Chronic Graft Versus Host Disease: Difference between revisions

m (cGVHD is a disabling disease which can affect upto 80% of the HSCT recepients and the musculoskeletal manifestations can impact patients upto 2 decades.)
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What is cGVHD?
<div class="editorbox"> '''Original Editor '''- [[User:Jaleel Mohammed|Jaleel Mohammed]] '''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}}</div>
==Introduction==
[[File:Bone Marrow Transplant.png|399x399px|alt=|thumb|Bone Marrow Transplant]]Graft versus host disease (GvHD) is a very common complication of [[Bone Marrow|bone marrow]] transplantations ( also known as allogeneic haematopoietic stem cell transplantation). Anti-rejection drugs have decreased the incidence, however it still occurs regularly.<ref name=":0">Radiopedia [https://radiopaedia.org/articles/graft-versus-host-disease Graft versus host disease] Available: https://radiopaedia.org/articles/graft-versus-host-disease<nowiki/>(accessed 28.1.2023)</ref>


Chronic graft-versus-host disease (cGVHD) is a common and potentially life-threatening complication which develops as a result of allogeneic hematopoietic cell transplantation (HCT),  when the transplanted cells react against the body of the recipient. The prevalence of this disease, unfortunately, varies from between 25–80% in long-term survivors. cGVHD can lead to serious physical and functional impairment that affects the quality of life as it is often diagnosed late in its course when the disability has already begun.
GvHD cause significant damage to the tissues affected, directly contributing to functional impairment. Treatment typically involves high-dose [[Corticosteroid Medication|corticosteroids]] to suppress the [[Immune System|immune system]] which can be destructive. [[Rehabilitation Contexts|Rehabilitation]] often is required to restore function and [[Quality of Life|quality of life]].<ref>Smith SR, Asher A. [https://www.sciencedirect.com/science/article/abs/pii/S1047965116300717?via%3Dihub Rehabilitation in chronic graft-versus-host disease.] Physical Medicine and Rehabilitation Clinics. 2017 Feb 1;28(1):143-51.Available: https://www.sciencedirect.com/science/article/abs/pii/S1047965116300717?via%3Dihub (accessed 29.1.2023)</ref>


'''What are the Signs and Symptoms of GVHD?'''
== Pathology ==
'''Acute GVHD:''' In the early weeks and months after your transplant (usually within the first four months) you might notice a rash, or an upset stomach with nausea, vomiting or diarrhoea, or it could affect your liver tests, sometimes causing jaundice (yellow discolouration to the skin).  
Graft versus host disease presents either early/acute (<100 days) or late/chronic (>100 days) post bone marrow transplantation, being a major complication. The skin, gastrointestinal tract (in particular the small bowel), and liver are the organs most often affected.<ref name=":0" />


'''Chronic GVHD:'''  Can happen a few months after transplant and can reoccur for several years after transplant. Like acute GVHD, it may affect your skin, gut, liver or mouth. But it can also affect other parts of your body, such as your eyes, lungs, vagina and joints. Chronic GVHD may be mild or severe, and for some people can go on for several months or even years.
==How is GVHD Diagnosed?==


A clinical diagnosis is usually made.  Typically diagnosis includes the following:


'''What are the clinical manifestations for cGVHD?'''
# Immunologically competent cells present in the graft.
cGVHD can effect basically any organs, with  the most commonly affected sites being: the skin, the mouth, the eyes, the gastrointestinal tract, the muscles and joints, the lungs, the liver, and genitalia. From a musculoskeletal point of view, the main concern for the Allied Health Professionals are:
# The recipient has transplantation alloantigens appearing foreign to the graft and hence stimulate it antigenically.
SKIN
# The recipient cannot mount an effective immunologic reaction against the graft, or enough time is allowed for the graft to manifest an immune response.<ref name=":1">Vaillant AA, Modi P, Mohammadi O. [https://www.ncbi.nlm.nih.gov/books/NBK538235/ Graft versus host disease.] InStatPearls [Internet] 2022 Jul 8. StatPearls Publishing.Available:https://www.ncbi.nlm.nih.gov/books/NBK538235/ (accessed 28.1.2023)</ref>
FASCIA
PERIPHERAL NERVES
MUSCLE
BONE (Due to use of cortisone)
Manifestations typically appear within the first year after HCT. The most commonly affected joints include fingers, wrists, elbows, shoulders, ankles, and hips, with the distal joints often affected first (anecdotal evidence). cGVHD is an independent risk factor for joint destruction and associated pain and dysfunction.


'''What is the potential impact of musculoskeletal cGVHD on the Quality of Life (QoL) of the patients?'''
== Treatment ==
The number of long-term survivors after allogeneic HCT has been increasing over the past years, and quality of life (QOL) has become an important end-point. The QoL in these patient group can be affected due to:
Treatment for GVHD is determined by the severity of symptoms and organs involved. Treatment options are based on immunosuppression with corticosteroids being the most commonly used treatment.<ref name=":1" />


Decreased range of motion, significantly restricting a patient’s ability to perform activities of daily living.
==What Are the Clinical Manifestations for cGVHD?==
Painful joint contractures in some patients resulting in impaired function.  
[[File:CGVHD.jpg|thumb|214x214px|Hand GVHD with Sclerosis]]Acute GVHD typically involves the skin, gastrointestinal tract, and liver. It may involve the lungs, kidneys, eyes, and hematopoietic system. Decreased responsiveness to active immunization may also occur.
Chronic graft versus host disease is an independent risk factor for joint destruction and associated pain and dysfunction.
Some patients also show deteriorated role functioning and global QOL, increased fatigue, and skin problems.
The long-term sexual complications include decreased libido, vaginal alterations, erectile and ejaculatory dysfunctions.
Due to physical dysfunction, many patients also struggle to return to work post cGVHD.


'''How is GVHD diagnosed?'''
* Skin manifestation commonly arise as pruritic or painful maculopapular rash, first involving the palms, soles shoulders, and nape of the neck.
‘’Some GVHD may be a good thing because it means that your new immune system is working and is likely to be attacking any remaining disease cells. This can help stop the disease from coming back. You might hear this called graft versus tumour effect’’.   
* GI symptoms usually include diarrhea and abdominal pain, but mucositis, mucosal ulceration, nausea, and vomiting. Liver involvement usually include abnormal liver function tests with elevated bilirubin and alkaline phosphatase levels.   


The early symptoms of GVHD are often the same as some side effects and complications after a transplant, so diagnosing GVHD can be hard. Although not definite some early signs might be clawing of the fingers in hand, reduce in ankle movement, skin changes and pain in the joints. Diagnosis is often made from your symptoms, as well as from the results of laboratory tests and tissue samples.
Chronic GVHD has a presentation similar to collagen vascular disorders and systemic sclerosis. Death may occur due to recurrent infections (as a complication of immunosuppression).<ref name=":1" />
 
== Physiotherapy ==
In put from the physiotherapist team may occur in the rehabilitation phase. Rehabilitation interventions may be required to manage complications such as reduced [[Cardiopulmonary Exercise Testing (CPET) In Adults|cardiopulmonary]] function, [[Myopathy Steroid Induced|steroid-induced myopathy]] or [[Post-traumatic arthritis|bone and joint destruction]]Physiotherapy can help client maintain a good quality of life.
 
Treatment may include:
 
* Increasing muscle strength
* Improving [[balance]]
* Increasing flexibility
* Reducing risk of [[Falls and Exercise|falls]]
* Preventing development of muscular and joint contractures
* Providing equipment for mobility eg [[Walking Aids|walking aids]], [[Introduction to Orthotics|orthoses]], callipers and wheelchairs
* Advising on [[Moving and Handling|moving and handling]] techniques and equipment.<ref>Manchester Physio [https://www.manchesterneurophysio.co.uk/adults/conditions-we-treat/orthopaedic-msk-conditions/myopathy/physiotherapy-treatment-for-myopathies.php Myopathies] Available from:https://www.manchesterneurophysio.co.uk/adults/conditions-we-treat/orthopaedic-msk-conditions/myopathy/physiotherapy-treatment-for-myopathies.php (last accessed 22.11.2020)</ref>
 
===References===
[[Category:Autoimmune Disorders]]
[[Category:Conditions]]

Latest revision as of 02:29, 29 January 2023

Original Editor - Jaleel Mohammed Top Contributors - Lucinda hampton, Kim Jackson, Jaleel Mohammed and Aminat Abolade

Introduction[edit | edit source]

Bone Marrow Transplant

Graft versus host disease (GvHD) is a very common complication of bone marrow transplantations ( also known as allogeneic haematopoietic stem cell transplantation). Anti-rejection drugs have decreased the incidence, however it still occurs regularly.[1]

GvHD cause significant damage to the tissues affected, directly contributing to functional impairment. Treatment typically involves high-dose corticosteroids to suppress the immune system which can be destructive. Rehabilitation often is required to restore function and quality of life.[2]

Pathology[edit | edit source]

Graft versus host disease presents either early/acute (<100 days) or late/chronic (>100 days) post bone marrow transplantation, being a major complication. The skin, gastrointestinal tract (in particular the small bowel), and liver are the organs most often affected.[1]

How is GVHD Diagnosed?[edit | edit source]

A clinical diagnosis is usually made. Typically diagnosis includes the following:

  1. Immunologically competent cells present in the graft.
  2. The recipient has transplantation alloantigens appearing foreign to the graft and hence stimulate it antigenically.
  3. The recipient cannot mount an effective immunologic reaction against the graft, or enough time is allowed for the graft to manifest an immune response.[3]

Treatment[edit | edit source]

Treatment for GVHD is determined by the severity of symptoms and organs involved. Treatment options are based on immunosuppression with corticosteroids being the most commonly used treatment.[3]

What Are the Clinical Manifestations for cGVHD?[edit | edit source]

Hand GVHD with Sclerosis

Acute GVHD typically involves the skin, gastrointestinal tract, and liver. It may involve the lungs, kidneys, eyes, and hematopoietic system. Decreased responsiveness to active immunization may also occur.

  • Skin manifestation commonly arise as pruritic or painful maculopapular rash, first involving the palms, soles shoulders, and nape of the neck.
  • GI symptoms usually include diarrhea and abdominal pain, but mucositis, mucosal ulceration, nausea, and vomiting. Liver involvement usually include abnormal liver function tests with elevated bilirubin and alkaline phosphatase levels.

Chronic GVHD has a presentation similar to collagen vascular disorders and systemic sclerosis. Death may occur due to recurrent infections (as a complication of immunosuppression).[3]

Physiotherapy[edit | edit source]

In put from the physiotherapist team may occur in the rehabilitation phase. Rehabilitation interventions may be required to manage complications such as reduced cardiopulmonary function, steroid-induced myopathy or bone and joint destructionPhysiotherapy can help client maintain a good quality of life.

Treatment may include:

  • Increasing muscle strength
  • Improving balance
  • Increasing flexibility
  • Reducing risk of falls
  • Preventing development of muscular and joint contractures
  • Providing equipment for mobility eg walking aids, orthoses, callipers and wheelchairs
  • Advising on moving and handling techniques and equipment.[4]

References[edit | edit source]

  1. ↑ 1.0 1.1 Radiopedia Graft versus host disease Available: https://radiopaedia.org/articles/graft-versus-host-disease(accessed 28.1.2023)
  2. ↑ Smith SR, Asher A. Rehabilitation in chronic graft-versus-host disease. Physical Medicine and Rehabilitation Clinics. 2017 Feb 1;28(1):143-51.Available: https://www.sciencedirect.com/science/article/abs/pii/S1047965116300717?via%3Dihub (accessed 29.1.2023)
  3. ↑ 3.0 3.1 3.2 Vaillant AA, Modi P, Mohammadi O. Graft versus host disease. InStatPearls [Internet] 2022 Jul 8. StatPearls Publishing.Available:https://www.ncbi.nlm.nih.gov/books/NBK538235/ (accessed 28.1.2023)
  4. ↑ Manchester Physio Myopathies Available from:https://www.manchesterneurophysio.co.uk/adults/conditions-we-treat/orthopaedic-msk-conditions/myopathy/physiotherapy-treatment-for-myopathies.php (last accessed 22.11.2020)
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