Objective:

To assess the efficacy of intravenous immunoglobulin (IVIG) therapy in Autoimmune Dysautonomia

Background:

IVIG has been successfully used in the past for pain management and Autoimmune Gastric Dysmotility Disorder (AGID) of adult patients with suspected autoimmune etiology. However, the literature on efficacy of IVIG in children is scarce.

Design/Methods:

A retrospective review of AGID patients treated with IVIG was conducted. All symptoms were reviewed.

Results:

Patient 1 was positive for IgM TS-HDS Ab. Patients 2 and 3 were positive for Neuronal Acetylcholine Receptor Ganglionic (alpha-3) Ab. Patients 1 and 2 had Small Fiber Neuropathy diagnosis. Age at diagnosis of patients 1, 2, and 3 were 14, 17.25, and 16.5 years, respectively. Duration of treatment for patient 3 was 6 months, and 1 year for patients 1 and 2. Post treatment, patient 1 did not require venting of J tube; though still dependent on Total Parenteral Nutrition (TPN). Patient 1 is no longer dependent on enemas, as distal colonic and antroduodenal contractions have normalized. Esophageal dysmotility remains unchanged. Abdominal pain and distention, Postural Orthostatic Tachycardia Syndrome (POTS), and neuropathic pain improved. Patient 2 presented with intractable vomiting and POTS. She was dependent on Nasogastric tube (NG) feedings due to vomiting and was on cyproheptadine, erythromycin, bethanechol, amitriptyline, and prucalopride. Post treatment, vomiting resolved and all but prucalopride were discontinued. Colonic motility has not been re-assessed, however constipation, abdominal distention, and pain resolved. Polyethylene glycol and Bisacodyl were discontinued. Likewise, her POTS resolved.  Patient 3 had hyperadrenergic POTS, persistent vomiting, and constipation, suffering a 4 kg weight loss. Following treatment, her vomiting and constipation improved, regaining 3.5 kg. Activity levels and orthostatic dizziness improved, and midodrine and fludrocortisone were discontinued.

Conclusions: