Overview
Lymphoid interstitial pneumonia (LIP), also sometimes called lymphocytic interstitial pneumonia or lymphoid interstitial pneumonitis, is a rare lung disease where certain immune cells called lymphocytes build up in the walls of the air sacs (alveoli) in the lungs. This buildup causes the lung tissue to become inflamed and, over time, can make it harder for the lungs to work properly. LIP is considered a type of interstitial lung disease, meaning it affects the tissue and space around the air sacs rather than the airways themselves. The most common symptoms include a dry cough that does not go away, shortness of breath especially during activity, and fatigue. Some people also develop fever, unintended weight loss, and chest discomfort. LIP is often linked to other conditions, especially autoimmune diseases like Sjögren's syndrome, HIV infection (particularly in children), and certain immune deficiency disorders. In some cases, no underlying cause is found, and it is called idiopathic LIP. Treatment usually involves corticosteroids like prednisone to reduce inflammation in the lungs. Some people respond well and see significant improvement, while others may have a more persistent course. When LIP is linked to an underlying condition, treating that condition is also an important part of care. Regular monitoring by a lung specialist is essential to track how the disease is progressing and adjust treatment as needed.
Also known as:
Key symptoms:
Persistent dry coughShortness of breath, especially with activityFatigue and low energyUnintended weight lossFeverChest discomfort or tightnessClubbing of the fingers (fingertips become rounded and enlarged)Night sweatsDifficulty exercising or doing physical activitiesLow oxygen levels in the blood
Clinical phenotype terms (34)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
4 eventsImplantology Institute
National Cancer Institute (NCI) — PHASE2, PHASE3
UNC Lineberger Comprehensive Cancer Center — PHASE2
Thomas Jefferson University — PHASE2
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
1 availableNeutrexin
Concurrent leucovorin administration as an alternative therapy for the treatment of moderate to severe PCP in immunocompromised patients who are intolerant of, or are refractory to, trimethoprim sulfa…
Concurrent leucovorin administration as an alternative therapy for the treatment of moderate to severe PCP in immunocompromised patients who are intolerant of, or are refractory to, trimethoprim sulfamethoxazole
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 Lymphoid interstitial pneumonia.
Community
No community posts yet. Be the first to share your experience with Lymphoid interstitial pneumonia.
Start the conversation →Latest news about Lymphoid interstitial pneumonia
Disease timeline:
New recruiting trial: 3D Evaluation of the Lip Support in a Full-arch Implant-supported Rehabilitations
A new clinical trial is recruiting patients for Lymphoid interstitial pneumonia
New recruiting trial: Radiation Therapy With or Without Cisplatin in Treating Patients With Stage III-IVA Squamous Cell Carcinoma of the Head and Neck Who Have Undergone Surgery
A new clinical trial is recruiting patients for Lymphoid interstitial pneumonia
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
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 is causing my LIP — is there an underlying condition we should be treating?,How will we know if the treatment is working, and how long will I need to take corticosteroids?,What are the risks of long-term corticosteroid use and how can we minimize them?,Should I be tested for autoimmune diseases or immune deficiencies?,What signs should prompt me to seek emergency care?,Is there a risk that my LIP could progress to lung scarring or lymphoma, and how will we monitor for that?,Are there any clinical trials I might be eligible for?
Common questions about Lymphoid interstitial pneumonia
What is Lymphoid interstitial pneumonia?
Lymphoid interstitial pneumonia (LIP), also sometimes called lymphocytic interstitial pneumonia or lymphoid interstitial pneumonitis, is a rare lung disease where certain immune cells called lymphocytes build up in the walls of the air sacs (alveoli) in the lungs. This buildup causes the lung tissue to become inflamed and, over time, can make it harder for the lungs to work properly. LIP is considered a type of interstitial lung disease, meaning it affects the tissue and space around the air sacs rather than the airways themselves. The most common symptoms include a dry cough that does not go
How is Lymphoid interstitial pneumonia inherited?
Lymphoid interstitial pneumonia follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Are there clinical trials for Lymphoid interstitial pneumonia?
Yes — 3 recruiting clinical trials are currently listed for Lymphoid 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 Lymphoid interstitial pneumonia?
25 specialists and care centers treating Lymphoid interstitial pneumonia are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.
What treatment and support options exist for Lymphoid interstitial pneumonia?
1 patient support program are currently tracked on UniteRare for Lymphoid interstitial pneumonia. See the treatments and support programs sections for copay assistance, eligibility, and contact details.