Pulmonary nodular lymphoid hyperplasia

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ORPHA:60026OMIM:178610J98.4
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Overview

Pulmonary nodular lymphoid hyperplasia (PNLH) is a rare, benign condition where clusters of immune cells called lymphocytes grow and form small nodules (lumps) in the lung tissue. It is sometimes called pulmonary lymphoid hyperplasia or nodular lymphoid lesion of the lung. These nodules are not cancerous, but they can look similar to other lung conditions on scans, which makes diagnosis tricky. The condition belongs to a group of lung disorders involving abnormal but non-cancerous growth of lymphoid (immune) tissue. Many people with PNLH have no symptoms at all and the nodules are found by accident during a chest X-ray or CT scan done for another reason. When symptoms do occur, they can include a mild cough, shortness of breath, or a general feeling of chest discomfort. Some patients have an underlying autoimmune condition or immune system problem, such as Sjögren's syndrome or common variable immunodeficiency (CVID), which may be linked to the development of PNLH. Treatment depends on how severe the condition is and whether symptoms are present. Many patients are simply monitored over time with regular imaging. When treatment is needed, corticosteroids (anti-inflammatory medicines) are often used. In some cases, the nodules are surgically removed, both to confirm the diagnosis and to treat the condition. The overall outlook is generally good, especially when there is no underlying serious immune disorder. Regular follow-up with a lung specialist is important to watch for any changes.

Also known as:

Key symptoms:

Persistent dry coughShortness of breath, especially with activityMild chest discomfort or tightnessFatigue or low energyAccidental finding on chest X-ray or CT scan with no symptomsOccasional low-grade feverUnintentional weight loss in some casesReduced exercise tolerance

Clinical phenotype terms (10)— hover any for plain English
Follicular hyperplasiaHP:0002729Nodular pattern on pulmonary HRCTHP:0025392PlasmacytosisHP:0030150Mediastinal lymphadenopathyHP:0100721Ground-glass opacificationHP:0025179
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Pulmonary nodular lymphoid hyperplasia.

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No actively recruiting trials found for Pulmonary nodular lymphoid hyperplasia at this time.

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No specialists are currently listed for Pulmonary nodular lymphoid hyperplasia.

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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 Pulmonary nodular lymphoid hyperplasia.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.Is my diagnosis of PNLH confirmed by biopsy, and has lymphoma been fully ruled out?,Do I need treatment right now, or is a watch-and-wait approach appropriate for me?,Should I be tested for an underlying autoimmune condition or immune deficiency?,How often do I need follow-up CT scans, and what changes should prompt me to call you sooner?,What symptoms should make me go to the emergency room?,Are there any clinical trials or specialist centers I should consider?,Will this condition affect my ability to work or exercise long-term?

Common questions about Pulmonary nodular lymphoid hyperplasia

What is Pulmonary nodular lymphoid hyperplasia?

Pulmonary nodular lymphoid hyperplasia (PNLH) is a rare, benign condition where clusters of immune cells called lymphocytes grow and form small nodules (lumps) in the lung tissue. It is sometimes called pulmonary lymphoid hyperplasia or nodular lymphoid lesion of the lung. These nodules are not cancerous, but they can look similar to other lung conditions on scans, which makes diagnosis tricky. The condition belongs to a group of lung disorders involving abnormal but non-cancerous growth of lymphoid (immune) tissue. Many people with PNLH have no symptoms at all and the nodules are found by ac

How is Pulmonary nodular lymphoid hyperplasia inherited?

Pulmonary nodular lymphoid hyperplasia follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Pulmonary nodular lymphoid hyperplasia typically begin?

Typical onset of Pulmonary nodular lymphoid hyperplasia is adult. Age of onset can vary across affected individuals.