Pediatric Primary Immune Regulatory Disorder (PIRD) Care

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Tailored care for primary immune regulatory disorder (PIRD)
Primary immune regulatory disorder (PIRD) is a name for a group of rare immune system disorders with a genetic cause. Symptoms of PIRD usually appear within the first few years of life and include hyperinflammation (overactive immune system), severe allergies and autoimmune conditions. PIRD can be difficult to treat.
Targeted PIRD testing and therapies
Children with PIRD have an immune system that may be overactive, underactive or doesn't respond as it should to challenges.
Many genetic mutations can lead to PIRD. Our specialists use genetic testing to determine the underlying cause of the condition. Once they have the right diagnosis, they design a treatment plan for your child that targets the immune system issue.
For example, a genetic test may show a defect in a gene that makes a specific protein that plays an important role in regulating the immune system. Our team can then treat the child with a drug that delivers this vital protein.
Stem cell transplant
For some patients, the best treatment is a stem cell transplant (also known as bone marrow transplant or BMT). In these cases, we collaborate with our Pediatric Bone Marrow Transplant (BMT), Cellular & Gene Therapy team to provide seamless, comprehensive care.
Tailored care for primary immune regulatory disorder (PIRD)
Primary immune regulatory disorder (PIRD) is a name for a group of rare immune system disorders with a genetic cause. Symptoms of PIRD usually appear within the first few years of life and include hyperinflammation (overactive immune system), severe allergies and autoimmune conditions. PIRD can be difficult to treat.
Targeted PIRD testing and therapies
Children with PIRD have an immune system that may be overactive, underactive or doesn't respond as it should to challenges.
Many genetic mutations can lead to PIRD. Our specialists use genetic testing to determine the underlying cause of the condition. Once they have the right diagnosis, they design a treatment plan for your child that targets the immune system issue.
For example, a genetic test may show a defect in a gene that makes a specific protein that plays an important role in regulating the immune system. Our team can then treat the child with a drug that delivers this vital protein.
Stem cell transplant
For some patients, the best treatment is a stem cell transplant (also known as bone marrow transplant or BMT). In these cases, we collaborate with our Pediatric Bone Marrow Transplant (BMT), Cellular & Gene Therapy team to provide seamless, comprehensive care.
Providers

Morna J. Dorsey, MD, MS
Pediatric Allergy and Immunology
Kristin A. Shimano, MD
Pediatric Hematology-Oncology
Sofia Verstraete, MD
Pediatric Gastroenterology
Emily Von Scheven, MD
Rheumatology • Pediatric Rheumatology
Awards & achievements
Related conditions & treatments
Conditions
- Allergic Disorders
- Autoimmune Diseases
- Genetic Disorders
- Immune Dysregulation
- Ipex Syndrome
Treatments
- Bone Marrow Transplant
- Genetic Testing
- Stem Cell Transplant
Clinical trials
A Study of Treatment of Inflammation Before Stem Cell Transplant in People With a Primary Immune Regulatory Disorder (PIRD) and/or an Autoinflammatory ConditionOpens in a new window
The researchers are doing this study to find out whether emapalumab or a combination of fludarabine and dexamethasone are effective in preparing people with a primary immune regulatory disorder (PIRD) and/or an autoinflammatory condition to receive a stem cell transplant...
















