Plasma Exchange for Autoimmune Autonomic Failure

INCLUSION CRITERIA: The subjects have primary chronic autonomic failure, intact cardiac sympathetic innervation, and a circulating antibody to the nicotinic cholinergic receptor mediating ganglionic neurotransmission. This triad will be identified based on results of participation in NIH Clinical Protocol 03-N-0004. EXCLUSION CRITERIA: Subjects are excluded if they do not meet all three of the above Inclusion Criteria. Venous Access Candidate subjects are excluded if there is inadequate peripheral venous access, identified during participation in NIH Clinical Protocol 03-N-0004. Abnormal Clinical Pathology Test Results Candidate subjects are excluded based on abnormal Clinical Pathology results identified during participation in NIH Clinical Protocol 03-N-0004 (platelet count less than 100,000 per microliter, HIV-1 or 2 or chronic hepatitis B or C infection, or pregnancy). Medical Risk Candidate subjects are excluded for safety reasons if, in the opinion of the Associate Investigator of the NIH Department of Transfusion Medicine (DTM), therapeutic plasma exchange would pose an excessive risk of complications (e.g., stroke, hemorrhage, infection) outweighing the potential scientific and clinical benefit. In female patients with child-bearing potential, a blood test for pregnancy is done prior to testing on each visit. The finding of a positive test for pregnancy excludes the patient from further participation. Patients with autonomic failure have an increased risk of hypotension in response to manipulations that decrease venous return to the heart, such as plasma exchanges. Patients with autonomic failure typically also have high blood pressure when they are lying down. We expect that at the beginning of plasma exchanges, the blood pressure will be relatively high, and the plasma exchanges may be started with the patient in a sitting position. The Principal Investigator or one of the other Associate Investigators of the Clinical Neurocardiology Section will monitor the blood pressure frequently or continuously during the plasma exchanges. Patients with autonomic failure often tolerate remarkably low blood pressure. A systolic blood pressure of 90 mm Hg will be the threshold for intervening. The first intervention will be to place the patient in a supine position. Second, we would administer saline and albumin and slow down the rate of or stop the plasma exchange. Drugs to control blood pressure will also be on hand for immediate emergency use if needed. If we think that we cannot prevent low blood pressure and fainting during future plasma exchanges, then we would exclude the patient from further plasma exchanges under this Protocol. If uncontrollable hypotension occurs in a second patient, then the Protocol will be terminated. Patients are tested for hepatitis and for HIV before undergoing plasma exchange. Blood from people with hepatitis or HIV can contaminate the Blood Bank equipment. If a subject has hepatitis or HIV, the subject will be excluded from further participation in the study. The Consent Form includes standard consent language about HIV testing. Age - The Protocol is restricted to subjects at least 18 years old. Medications - Medications likely to interfere with the scientific results will be discontinued on an inpatient basis. Termination of Participation - A subject may refuse certain tests or procedures, or may terminate participation early, without loss of benefits to which the subject was previously entitled. The Investigators may also exclude a subject from further participation, such as in the event of known or suspect falsification of medical history information or refusal to undergo planned tests or procedures, without loss of benefits to which the subject was previously entitled.