PMID- 29781817
OWN - NLM
STAT- MEDLINE
DCOM- 20200205
LR  - 20220410
IS  - 1536-3686 (Electronic)
IS  - 1075-2765 (Linking)
VI  - 26
IP  - 5
DP  - 2019 Sep/Oct
TI  - Intravenous Immunoglobulin Therapy in Refractory Autoimmune Dysautonomias: A 
      Retrospective Analysis of 38 Patients.
PG  - 570-582
LID - 10.1097/MJT.0000000000000778 [doi]
AB  - BACKGROUND: Intravenous immunoglobulin (IVIG) has recognized efficacy in 
      autoimmune peripheral nerve disorders, but there has been limited study of the 
      use of IVIG in autoimmune dysautonomias. STUDY QUESTION: To determine the 
      efficacy and safety of IVIG in patients with disabling, refractory autoimmune 
      dysautonomias, including patients with postural tachycardia syndrome and 
      gastrointestinal dysmotility. STUDY DESIGN: Patients with one or more autonomic 
      disorder(s) and persistent serological evidence for autoimmunity who were unable 
      to work or attend school despite usual treatments for dysautonomia were treated 
      with IVIG for at least 3 months at a dose of at least 1 gm/kg monthly. MEASURES 
      AND OUTCOMES: Outcome measures included the composite autonomic symptom scale 31 
      survey and a functional ability score. RESULTS: There were 38 patients, 84% 
      female and mean age of 28.4 years. Of patients, 83.5% improved on IVIG as defined 
      by at least 20% improvement in the composite autonomic symptom scale 31 and/or 
      functional ability score. The mean pretreatment functional ability score was 21% 
      (mostly bedridden), which improved to a mean of 74% (nearing able to return to 
      work/school) for responsive patients after at least 1 year of IVIG. The mean time 
      to the first sign of response was 5.3 weeks. There were no serious adverse 
      events. The Mayo autoimmune dysautonomia panel antibodies and traditional Sjogren 
      antibodies were present in only 13% and 8% of patients, respectively, but 
      antiphospholipid antibodies and novel Sjogren antibodies were present in 76% and 
      42% of patients, respectively. CONCLUSIONS: There is increasing evidence that 
      IVIG is safe and effective in a subset of patients with autonomic disorders and 
      evidence for autoimmunity. A 4-month IVIG trial should be considered in severely 
      affected patients who are refractory to lifestyle and pharmacological therapies. 
      Antiphospholipid antibodies and novel Sjogren antibodies are often present in 
      these patients and correlate with a high response rate to IVIG.
FAU - Schofield, Jill R
AU  - Schofield JR
AD  - Department of Medicine, Denver University of Colorado Anschutz Medical Campus, 
      Aurora, CO.
FAU - Chemali, Kamal R
AU  - Chemali KR
AD  - Department of Neurology, Eastern Virginia Medical School, Norfolk, VA.
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Am J Ther
JT  - American journal of therapeutics
JID - 9441347
RN  - 0 (Antibodies, Antiphospholipid)
RN  - 0 (Immunoglobulins, Intravenous)
RN  - 0 (Immunologic Factors)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Antibodies, Antiphospholipid/blood/immunology
MH  - Autoimmune Diseases of the Nervous System/blood/*drug therapy/immunology
MH  - Child
MH  - Dose-Response Relationship, Drug
MH  - Female
MH  - Humans
MH  - Immunoglobulins, Intravenous/*administration & dosage/adverse effects
MH  - Immunologic Factors/*administration & dosage/adverse effects
MH  - Male
MH  - Middle Aged
MH  - Primary Dysautonomias/blood/*drug therapy/immunology
MH  - Retrospective Studies
MH  - Treatment Outcome
MH  - Young Adult
EDAT- 2018/05/22 06:00
MHDA- 2020/02/06 06:00
CRDT- 2018/05/22 06:00
PHST- 2018/05/22 06:00 [pubmed]
PHST- 2020/02/06 06:00 [medline]
PHST- 2018/05/22 06:00 [entrez]
AID - 10.1097/MJT.0000000000000778 [doi]
PST - ppublish
SO  - Am J Ther. 2019 Sep/Oct;26(5):570-582. doi: 10.1097/MJT.0000000000000778.