- #ANTI HUMAN FGFR3 ANTIBODY CROSS REACTIVITY SKIN#
- #ANTI HUMAN FGFR3 ANTIBODY CROSS REACTIVITY SERIES#
Severe kidney disease (creatinine 1.5x > normal value).Severe liver disease (ALAT 3x > normal value).Cardiac insufficiency (New York Heart Association III/IV), cardiomyopathy, significant cardiac dysrhythmia requiring treatment, unstable or advanced ischemic heart disease).Patients with generalized, severe musculoskeletal conditions other than SFN that prevent a sufficient assessment of the patient by the physician.For example patients with diabetes, HIV, Sjogrens, Vitamin deficiency etc. Any other known cause for small fiber neuropathy other than the presence of the elevated titers of auto-antibodies.Patients must have elevated titers of autoantibodies to TS-HDS or FFR3 as measured in Dr Alan Pestronk's lab at Washington University in St Louis.Patients must have a baseline pain score on a VAS scale of Greater or equal to 4/10.
#ANTI HUMAN FGFR3 ANTIBODY CROSS REACTIVITY SKIN#
#ANTI HUMAN FGFR3 ANTIBODY CROSS REACTIVITY SERIES#
(This case series was presented as a poster at the American Academy of Neurology meeting in 2017) The investigators believe these anecdotal cases suggest that TS-HDS and FGFR-3 antibodies may be a marker for a group of SFN patients that are immune mediated and may respond to IVIG. After 6 months of therapy the IENFD was 8.4, 5.7, 3.3 respectively (these are clinically significant improvement in nerve fiber density. Pre-treatment IENFD was 1.6, 1.7, and 2.4 at the calf. In addition there was a clear increase in the intra-epidermal nerve fiber density (IENFD) after 6 months of therapy. All 3 cases showed marked improvement in pain scores. He examined skin biopsies for intra-epidermal nerve fiber density and patient self-reported pain scores at baseline and after six months of therapy. Levine (a co-investigator on this project) recently presented 3 cases of small fiber associated with elevated levels of auto-antibodies to TS-HDS or FGFR-3 who were treated with IVIG at 2 gm/kg/month for 6 months. The antibodies to TS-HDS and FGFR-3 are detected in up to 20% of patients with otherwise idiopathic small fiber neuropathy, but are rare in patients without small fiber neuropathy.ĭr. FGFR-3 is a secreted cell surface receptor genetic defects of FGFR-3 are linked to achrondroplasia and other bony abnormalities. Patients with elevated levels of IgM against TS-HDS display clear small fiber loss with IgM deposits around the outside of medium- & larger-sized capillaries with C5b-9 complement deposits. TS-HDS is a disaccharide component of glycosylation of heparin and heparin sulfate. We have recently uncovered two novel autoantibodies, TS-HDS and FGFR-3, that are targeted again peripheral neural structure. There is therefore a growing interest in the potential for using IVIG in small fiber neuropathy without direct proof that the disorder is caused by immune reactions. However, despite this broad differential at least 30% of cases of small fiber neuropathies remain idiopathic. Small fiber neuropathies, and mixed small and large fiber neuropathies, have many potential causes including diabetes, vitamin deficiencies, environmental and toxic exposures, HIV, autoimmune and paraproteinemias. Small Fiber Neuropathy Idiopathic Peripheral Neuropathyĭrug: Intravenous immunoglobulin Drug: 0.9% Sodium Chloride
The data gained from this pilot study will establish a rationale, with an appropriate screening test, for the use of immune globulin for the treatment of small fiber neuropathy. The co-primary endpoints will be a change in neuropathic pain (by VAS pain score) and a change in intra-epidermal nerve fiber density (by punch skin biopsy). The investigators hypothesize that individuals with auto-antibodies targeting neuronal antigens (TS-HDS and FGFR3) and confirmed evidence of small fiber neuropathy (by skin biopsy analysis of intra-epidermal nerve fiber density) will have an improvement in both nerve fiber density and pain after treatment with immune globulin. The objective of this study is to develop a rationale for the selective treatment of small fiber neuropathy with immune globulin (IVIG) in the appropriate patients.
Why Should I Register and Submit Results?.
0 kommentar(er)
