What are the other Names for this Condition? (Also known as/Synonyms)

• cGvHD (Chronic Graft Versus Host Disease)
• Chronic GvHD

What is Chronic Graft Versus Host Disease? (Definition/Background Information)

• Chronic Graft Versus Host Disease (cGvHD) is a complication that can occur after a stem cell or bone marrow transplant in which the newly transplanted donor cells attack the transplant recipient's body
• The exact cause of chronic GVHD is unknown. It likely results from a complex immune-mediated interaction between the donor and recipient cells
• Symptoms may include skin rash, mouth sores, dry eyes, liver inflammation, development of scar tissue in the skin and joints, and damage to the lungs
• Chronic GVHD is treated with prednisone or other similar anti-inflammatory or immunosuppressive medications

(Source: Chronic Graft Versus Host Disease; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

• The Chronic Graft Versus Host Disease is considered by some scientists to be a progression of the acute subtype, while others consider this a completely different entity. Chronic GvHD can occur between weeks of transplantation to years after the procedure
• Chronic Graft Versus Host Disease can affect different parts of the body, including the eyes, skin, joints, nerves, muscles, gastrointestinal tract, genito-urinary tract and lungs

Who gets Chronic Graft Versus Host Disease? (Age and Sex Distribution)

• Chronic Graft Versus Host Disease is a rare condition that may occur in individuals who have undergone allogenic stem cell or bone marrow transplantation
• The presentation of symptoms may occur any time after a transplantation procedure; from a few weeks, to even after several years
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Chronic Graft Versus Host Disease? (Predisposing Factors)

The risk factors for Chronic Graft Versus Host Disease include:

• Having undergone recent transplantation of stem cells or bone marrow from a human leukocyte antigen (HLA) mismatched related donor
• Having undergone stem cell/marrow transplant from a HLA matched unrelated donor
• Advanced age
• Having had episode(s) of the acute form of Graft Versus Host Disease

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Chronic Graft Versus Host Disease? (Etiology)

• The exact cause of Chronic Graft Versus Host Disease is unknown
• It likely results from a complex immune-mediated interaction between the donor and recipient cells

(Source: Chronic Graft Versus Host Disease; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

What are the Signs and Symptoms of Chronic Graft Versus Host Disease?

The signs and symptoms of Chronic Graft Versus Host Disease may include:

• Rashes on the skin; thickening of skin, scar tissue formation 
• White patches inside the mouth
• Mouth sores; dry mouth
• Dry eyes; changes in vision
• Inflammation of the liver; jaundice
• Swelling in the abdomen
• Problems with swallowing, including pain during swallowing
• Sensitivity to certain types of food (such as spicy foods)
• Development of scar tissue in joints
• Shortness of breath
• Burning sensation during urination; frequent urination
• Presence of blood in urine
• Vaginal dryness in women
• Erectile dysfunction in men
• Tiredness
• Muscle weakness

How is Chronic Graft Versus Host Disease Diagnosed?

Chronic Graft Versus Host Disease is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests
• Imaging studies
• Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Chronic Graft Versus Host Disease?

The complications of Chronic Graft Versus Host Disease may include:

• Severe pain, due to the condition affecting skeletal, muscular, and nervous systems
• Damaged lungs
• Weight loss
• Reduced quality of life
• Serious and life-threatening infections 

Complications may occur with or without treatment, and in some cases, due to treatment as well. This may include:

How is Chronic Graft Versus Host Disease Treated?

• The treatment for Chronic Graft Versus Host Disease may include long-term oral or intravenous immune-suppressor medications, such as steroids
• Since the immune system is suppressed with such treatments, additional medications may be necessary to prevent infections by pathogenic microbes (such as bacteria, fungi and viruses)

How can Chronic Graft Versus Host Disease be Prevented?

• Chronic Graft Versus Host Disease may be preventable through the following measures:
  • Detailed and precise matching of donor by tissue typing and HLA testing at the DNA level
  • Administration of immune-suppressive medication immediately after transplantation
• Active research is currently being performed to explore the possibilities for treatment and prevention of conditions such as Chronic Graft Versus Host Disease

Regular medical screening at periodic intervals with tests and physical examinations are recommended.

What is the Prognosis of Chronic Graft Versus Host Disease? (Outcomes/Resolutions)

• The prognosis of Chronic Graft Versus Host Disease is dependent upon the severity of the signs and symptoms and associated complications, if any
• Individuals with mild conditions have better prognosis than those with severe symptoms and complications. However, the affected individuals often experience reduced quality of life
• Since the treatment for Chronic Graft Versus Host Disease involves immune-suppressive medication, serious and life-threatening infections may occur
• Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Chronic Graft Versus Host Disease:

• Allogenic transplantation refers to cells or bone marrow from a matching donor, who may or may not be related to the recipient
• Although individuals with Chronic Graft Versus Host Disease may experience reduced quality of life, it has been reported that such an immune response may identify and destroy surviving cancer cells (also known as host versus tumor effect)

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/