Upper airway resistance syndrome

Upper airway resistance syndrome is a sleep disorder characterized by the narrowing of the airway that can cause disruptions to sleep.[1][2] The primary symptoms include chronic insomnia, anxiety, fatigue or sleepiness, unrefreshing sleep, and difficulty concentrating. UARS can be diagnosed by polysomnograms capable of detecting Respiratory Effort-related Arousals. It can be treated with lifestyle changes, dental devices, surgery, or CPAP therapy.[3] UARS is considered a variant of sleep apnea.[4]

Upper airway resistance syndrome
Other namesUARS

Signs and symptoms

Symptoms of UARS are similar to those of obstructive sleep apnea, but not inherently overlapping. Fatigue, insomnia, daytime sleepiness, unrefreshing sleep, anxiety, and frequent awakenings during sleep are the most common symptoms.

Many patients experience chronic insomnia that creates both a difficulty falling asleep and staying asleep. As a result, patients typically experience frequent sleep disruptions.[5] Some patients with UARS snore, but not all.[4]

Some patients experience hypotension, which may cause lightheadedness, and patients with UARS are also more likely to experience headaches and irritable bowel syndrome.[5]

Predisposing factors include a high and narrow hard palate, an abnormally small intermolar distance, an abnormal overjet greater than or equal to 3 millimeters, and a thin soft palatal mucosa with a short uvula. In 88% of the subjects, there is a history of early extraction or absence of wisdom teeth. There is an increased prevalence of UARS in east Asians. [6]

Pathophysiology

Upper airway resistance syndrome is caused when the upper airway narrows without closing. Consequently, airflow is either reduced or compensated for through an increase in inspiratory efforts. This increased activity in inspiratory muscles leads to the arousals during sleep which patients may or may not be aware of.[1]

A typical UARS patient is not obese and possesses small jaws, which can result in a smaller amount of space in the nasal airway and behind the base of the tongue.[4] Patients may have other anatomical abnormalities that can cause UARS such as deviated septum, inferior turbinate hypertrophy, a narrow hard palate that reduces nasal volume, enlarged tonsils, or nasal valve collapse.[7][2] UARS affects equal numbers of males and females.[1]

Why some patients with airway obstruction present with UARS and not OSA is thought to be caused by alterations in nerves located in the palatal mucosa. UARS patients have largely intact and responsive nerves, while OSA patients show clear impairment and nerve damage. Functioning nerves in the palatal mucosa allow UARS patients to more effectively detect and respond to flow limitations before apneas and hypopneas can occur. What damages the nerves is not definitively known, but it is hypothesized to be caused by the long term effects of Gastroesophageal reflux and/or snoring. [8]

Diagnosis

UARS is diagnosed using the Respiratory Disturbance Index (RDI). A patient is considered to have UARS when they have an Apnea-Hypopnea Index (AHI) less than 5, but an RDI greater than or equal to five. Unlike the Apnea-Hypopnea Index, the Respiratory Disturbance Index includes Respiratory Effort-related Arousals (RDI = AHI + RERA Index). [9] In 2014, the definition of Sleep Apnea was changed to include patients with UARS by using RDI to determine sleep apnea severity.

Polysomnograms can be used to help diagnose UARS. On polysomnograms, a UARS patient will have very few apneas and hypopneas, but many Respiratory effort-related Arousals. RERAs are periods of increased respiratory effort lasting for more than ten seconds and ending in arousal. Whether or not an event is classified as a RERA or Hypopnea depends on the definition of Hypopnea used by the sleep technician.[10] The American Academy of Sleep Medicine currently recognizes two definitions. The scoring of Respiratory Effort-related Arousals is currently designated as "optional" by the AASM. Thus, many patients who receive sleep studies may receive a negative result, even if they have UARS. [11]

Based on symptoms, patients are commonly misdiagnosed with idiopathic insomnia, idiopathic hypersomnia, chronic fatigue syndrome, fibromyalgia, or a psychiatric disorder such as ADHD or depression.[5] Studies have found that children with UARS are frequently misdiagnosed with ADHD. One study found UARS or OSA present in up to 56% of children with ADHD.[12] Symptoms of ADHD caused by UARS remit with treatment.[13]

Management

Behavioral modification

Behavioral modifications include getting at least 7–8 hours of sleep and various lifestyle changes, such as positional therapy.[14] Sleeping on one's side rather than in a supine position or using positional pillows can provide relief, but these modifications may not be sufficient to treat more severe cases.[14] Avoiding sedatives including alcohol and narcotics can help prevent the relaxation of airway muscles, and thereby reduce the chance of their collapse. Avoiding sedatives may also help to reduce snoring.[14]

Medications

Nasal steroids may be prescribed in order to ease nasal allergies and other obstructive nasal conditions that could cause UARS.[14]

Positive airway pressure therapy

Positive airway pressure therapy is similar to that in obstructive sleep apnea and works by stenting the airway open with pressure, thus reducing the airway resistance. Use of a CPAP mask can help ease the symptoms of UARS. Therapeutic trials have shown that using a CPAP mask with pressure between four and eight centimeters of water can help to reduce the number of arousals and improve sleepiness.[4] CPAP masks are the most promising treatment for UARS, but effectiveness is reduced by low patient compliance.[15]

Recent studies have shown that more advanced PAP devices, such as Bilevel PAP and Adaptive Servo Ventilation, are more effective for treating UARS as they provide better pressure support on exhale, mimicking normal breathing and making higher pressures more tolerable. [16]

Oral appliances

Oral appliances to protrude the tongue and lower jaw forward have been used to reduce sleep apnea and snoring, and hold potential for treating UARS, but this approach remains controversial.[15] Oral appliances may be a suitable alternative for patients who cannot tolerate CPAP.[14]

Surgery

For nasal obstruction, options can be septoplasty, turbinate reductions, or surgical palate expansion.[2]

Orthognathic surgeries that expands the airway, such as Maxillomandibular advancement (MMA) or Surgically Assisted Rapid Palatal Expansion (SARPE) are the most effective surgeries for sleep disordered breathing. MMA is often completely curative.[17]

Though less common methods of treatment, various surgical options including uvulopalatopharyngoplasty (UPPP), hyoid suspension, and linguloplasty exist. These procedures increase the dimensions of the upper airway and reduce the collapsibility of the airway.[3] One should also be screened for the presence of a hiatal hernia, which may result in abnormal pressure differentials in the esophagus, and in turn, constricted airways during sleep.[3] Palatal tissue reduction via radiofrequency ablation has also been successful in treating UARS.[15]

Treatment in children

The primary treatment for children is the removal of enlarged tonsils and adenoids via a tonsillectomy and adenoidectomy. Orthodontic treatment to expand the volume of the nasal airway, such as nonsurgical Rapid Palatal expansion is common.[18][14]

See also

References
  1. Shneerson, John M., ed. (2005). Sleep Medicine (Second ed.). New York: Blackwell Publishing. pp. 229-237.
  2. de Oliveira, Pedro Wey Barbosa; Gregorio, Luciano Lobato; Silva, Rogério Santos; Bittencourt, Lia Rita Azevedo; Tufik, Sergio; Gregório, Luis Carlos (July 2016). "Orofacial-cervical alterations in individuals with upper airway resistance syndrome" (PDF). Brazilian Journal of Otorhinolaryngology. 82 (4): 377–384. doi:10.1016/j.bjorl.2015.05.015. PMID 26671020.
  3. de Godoy, Luciana B.M.; Palombini, Luciana O.; Guilleminault, Christian; Poyares, Dalva; Tufik, Sergio; Togeiro, Sonia M. (2015). "Treatment of upper airway resistance syndrome in adults: Where do we stand?". Sleep Science: 42–48 – via Elsevier.
  4. Cuelbras, Antonio (1996). Clinical Handbook of Sleep Disorders. New York: Butterworth-Heinemann. pp. 207.
  5. Kushida, Clete A., ed. (2009). Handbook of Sleep Disorders (Second ed.). New York: Inform Healthcare. pp. 339–347.
  6. Guilleminault, C., & Chowdhuri, S. (2000). Upper Airway Resistance Syndrome Is a Distinct Syndrome. American Journal of Respiratory and Critical Care Medicine, 161(5), 1412–1413. https://doi.org/10.1164/ajrccm.161.5.16158a
  7. Garcha, Puneet S.; Aboussouan, Loutfi S.; Minai, Omar (January 2013). "Sleep-Disordered Breathing". Cleveland Clinic Disease Management. Retrieved 15 March 2017.
  8. Guilleminault, C., Li, K., Chen, N.-H., & Poyares, D. (2002). Two-Point Palatal Discrimination in Patients With Upper Airway Resistance Syndrome, Obstructive Sleep Apnea Syndrome, and Normal Control Subjects. Chest, 122(3), 866–870. https://doi.org/10.1378/chest.122.3.866
  9. de Godoy, L. B. M., Palombini, L. O., Guilleminault, C., Poyares, D., Tufik, S., & Togeiro, S. M. (2015). Treatment of upper airway resistance syndrome in adults: Where do we stand? Sleep Science, 8(1), 42–48. https://doi.org/10.1016/j.slsci.2015.03.001
  10. de Godoy, L. B. M., Palombini, L. O., Guilleminault, C., Poyares, D., Tufik, S., & Togeiro, S. M. (2015). Treatment of upper airway resistance syndrome in adults: Where do we stand? Sleep Science, 8(1), 42–48. https://doi.org/10.1016/j.slsci.2015.03.001
  11. Berry, R. B., Budhiraja, R., Gottlieb, D. J., Gozal, D., Iber, C., Kapur, V. K., Marcus, C. L., Mehra, R., Parthasarathy, S., Quan, S. F., Redline, S., Strohl, K. P., Ward, S. L. D., & Tangredi, M. M. (2012). Rules for Scoring Respiratory Events in Sleep: Update of the 2007 AASM Manual for the Scoring of Sleep and Associated Events. Journal of Clinical Sleep Medicine, 8(5), 597–619. https://doi.org/10.5664/jcsm.2172
  12. Huang, Y.-S., Chen, N.-H., Li, H.-Y., Wu, Y.-Y., Chao, C.-C., & Guilleminault, C. (2004). Sleep disorders in Taiwanese children with attention deficit/hyperactivity disorder. Journal of Sleep Research, 13(3), 269–277. doi:10.1111/j.1365-2869.2004.00408.x
  13. Amiri, S., AbdollahiFakhim, S., Lotfi, A., Bayazian, G., Sohrabpour, M., & Hemmatjoo, T. (2015). Effect of adenotonsillectomy on ADHD symptoms of children with adenotonsillar hypertrophy and sleep disordered breathing. International Journal of Pediatric Otorhinolaryngology, 79(8), 1213–1217. doi:10.1016/j.ijporl.2015.05.015
  14. "Upper Airway Resistance Syndrome (UARS)". Stanford Medicine. Retrieved February 28, 2017.
  15. Exar EN, Collop NA (Apr 1999). "The upper airway resistance syndrome". Chest. 115 (4): 1127–39. doi:10.1378/chest.115.4.1127.
  16. Krakow B, Mciver ND, Ulibarri VA, Krakow J, Schrader RM. Prospective Randomized Controlled Trial on the Efficacy of Continuous Positive Airway Pressure and Adaptive Servo-Ventilation in the Treatment of Chronic Complex Insomnia. EClinicalMedicine. 2019;13:57-73.
  17. de Godoy, L. B. M., Palombini, L. O., Guilleminault, C., Poyares, D., Tufik, S., & Togeiro, S. M. (2015). Treatment of upper airway resistance syndrome in adults: Where do we stand? Sleep Science, 8(1), 42–48. https://doi.org/10.1016/j.slsci.2015.03.001
  18. Guilleminault, Christian and Khramtsov, Andrei. (December 2001). “Upper airway resistance syndrome in children”. Seminars in Pediatric Neurology: 207-215 - via Elsevier.
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