Overview
Pulmonary interstitial glycogenosis (PIG) is a rare infantile interstitial lung disease characterized by the accumulation of glycogen-laden mesenchymal cells within the pulmonary interstitium (the tissue surrounding the air sacs of the lungs). This condition primarily affects neonates and young infants, typically presenting within the first weeks to months of life with respiratory distress, tachypnea (rapid breathing), hypoxemia (low blood oxygen levels), and sometimes failure to thrive. PIG can occur as an isolated (diffuse) condition or in association with other lung developmental abnormalities such as pulmonary growth abnormalities, congenital heart disease, or other congenital malformations. The diagnosis of PIG is established through lung biopsy, which reveals the characteristic expansion of the alveolar interstitium by immature mesenchymal cells containing abundant cytoplasmic glycogen, confirmed by periodic acid-Schiff (PAS) staining. High-resolution computed tomography (CT) of the chest may show ground-glass opacities and hyperinflation. The condition is thought to represent a disorder of lung mesenchymal cell maturation rather than a storage disease. The prognosis for isolated PIG is generally favorable, with many infants showing gradual clinical improvement over months. Treatment is primarily supportive and may include supplemental oxygen, nutritional support, and in some cases, corticosteroid therapy, which has been reported to accelerate clinical improvement in certain patients. The prognosis may be less favorable when PIG occurs in association with other lung growth abnormalities or congenital anomalies. Long-term outcomes are still being studied given the rarity of the condition.
Also known as:
Sporadic
Usually appears on its own, not inherited from a parent
Neonatal
Begins at or shortly after birth (first 4 weeks)
FDA & Trial Timeline
10 eventsStudio Odontoiatrico Associato Dr. P. Cicchese e L. Canullo — NA
Universidade do Porto — NA
Otsuka Pharmaceutical Factory, Inc. — PHASE1, PHASE2
ConvaTec Inc. — NA
Shupei Li — NA
ConvaTec Inc. — NA
Erasmus Medical Center
Reprise Biomedical, Inc. — NA
ConvaTec Inc.
Aristotle University Of Thessaloniki — PHASE2
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Pulmonary interstitial glycogenosis.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Pulmonary interstitial glycogenosis at this time.
New trials open frequently. Follow this disease to get notified.
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 Pulmonary interstitial glycogenosis.
Community
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Start the conversation →Latest news about Pulmonary interstitial glycogenosis
Disease timeline:
New recruiting trial: Double Pig Tail Stent-assisted Fixation of Fully Covered Metal Stents for the Treatment of Benign Duodenal Strictures
A new clinical trial is recruiting patients for Pulmonary interstitial glycogenosis
New recruiting trial: INNOVEN: Efficacy of Porcine Placental Extracellular Matrix Plus Standard of Care (SOC) Versus SOC Alone
A new clinical trial is recruiting patients for Pulmonary interstitial glycogenosis
New recruiting trial: The Evaluation of Training an Emergency Reflex Action Drill in the Emergency Surgical Airway in Dutch Paramedics
A new clinical trial is recruiting patients for Pulmonary interstitial glycogenosis
New recruiting trial: A Study to Investigate Safety and Effectiveness of Porcine Pancreatic Cells (OPF-310) in Patients With Type 1 Diabetes Mellitus
A new clinical trial is recruiting patients for Pulmonary interstitial glycogenosis
New recruiting trial: IDEAL: Efficacy of Porcine Placental Extracellular Matrix Augmented Plus Standard of Care (SOC) Versus SOC Alone for the Management of Diabetic Foot Ulcers
A new clinical trial is recruiting patients for Pulmonary interstitial glycogenosis
New recruiting trial: The Use of a Porcine Collagen Matrix for the Prevention of Buccal Bone Wall Resorption During Implant Placement in the Aesthetic Zone.
A new clinical trial is recruiting patients for Pulmonary interstitial glycogenosis
New recruiting trial: Study of Miro3D Wound Matrix for Healing Wounds and Ulcers in Outpatient Care
A new clinical trial is recruiting patients for Pulmonary interstitial glycogenosis
New recruiting trial: Surgical Skill Labs for Robotic Mastectomy and Educational Program Using a Surgical Guide by Artificial Intelligence
A new clinical trial is recruiting patients for Pulmonary interstitial glycogenosis
New recruiting trial: Spacer Graft Study
A new clinical trial is recruiting patients for Pulmonary interstitial glycogenosis
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Common questions about Pulmonary interstitial glycogenosis
What is Pulmonary interstitial glycogenosis?
Pulmonary interstitial glycogenosis (PIG) is a rare infantile interstitial lung disease characterized by the accumulation of glycogen-laden mesenchymal cells within the pulmonary interstitium (the tissue surrounding the air sacs of the lungs). This condition primarily affects neonates and young infants, typically presenting within the first weeks to months of life with respiratory distress, tachypnea (rapid breathing), hypoxemia (low blood oxygen levels), and sometimes failure to thrive. PIG can occur as an isolated (diffuse) condition or in association with other lung developmental abnormalit
How is Pulmonary interstitial glycogenosis inherited?
Pulmonary interstitial glycogenosis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Pulmonary interstitial glycogenosis typically begin?
Typical onset of Pulmonary interstitial glycogenosis is neonatal. Age of onset can vary across affected individuals.
Which specialists treat Pulmonary interstitial glycogenosis?
6 specialists and care centers treating Pulmonary interstitial glycogenosis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.