Autoimmune disorder with antibodies

Category: Health

Autoimmune autonomic ganglionopathy (AAG) is a rare autoimmune disorder with antibodies against ganglionic nicotinic acetylcholine receptor (α3-nAChR) of the autonomic ganglia¹. Patients may experience symptoms of acute to subacute, generalized sympathetic, parasympathetic and enteric autonomic failure. Antibodies are detected in about 50% of patients and levels correlate with the severity of dysautonomia. An understanding of the underlying pathophysiology is imperative for timely diagnosis and management, including the appropriate use of immunosuppressive therapy in conjunction with plasmapheresis, supportive care and a search for malignancy. Patients may be seronegative for α3-nAChR antibodies but may still benefit from immunotherapy³. The clinical course of AAG is variable. Spontaneous improvement occurs in about one-third of patients, but recovery is typically incomplete.