| Skin
Diseases Similar to Scleroderma (Main Menu) |
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| Graft-versus-Host
Disease (GVHD) |
| This page
was written by Shelley
Ensz and has not yet been medically edited. See disclaimer. |
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| Overview of Graft-versus-Host
Disease (GVHD) |
| Graft-versus-Host Disease (GVHD)
is an autoimmune response to foreign cells, such as from skin
grafting, bone marrow transplants, stem cell transplants, blood
transfusions, or organ transplants. |
| What
is GVHD? When you receive a donor's stem cells (the “graft”),
their job is to recreate the donor's immune system in your
body (the “host”). Graft versus host disease (GVHD) is the
term used when this new immune system attacks your body.
Your donor's cells see your body as “foreign” and attack
it––causing damage. GVHD most commonly affects the skin,
liver, and gastrointestinal tract. National Institutes
of Health (NIH). |
| Lichen
sclerosus and eosinophilic fasciitis as manifestations of
chronic graft-versus-host disease: expanding the sclerodermoid
spectrum. We describe 6 patients, all of whom developed
LS and two of whom also developed EF in the context of chronic
GVHD. Although all patients eventually developed morpheaform
and/or sclerodermoid GVHD, LS was a prominent part of the
initial presentation of chronic cutaneous GVHD in every case. PubMed.
J Am Acad Dermatol. 2005 Oct;53(4):591-601. (Also see: Lichen
Sclerosus and Eosinophilic
Fasciitis) |
| Sclerodermatous
Chronic Graft-Versus-Host Disease. The presence of lichen
sclerosus, morphea, septal fibrosis, and fasciitis suggests
that the sclerosis can start at and affect any level of the
skin. Most patients have a good prognosis with treatment
with prednisone and systemic immunosuppression. M. Matias
De La Mano. AB0117 EULAR 2004. |
| Causes of Graft-versus-Host
Disease (GVHD) |
| Autoimmune-like
disease post-bone marrow transplantation. Chronic graft-vs-host
disease is the major complication after bone marrow transplantation
and mimics some autoimmune diseases, such as scleroderma,
sicca syndrome, primary biliary cirrhosis and an increased
prevalence of various autoantibodies. PubMed. Rev Med
Interne. 2004 Jul;25(7):514-523. (Also see: Stell
Cell Transplants) |
| Impaired
thymic negative selection causes autoimmune graft-versus-host
disease. These results demonstrate that impaired thymic
negative selection can cause lethal autoimmune disease indistinguishable
from acute GVHD in the context of a proinflammatory milieu
when peripheral regulatory mechanisms are absent. PubMed.
Blood 2003 Mar 27 |
| Diagnosis of Graft-versus-Host
Disease (GVHD) |
| Cutaneous
gene expression by DNA microarray in murine sclerodermatous graft-versus-host
disease, a model for human scleroderma. These constellations of
immunologic changes provide a "fingerprint" for fibrosing autoimmune
disease. They are useful to understand the pathogenesis of Scl GVHD, to
identify markers for early diagnosis of disease, and to devise more effective
strategies for intervention in early scleroderma and Scl GVHD. PubMed.
J Invest Dermatol. 2007 Feb;127(2):281-92. (Also see: Skin Fibrosis) |
| A
Pediatric Case of Sclerodermatous Chronic Graft-versus-Host
Disease. In the future, clarification of the pathogenesis
of chronic GVHD and establishment of therapy will be necessary. PubMed.
Pediatr Dermatol. 2003 Jul-Aug;20(4):327-31. |
| Bullous
scleroderma-like changes in chronic graft-versus-host disease. Chronic
cutaneous GVHD is categorized according to the type of skin
lesions into lichenoid and sclerodermoid variants, but bullous
scleroderma-like changes are exceptional. PubMed. J Eur
Acad Dermatol Venereol 2003 Mar;17(2):200-3. |
| Treatments for Graft-versus-Host
Disease (GVHD) |
| New
Treatments Fight Graft-versus.-Host-Disease (GVHD) In Bone
Marrow Transplant Patients. Using anti-inflammatory proteins
in combination with steroids has yielded a response rate
of over two-thirds, twice the traditional rate of complete
response. Medical News Today. 02/06/07. |
| Safety
and potential efficacy of low-dose methotrexate for treatment
of chronic graft-versus-host disease. Methotrexate (MTX)
appears to be a well-tolerated, inexpensive and possibly
steroid-sparing agent that is worthy of further evaluation
in prospective trials for treatment of chronic GVHD. PubMed.
Bone Marrow Transplant. 2005 Jun 20. |
| Medium
dose long-wavelength ultraviolet A (UVA1) phototherapy for
the treatment of acute and chronic graft-versus-host disease
of the skin. UVA1 as an adjunct to systemic immunosuppressive
treatment was found to be safe, and effective in 10 patients
with chronic cutaneous (seven lichenoid and three sclerodermoid)
graft-versus-host disease (GVHD) after stem cell transplantation. PubMed.
Bone Marrow Transplant. 2005 Jan 24. |
| Chronic
graft-versus-host-disease-like dermopathy in a child with
CD4+ cell microchimerism. We report the case of an 11-year-old
boy suffering from a severe progressive chronic skin disease
with clinical features of progressive systemic scleroderma,
systemic lupus erythematosus and dermatomyositis. A distinct
improvement of erythemas and sclerosis could be achieved
by means of low-dose UVA1 phototherapy which was applied
with escalating single doses of 3-12 J/cm2 for 35 consecutive
days. PubMed. Dermatology. 2005;210(1):68-71. |
| Mechanism
of action of extracorporeal photochemotherapy (ECP) in chronic
graft-versus-host disease. ECP, considered as an effective
treatment for patients with erythrodermic cutaneous T-cell
lymphoma, has recently been used successfully in the treatment
of GvHD. Only a better and more complete understanding of
the various mechanisms involved will enable this new therapy
to be made more effective and selective. PubMed. Br J
Dermatol. 2004 Jun;150(6):1055-60. |
| Patient Stories for
Graft-versus-Host Disease (GVHD) |
| Blog: Jackie Sue We Love You. Jackie has GVHD. THe blog covers her progress after her mini-allo MUD transplant. Her transplant was the first one to be performed by the Mayo Clinic. Bruce and Jackie. |
| Klaas:
Scleroderma Graft-vs-Host Disease (GVHD) So I would
like to know if, and when, I will be able to walk again... |
| (Spanish/Español)
Klaas: Sclerodermia da GVHD Essiste un modo per capire
se ci sono delle speranze e quali sono... |
| (Italiano)
Fabiola: Paziente Affetta da Sclerodermia Morphea Dopo
5 anni si e' svegliata una sclero conseguenza pero' della
GVHD (Graft Versus Host Disease) che viene dopo il trapianto
di midollo... |
| Research for Graft-versus-Host
Disease (GVHD) |
| Stem Cell
Finding Could Help Reduce Transplant Rejection. A new
study involving a type of stem cells from the lungs of transplant
patients demonstrates for the first time that these progenitor
cells reside in adult organs and are not derived from bone
marrow, which leads to the possibility that the cells may
be able to help with the rejection of donated organs.Newswise.
University of Michigan Health System. 03/08/07. (Also see: Pulmonary
Fibrosis) |
| New
Treatments Fight Graft-versus.-Host-Disease (GVHD) In Bone
Marrow Transplant Patients. Using anti-inflammatory proteins
in combination with steroids has yielded a response rate
of over two-thirds, twice the traditional rate of complete
response. Medical News Today. 02/06/07. |
| Emergent
autoimmunity in graft versus host disease. These results
indicate that donor T cell-mediated pathological damage during
GVHD becomes donor APC (antigen presenting cells)-dependent,
and provide a mechanistic explanation for the longstanding
observation that GVHD is associated with autoimmune clinical
manifestations. PubMed. Blood. 2005 Mar 3. |
| A
modified model of graft-versus-host-induced systemic sclerosis
(scleroderma) exhibits all major aspects of the human disease. This
modified model of GVH-induced SSc is likely to contribute
to better understanding of the disease mechanisms and, ultimately,
improved treatments for patients. PubMed. Arthritis Rheum.
2004 Apr;50(4):1319-31. |
| Immunophenotyping
of Chimeric Cells and Surrounding Cellular Infiltrates in
Linear Scleroderma and Morphea Skin Tissue. The role
of chimerism remains elusive. However, with the identification
of dendritic cells in the lesion we can hypothesize that
the cells involved in the inflammatory response in LS/morphea
are similar to lesions seen in cGVHD (chronic graft-vs-host
disease) where dendritic cells are proposed to play a role
in disease. Kelly Mc Nallan. ACR Conference Oct. 2003.
(Also see: Fetal
Cells and Scleroderma, Morphea, Linear,
and Causes
of Scleroderma: Dendritic Cells) |