Combined immunomodulatory therapies resulted in stepwise recovery in autoimmune autonomic ganglionopathy

Some intractable cases of autoimmune autonomic ganglionopathy are treated with diverse combinations of immunomodulatory therapies. However, it is unclear which of the immunotherapies directly contributed to the relief of dysautonomia when several immunotherapies have been utilized. To evaluate which of the immunomodulatory therapies are effective, we describe a 60-year-old man with autoimmune autonomic ganglionopathy who was successfully treated by combined immunomodulatory therapies. The patient showed subacute pure autonomic failure without antecedent infectious symptoms, and was admitted to Yamaguchi University Hospital, Yamaguchi, Japan. He demonstrated involvement of cardiovascular, pupillary, sudomotor, gastrointestinal and bladder functions. Serum ganglionic acetylcholine receptor autoantibody titer was highly elevated. The diagnosis of autoimmune autonomic ganglionopathy was made. Plasma exchange was repeated nine times, resulting in minimal improvement. Subsequently, intravenous immunoglobulin therapy followed by steroid administration was initiated, after which his symptoms gradually improved. Plasma noradrenaline levels showed a stepwise elevation after each treatment, including plasma exchange. Our case adds to the evidence that combining several immunomodulatory therapies is beneficial for some intractable cases of autoimmune autonomic ganglionopathy that do not adequately respond to a single immunomodulatory therapy. Each immunomodulatory therapy should be effective even though they are not enough to show clinical improvement.