Ganglionic Antibody Level as a Predictor of Severity of Autonomic Failure
| Autor: | Paola Sandroni, Eduardo E. Benarroch, Phillip A. Low, Jeremy K. Cutsforth-Gregory, David M. Sletten, Mariana D. Suarez, Robert D. Fealey, Andrew McKeon, Elizabeth A. Coon, Wolfgang Singer |
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| Rok vydání: | 2018 |
| Předmět: |
0301 basic medicine
Male medicine.medical_specialty Databases Factual Autoimmunity Autoimmune autonomic ganglionopathy Immunologic Tests Receptors Nicotinic Gastroenterology Sensitivity and Specificity Severity of Illness Index Article 03 medical and health sciences Orthostatic vital signs 0302 clinical medicine Predictive Value of Tests Internal medicine Severity of illness medicine Autonomic reflex Humans Anhidrosis Pure autonomic failure Ganglia Autonomic Autoantibodies business.industry Autoantibody General Medicine Middle Aged medicine.disease 030104 developmental biology Autonomic Nervous System Diseases ROC Curve Predictive value of tests Female medicine.symptom business Clinical Laboratory Information Systems 030217 neurology & neurosurgery |
| Zdroj: | Mayo Clinic proceedings. 93(10) |
| ISSN: | 1942-5546 |
| Popis: | Objective To assess antibody level as a test of autonomic failure (AF) associated with ganglionic nicotinic acetylcholine receptor antibody (AChR-Ab) autoimmunity. Patients and Methods We searched the Mayo Clinic laboratory database of 926 ganglionic AChR-Ab–seropositive patients seen at our institution between October 1, 1997, and April 1, 2015, for initial level of 0.05 nmol/L or higher and contemporaneous autonomic reflex screen (standardized evaluation of adrenergic, cardiovagal, and sudomotor functions) from which Composite Autonomic Scoring Scale (CASS) scores could be calculated. Results Of 289 patients who met inclusion criteria, 163 (56.4%) were women, median age was 54 years (range, 10-87 years), median antibody level was 0.11 nmol/L (range, 0.05-22.10 nmol/L), and median CASS total score was 2.0 (range, 0-10). Using receiver operating characteristic curve analysis, a level above 0.40 nmol/L predicted severe AF (CASS score, ≥7) with 92% specificity and 56% sensitivity. For at least moderate AF (CASS score ≥4 and anhidrosis ≥25%), a level of at least 0.20 nmol/L had 80% specificity and 59% sensitivity. Levels below 0.20 nmol/L were not predictive of the presence or absence of AF. For predicting orthostatic hypotension, ganglionic AChR-Ab level had excellent specificity above 0.4 nmol/L but lacked sensitivity. Autoantibodies to additional targets were present in 61 patients (21.1%). Conclusion Ganglionic AChR-Ab level of at least 0.40 nmol/L is a moderately sensitive and highly specific marker for severe AF, as is a level of at least 0.20 nmol/L for moderate AF if CASS score is coupled with anhidrosis of 25% or more, among patients with suspected ganglionic AChR-Ab autoimmune autonomic ganglionopathy. Antibody levels of less than 0.20 nmol/L have little clinical importance in the absence of clinical AF. |
| Databáze: | OpenAIRE |
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