Rare genetic immune disease

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ORPHA:183770
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1Active trials14Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Orphanet code 183770 refers to a rare genetic immune disease that falls within the broader category of primary immunodeficiency disorders. These are conditions where the immune system does not work properly due to changes (mutations) in specific genes. Patients with this type of disorder often have trouble fighting off infections because their immune cells do not develop or function as they should. This can lead to frequent, severe, or unusual infections that healthy individuals would normally be able to handle without difficulty. Symptoms can vary widely depending on the exact genetic cause and which part of the immune system is affected. Common problems include repeated bacterial, viral, or fungal infections, poor growth in children, chronic diarrhea, skin rashes, and swollen lymph nodes. Some patients may also develop autoimmune problems, where the immune system mistakenly attacks the body's own tissues. Treatment depends on the specific type and severity of the immune defect. Options may include immunoglobulin replacement therapy (infusions of antibodies), preventive antibiotics or antifungal medications, and in some cases, hematopoietic stem cell transplantation (bone marrow transplant), which can be curative. Gene therapy is an emerging option for certain types. Early diagnosis and management by an immunology specialist are essential to reduce complications and improve quality of life.

Key symptoms:

Frequent or severe infectionsInfections that don't respond well to standard antibioticsRecurring pneumonia or lung infectionsChronic ear infectionsChronic sinus infectionsPersistent skin infections or rashesChronic diarrheaPoor weight gain or failure to thrive in childrenSwollen lymph nodes or enlarged spleenMouth sores or thrush that keeps coming backAutoimmune symptoms such as joint pain or blood cell problemsDelayed growth and developmentUnexplained fevers

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

FDA & Trial Timeline

2 events
Mar 2025Transcriptomic Analysis to Put an End to Misdiagnosis in Patients With Rare Muscle Diseases

Assistance Publique Hopitaux De Marseille — NA

TrialRECRUITING
Apr 2017Collection of Immunology Specimens From Patients With Cancer or Blood Disorders, and Healthy Volunteers

University of Southern California

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Rare genetic immune disease.

1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

1 recruitingView all trials with filters →
Other1 trial
Collection of Immunology Specimens From Patients With Cancer or Blood Disorders, and Healthy Volunteers
Actively Recruiting
PI: Casey O'Connell, MD (University of Southern California) · Sites: Los Angeles, California · Age: 1999 yrs

Specialists

14 foundView all specialists →
EM
Elizabeth M Kang, M.D.
Bethesda, Maryland
Specialist

Rare Disease Specialist

PI on 11 active trials
JD
Jaap M van Laar, Prof. dr.
Specialist
PI on 1 active trial
AS
Ann (Annie) W Silk
Los Angeles, California
Specialist

Rare Disease Specialist

PI on 1 active trial
VM
V. Koneti Rao, M.D.
Specialist
PI on 5 active trials
MP
Maria Lourdes Posadas Martinez, PhD
Specialist
PI on 1 active trial
CB
Christine BODEMER
Bobigny
Specialist

Rare Disease Specialist

PI on 2 active trials
MP
Marcelo Serra, PhD
Buenos Aires, Buenos Aires
Specialist

Rare Disease Specialist

PI on 1 active trial
LS
Lecia V Sequist
Philadelphia, Pennsylvania
Specialist

Rare Disease Specialist

PI on 1 active trial
RP
Rosanna Cardani, PhD
San Donato Milanese, Milan
Specialist

Rare Disease Specialist

PI on 1 active trial
SD
Svetlana GOROKHOVA Dr
Marseille
Specialist

Rare Disease Specialist

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 Rare genetic immune disease.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Rare genetic immune disease

Disease timeline:

New trial: Transcriptomic Analysis to Put an End to Misdiagnosis in Patients With Rare Muscle Diseases

Phase NA trial recruiting. ARN extraction from muscle biopsies

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 specific type of immune deficiency does my child or I have, and which gene is affected?,What treatments are available, and is bone marrow transplant or gene therapy an option?,How can we reduce the risk of infections in daily life?,Are there any vaccines that should be avoided or that household members should receive?,What signs of infection or complications should prompt an emergency room visit?,How often will we need follow-up appointments and blood tests?,Are there clinical trials or new therapies being studied for this condition?

Common questions about Rare genetic immune disease

What is Rare genetic immune disease?

Orphanet code 183770 refers to a rare genetic immune disease that falls within the broader category of primary immunodeficiency disorders. These are conditions where the immune system does not work properly due to changes (mutations) in specific genes. Patients with this type of disorder often have trouble fighting off infections because their immune cells do not develop or function as they should. This can lead to frequent, severe, or unusual infections that healthy individuals would normally be able to handle without difficulty. Symptoms can vary widely depending on the exact genetic cause

Are there clinical trials for Rare genetic immune disease?

Yes — 1 recruiting clinical trial is currently listed for Rare genetic immune disease on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Rare genetic immune disease?

14 specialists and care centers treating Rare genetic immune disease are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.