IVIG in the Treatment of Autoimmune Small Fiber Neuropathy With TS-HDS, FGFR-3, or Plexin D1 Antibodies

Inclusion Criteria:

1. Patients ≥ age 18

2. Patient with clinical and biopsy evidence of pure small fiber neuropathy (with orwithout dysautonomia) as evidenced by reduced IENFD on skin biopsy using PGP 9.5 asthe immunostain. Biopsy must have been performed within 12 months of studyenrollment. If biopsies were not done at CRL, they will be repeated and done at 3sites (upper and lower thigh, lower calf), to have consistent and equivalent biopsydata with the follow up biopsy done after 6 mos of treatment

3. Patients must have elevated and/or abnormal titers of autoantibodies to TS-HDS-IgM,FGFR3-IgG, or Plexin-D1 measured by the Washington University NeuromuscularLaboratory (St Louis) within 12 mos of enrollment

4. Patients must have a baseline pain score on a visual analogue scale (VAS) of Greateror equal to 4/10

5. Patients must have a baseline Utah Early Neuropathy Scale (UENS) score of Greater orequal to 4/10

6. Small Fiber Neuropathy Screening List (SFNSL) score of 11/84 or greater

7. Non-pregnant, non-lactating female. Females of reproductive potential must use 2forms of contraception or continuously abstain from heterosexual sex duringtreatment

Exclusion Criteria:

1. Any other known cause for small fiber neuropathy other than the presence of theelevated titers of TS-HDS-IgM, FGFR3-IgG, or Plexin-D1 autoantibodies

2. Patients with generalized, severe musculoskeletal conditions other than SFN thatprevent a sufficient assessment of the patient by the physician

3. Electromyography/nerve conduction study (EMG/NCS) evidence of large fiberpolyneuropathy, to be confirmed by study PI

4. Underlying severe heart, kidney, liver disease, or HIV infection, (Note: If there isno previous HIV test result documented within the last 5 years, a test may beperformed in order to confirm eligibility)

5. Patients with a history of deep vein thrombosis within the last year prior tobaseline visit or pulmonary embolism ever; patients with susceptibility to embolismor deep vein thrombosis

6. Known significant IgA deficiency with antibodies to IgA

7. History of hypersensitivity, anaphylaxis or severe systemic response toimmuno-globulin, blood or plasma derived products, or any component of IVIG 10%

8. Known blood hyperviscosity, or other hypercoagulable states

9. Use of IgG products within six months prior to enrollment

10. Patients with a history of drug or alcohol abuse within the past five years prior toenrollment

11. Patients unable to understand or unwilling or unable to comply with the studyprotocol