Aseptic abscess syndrome

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Overview

Aseptic abscess syndrome is a rare inflammatory disease where the body forms pus-filled pockets (abscesses) in organs and tissues — but unlike typical abscesses, these are NOT caused by a bacterial or fungal infection. The word 'aseptic' means 'without infection.' The immune system becomes overactive and attacks the body's own tissues, creating these sterile (germ-free) collections of fluid and white blood cells. The condition is also sometimes called 'sterile abscess syndrome.' The disease most commonly affects the spleen, liver, and lymph nodes, but abscesses can also appear in the skin, muscles, bones, and lungs. Many patients also have an underlying inflammatory bowel disease, most often Crohn's disease, though the syndrome can occur on its own. Patients typically experience recurring fevers, pain in the abdomen, and general feelings of being very unwell. Blood tests often show signs of severe inflammation. Treatment focuses on calming the overactive immune system. Corticosteroids like prednisone are usually the first choice and often work well. For patients who do not respond or who relapse, stronger immune-suppressing medicines — including drugs used in inflammatory bowel disease — may be needed. The condition tends to be chronic, meaning it can come back, but many patients achieve good control with the right treatment.

Also known as:

Key symptoms:

Recurring high feversPain or tenderness in the abdomenEnlarged spleen or liverSwollen lymph nodesSkin abscesses or painful lumps under the skinExtreme tiredness and fatigueUnintended weight lossNight sweatsMuscle or joint painDiarrhea or other bowel symptoms (especially if Crohn's disease is also present)Feeling generally very unwell for long periods

Clinical phenotype terms (17)— hover any for plain English
Abnormal lymph node morphologyHP:0002733Increased total neutrophil countHP:0011897Liver abscessHP:0100523Abnormality of the pancreasHP:0001732Abnormal lung morphologyHP:0002088Brain abscessHP:0030049Abnormality of the lymphatic systemHP:0100763
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Aseptic abscess syndrome.

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No actively recruiting trials found for Aseptic abscess syndrome at this time.

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No specialists are currently listed for Aseptic abscess syndrome.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Aseptic abscess syndrome.

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Community

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Latest news about Aseptic abscess syndrome

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Questions for your doctor

Bring these to your next appointment

  • Q1.How certain are you of this diagnosis, and what other conditions have been ruled out?,Do I need a biopsy of the abscess, and what will it tell us?,Should I be tested for inflammatory bowel disease like Crohn's disease?,What is the plan if corticosteroids do not work or if my disease comes back?,How long will I need to stay on immune-suppressing medication?,What warning signs should make me go to the emergency room?,Are there any clinical trials or specialist centers I should be referred to?

Common questions about Aseptic abscess syndrome

What is Aseptic abscess syndrome?

Aseptic abscess syndrome is a rare inflammatory disease where the body forms pus-filled pockets (abscesses) in organs and tissues — but unlike typical abscesses, these are NOT caused by a bacterial or fungal infection. The word 'aseptic' means 'without infection.' The immune system becomes overactive and attacks the body's own tissues, creating these sterile (germ-free) collections of fluid and white blood cells. The condition is also sometimes called 'sterile abscess syndrome.' The disease most commonly affects the spleen, liver, and lymph nodes, but abscesses can also appear in the skin, mu

How is Aseptic abscess syndrome inherited?

Aseptic abscess syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Aseptic abscess syndrome typically begin?

Typical onset of Aseptic abscess syndrome is adult. Age of onset can vary across affected individuals.