Overview
Acute interstitial pneumonia (AIP), also known as Hamman-Rich syndrome, is a rare and serious lung disease that causes sudden, severe inflammation and scarring deep inside the lungs. Unlike many lung conditions that develop slowly over months or years, AIP comes on very quickly — often within days to weeks — and can rapidly make breathing very difficult. The disease affects the tiny air sacs in the lungs (called alveoli) and the tissue surrounding them, causing them to become inflamed, filled with fluid, and eventually scarred. This makes it very hard for oxygen to pass from the lungs into the bloodstream. The most common symptoms include severe shortness of breath, a dry cough, and low oxygen levels in the blood. Many people with AIP need to be placed on a breathing machine (mechanical ventilator) in an intensive care unit. The exact cause of AIP is not fully understood, and it does not appear to be caused by a known infection, medication, or other identifiable trigger — which makes it especially challenging to diagnose and treat. Treatment typically involves high-dose corticosteroids (like methylprednisolone) to reduce inflammation, along with supportive care in a hospital setting. Unfortunately, AIP has a high mortality rate, and survivors may be left with lasting lung damage. Early recognition and aggressive medical support are critical to improving outcomes.
Also known as:
Key symptoms:
Sudden, severe shortness of breathDry coughFeverLow oxygen levels in the bloodRapid breathingFeeling very tired or weakBluish tint to the lips or fingertips (from low oxygen)Chest tightness or discomfortCrackling sounds in the lungs heard through a stethoscopeConfusion or difficulty thinking clearly due to low oxygen
Clinical phenotype terms (33)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
4 eventsUniversity of California, Los Angeles — NA
University Magna Graecia
Vanderbilt University Medical Center
North East Japan Study Group
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Acute interstitial pneumonia.
2 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Acute interstitial pneumonia.
Community
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Start the conversation →Latest news about Acute interstitial pneumonia
1 articlesCaregiver Resources
NORD Caregiver Resources
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Mental Health Support
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What caused my AIP, and is there anything that could have triggered it?,What does my CT scan and biopsy show, and how severe is the lung damage?,What is the treatment plan, and how will we know if it is working?,What are the chances of recovery, and what long-term lung problems should I expect?,Is there a risk that AIP could come back, and what warning signs should I watch for?,Should I be referred to a pulmonary rehabilitation program after I leave the hospital?,Are there any clinical trials I might be eligible for?
Common questions about Acute interstitial pneumonia
What is Acute interstitial pneumonia?
Acute interstitial pneumonia (AIP), also known as Hamman-Rich syndrome, is a rare and serious lung disease that causes sudden, severe inflammation and scarring deep inside the lungs. Unlike many lung conditions that develop slowly over months or years, AIP comes on very quickly — often within days to weeks — and can rapidly make breathing very difficult. The disease affects the tiny air sacs in the lungs (called alveoli) and the tissue surrounding them, causing them to become inflamed, filled with fluid, and eventually scarred. This makes it very hard for oxygen to pass from the lungs into the
How is Acute interstitial pneumonia inherited?
Acute interstitial pneumonia follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Acute interstitial pneumonia typically begin?
Typical onset of Acute interstitial pneumonia is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Acute interstitial pneumonia?
Yes — 2 recruiting clinical trials are currently listed for Acute interstitial pneumonia on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Acute interstitial pneumonia?
25 specialists and care centers treating Acute interstitial pneumonia are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.