent and become more prominent during fever episodes. Some patients describe the rash as being more visible after a hot shower or physical activity. The fleeting nature of this rash can make it challenging to show to healthcare providers, so taking photographs when it appears can be helpful for diagnosis.

3. Joint Pain and Arthritis

Joint involvement is a prominent feature of Adult Still’s disease and can be one of the most debilitating symptoms. The arthritis associated with this condition can range from mild discomfort to severe, destructive joint disease.

Joint symptoms typically include:

• Pain, stiffness, and swelling in multiple joints
• Most commonly affects knees, wrists, ankles, elbows, shoulders, and hands
• Morning stiffness lasting an hour or more
• Symmetrical joint involvement (affecting both sides of the body)
• Joint symptoms may appear before, during, or after other symptoms

In some cases, the joint pain may be mild and transient, while in others it can be chronic and lead to permanent joint damage. The wrists and knees are particularly vulnerable to severe arthritis in Adult Still’s disease. Some patients may experience arthralgia (joint pain) without visible inflammation, while others develop obvious swelling and warmth in affected joints.

4. Sore Throat

A severe sore throat is a common early symptom of Adult Still’s disease, often appearing before other manifestations. This isn’t a typical sore throat associated with common colds or infections.

Characteristics of the sore throat include:

• Persistent severe pain that doesn’t respond to typical treatments
• Occurs in 50-75% of patients
• Usually appears early in the disease course
• Not associated with visible infection or pus
• May be accompanied by swollen lymph nodes in the neck

The sore throat can be so severe that patients have difficulty swallowing. Despite the intensity of the pain, examination of the throat typically shows only mild redness without the white patches or exudate seen in bacterial infections like strep throat. This discrepancy between symptoms and physical findings can be a clue pointing toward Adult Still’s disease rather than a common infection.

5. Muscle Pain and Weakness

Myalgia, or muscle pain, is another significant symptom experienced by many patients with Adult Still’s disease. This can substantially impact daily activities and quality of life.

Muscle-related symptoms include:

• Widespread muscle aches and tenderness
• Muscle pain often coinciding with fever episodes
• Generalized weakness and fatigue
• Difficulty performing routine physical activities
• Pain may be severe enough to limit mobility

The muscle pain in Adult Still’s disease can be diffuse, affecting large muscle groups throughout the body. Patients often describe feeling as though they have a severe flu. The intensity of muscle pain typically correlates with disease activity, becoming worse during flares and improving when the disease is better controlled.

6. Enlarged Lymph Nodes, Liver, and Spleen

Adult Still’s disease is a systemic condition that can cause enlargement of various organs and lymphatic structures. This internal involvement may not be immediately noticeable but can be detected through physical examination or imaging.

Organ involvement may include:

• Lymphadenopathy (swollen lymph nodes), especially in the neck, armpits, and groin
• Hepatomegaly (enlarged liver) occurring in about 40-65% of patients
• Splenomegaly (enlarged spleen) in approximately 25-55% of cases
• Mild elevation of liver enzymes in blood tests
• Occasionally, abdominal discomfort or feeling of fullness

These internal changes often indicate active disease and significant inflammation. While some patients may notice swollen lymph nodes themselves, liver and spleen enlargement typically requires medical examination or imaging studies to detect. Regular monitoring of these organs is important during disease management.

7. General Systemic Symptoms

Beyond the specific symptoms mentioned above, Adult Still’s disease causes a range of general symptoms that reflect the systemic nature of the inflammatory process.

These may include:

• Extreme fatigue and exhaustion
• Unintended weight loss (can be significant)
• Loss of appetite
• Night sweats (often drenching)
• Chest pain when breathing deeply (pleuritis)
• Abdominal pain
• Headaches

The fatigue associated with Adult Still’s disease can be overwhelming and is often described as more than just feeling tired—it’s a profound exhaustion that isn’t relieved by rest. Weight loss can occur due to reduced appetite, increased metabolic demands from chronic inflammation, and the overall impact of feeling unwell. Night sweats can be so severe that patients need to change their clothing or bedding multiple times per night.

Some patients may also experience chest pain due to inflammation of the lining around the lungs (pleuritis) or heart (pericarditis), though these complications are less common. Any chest pain should be evaluated promptly by a healthcare provider.

Main Causes and Risk Factors

The exact cause of Adult Still’s disease remains unknown, making it an idiopathic condition. However, researchers have identified several factors that may play a role in its development:

Potential contributing factors include:

Genetic Predisposition: While Adult Still’s disease is not directly inherited, certain genetic markers may increase susceptibility. Some studies have found associations with specific human leukocyte antigen (HLA) types, suggesting a genetic component to susceptibility.

Infectious Triggers: Various viral and bacterial infections have been proposed as potential triggers, including:

• Rubella virus
• Cytomegalovirus
• Epstein-Barr virus
• Parvovirus B19
• Certain bacterial infections

However, no single infectious agent has been conclusively proven to cause the disease. It’s believed that infections may trigger an abnormal immune response in genetically susceptible individuals.

Immune System Dysfunction: Adult Still’s disease is considered an autoinflammatory disorder, where the innate immune system becomes overactive without a clear external trigger. This results in excessive production of inflammatory proteins called cytokines, particularly interleukin-1 (IL-1), interleukin-6 (IL-6), and tumor necrosis factor (TNF).

Environmental Factors: Some researchers suggest that environmental exposures may play a role, though specific factors haven’t been identified.

Age and Gender: While not causes, certain demographic patterns exist:

• Two age peaks: 15-25 years and 36-46 years
• Slightly more common in women than men
• Occurs across all ethnic groups worldwide

It’s important to note that Adult Still’s disease is not contagious, not directly inherited, and cannot be prevented through lifestyle changes since the underlying cause is not yet fully understood.

Frequently Asked Questions

Is Adult Still’s disease the same as juvenile Still’s disease?

Adult Still’s disease and juvenile Still’s disease (also called systemic juvenile idiopathic arthritis) are very similar conditions with nearly identical symptoms. The main difference is the age of onset—juvenile Still’s disease occurs in children under 16, while Adult Still’s disease affects those 16 and older. Both are characterized by high fevers, rash, and joint inflammation.

How is Adult Still’s disease diagnosed?

There is no single test for Adult Still’s disease. Diagnosis is based on characteristic symptoms, physical examination, and exclusion of other conditions. Blood tests typically show elevated inflammatory markers, high white blood cell counts, and increased ferritin levels. The diagnosis requires the presence of specific criteria including fever, rash, and joint involvement, after ruling out infections, cancers, and other autoimmune diseases.

Is Adult Still’s disease serious?

Adult Still’s disease can range from mild to severe. While some patients experience a single episode that resolves completely, others have a chronic course with recurring flares. Potential complications can include destructive arthritis, heart inflammation, lung problems, and in rare cases, a life-threatening condition called macrophage activation syndrome. With proper medical management, most patients can achieve good disease control.

Can Adult Still’s disease go away on its own?

The disease course varies significantly among individuals. Some people experience a monophasic pattern with symptoms lasting several months then resolving completely without recurrence. However, many patients have a polycyclic pattern with alternating periods of active disease and remission, or a chronic persistent pattern. Medical treatment is typically necessary to control inflammation and prevent complications.

What should I do if I suspect I have Adult Still’s disease?

If you experience unexplained high fevers, a salmon-pink rash, severe sore throat, and joint pain, especially if these symptoms occur together, consult a healthcare provider promptly. A rheumatologist (specialist in inflammatory and autoimmune conditions) is typically best suited to evaluate and manage suspected Adult Still’s disease. Early diagnosis and treatment can help prevent complications and improve outcomes.

Can stress trigger Adult Still’s disease flares?

While stress doesn’t cause Adult Still’s disease, many patients report that physical or emotional stress can trigger disease flares or worsen symptoms. Managing stress through adequate rest, relaxation techniques, and maintaining overall health may help reduce the frequency or severity of flares, though this varies among individuals.

Is Adult Still’s disease contagious?

No, Adult Still’s disease is not contagious. It is an autoinflammatory disorder, meaning it results from an abnormal immune system response rather than an infection that can be passed from person to person. You cannot catch Adult Still’s disease from someone who has it.

References:

• Mayo Clinic – Adult Still’s Disease
• Johns Hopkins Medicine – Adult-Onset Still’s Disease
• American College of Rheumatology – Adult-Onset Still’s Disease
• National Organization for Rare Disorders (NORD) – Still Disease
• National Center for Biotechnology Information – Adult-Onset Still Disease