Sarcoidosis-related small fiber neuropathy: focus on fatigue, pain, restless legs syndrome, and cognitive function

##plugins.themes.themeTen.article.main##

Lisette Raasing https://orcid.org/0000-0003-0474-7550

Keywords

chronic pain, cognitive impairment, disease severity, fatigue, neuropathic symptoms, Patient reported outcome, prevalence, quality of life, Restless legs syndrome, sarcoidosis, severity, small fiber neuropathy, symptom burden, symptoms

Abstract

Background – Of all sarcoidosis patients 60-90% suffer from fatigue, 40-80% experience small fiber neuropathy, 35% suffer from cognitive impairment and 16-52% experience restless legs syndrome (RLS). The prevalence, severity and association between fatigue, pain, RLS and cognitive impairment in patients with sarcoidosis and small fiber neuropathy (SFN) is unknown and will be investigated in this study.


Methods - Healthy controls, patients with sarcoidosis with SFN and patients with sarcoidosis without SFN were compared. They completed multiple questionnaires (fatigue assessment scale (FAS), RLS rating scale, visual analogue scale (VAS) score for pain and cognitive failure questionnaire (CFQ)), to estimate the prevalence, severity, and correlation of fatigue, RLS, pain, SFN and cognitive impairment.


Results - Twenty healthy volunteers, 49 patients with sarcoidosis without SFN and 48 patients with sarcoidosis with SFN were included. Fatigue was the most prevalent symptom in patients with sarcoidosis with SFN (97%), followed by pain (85%), RLS (67%) and cognitive impairment (46%). Moreover, the severity of fatigue, pain, RLS and cognitive impairment was higher in patients with sarcoidosis with SFN compared with patients with sarcoidosis without SFN (resp. p=0.0006, p=0.003, p=0.02 and p=0.009). Finally, the FAS, RLS, VASmean, VASmax and CFQ showed a strong correlation with each other (R> 0.5, p<0.05).


Conclusions - Sarcoidosis patients with SFN showed an increased prevalence and higher severity of fatigue, pain, RLS and cognitive impairment compared to patients with sarcoidosis without SFN. Moreover, moderate to strong correlations were found between these symptoms.

Abstract 0 |

References

(1) E. D. Crouser, L. A. Maier, R. P. Baughman, et al., “Diagnosis and detection of sarcoidosis an official American Thoracic Society clinical practice guideline,” Am. J. Respir. Crit. Care Med., vol. 201, no. 8, pp. E26–E51, Apr. 2020, doi: 10.1164/RCCM.202002-0251ST.
(2) M. Drent, B. Strookappe, E. Hoitsma, et al., “Consequences of sarcoidosis,” Clin. Chest Med., vol. 36, no. 4, pp. 727–737, 2015, doi: 10.1016/j.ccm.2015.08.013.
(3) M. Drent, E. E. Lower, and J. De Vries, “Sarcoidosis-associated fatigue.,” Eur. Respir. J., vol. 40, no. 1, pp. 255–263, Jul. 2012, doi: 10.1183/09031936.00002512.
(4) J. Tavee and D. Culver, “Sarcoidosis and Small-fiber Neuropathy,” Curr. Pain Headache Reports, vol. 15, no. February, pp. 201–206, 2011, doi: 10.1007/s11916-011-0180-8.
(5) A. Hovaguimian and C. H. Gibbons, “Diagnosis and treatment of pain in small-fiber neuropathy,” Curr. Pain Headache Rep., vol. 15, no. 3, pp. 193–200, 2011, doi: 10.1007/s11916-011-0181-7.
(6) J. Tavee, “Peripheral neuropathy in sarcoidosis,” J. Neuroimmunol., vol. 368, no. January, p. 577864, 2022, doi: 10.1016/j.jneuroim.2022.577864.
(7) M. Voortman, C. M. R. Hendriks, M. D. P. Elfferich, et al., “The burden of sarcoidosis symptoms from a patient perspective,” Lung, vol. 197, no. 2, pp. 155–161, 2019, doi: 10.1007/s00408-019-00206-7.
(8) M. Voortman, J. De Vries, C. M. R. Hendriks, et al., “Everyday cognitive failure in patients suffering from neurosarcoidosis,” Sarcoidosis Vasc. Diffus. Lung Dis., vol. 36, no. 1, pp. 2–10, 2019, doi: 10.36141/svdld.v36i1.7412.
(9) M. D. Elfferich, P. J. Nelemans, R. W. Ponds, et al., “Everyday cognitive failure in sarcoidosis: The prevalence and the effect of anti-TNF-α treatment,” Respiration, vol. 80, no. 3, pp. 212–219, 2010, doi: 10.1159/000314225.
(10) S. Indart, J. Hugon, P. J. Guillausseau, et al., “Impact of pain on cognitive functions in primary Sjögren syndrome with small fiber neuropathy: 10 cases and a literature review,” Med. (United States), vol. 96, no. 16, pp. 1–6, 2017, doi: 10.1097/MD.0000000000006384.
(11) D. Patoulias and P. Keryttopoulos, “Diagnostic dilemma between sarcoidosis and primary Sjögren syndrome: mimicry, concomitance or coincidence? An up-to-date clinician’s perspective.,” Folia Med. Cracov., vol. 58, no. 1, pp. 5–23, 2018.
(12) R. P. Allen, D. Picchietti, W. A. Hening, et al., “Restless legs syndrome: diagnostic criteria, special considerations, and epidemiology. A report from the restless legs syndrome diagnosis and epidemiology workshop at the National Institutes of Health.,” Sleep Med., vol. 4, no. 2, pp. 101–119, Mar. 2003, doi: 10.1016/s1389-9457(03)00010-8.
(13) L. Abetz, R. Allen, A. Follet, et al., “Evaluating the quality of life of patients with restless legs syndrome,” Clin. Ther., vol. 26, no. 6, pp. 925–935, 2004, doi: 10.1016/S0149-2918(04)90136-1.
(14) W. P. E. De Kleijn, M. Drent, J. K. Vermunt, et al., “Types of fatigue in sarcoidosis patients,” J. Psychosom. Res., vol. 71, no. 6, pp. 416–422, 2011, doi: 10.1016/j.jpsychores.2011.09.006.
(15) J. Verbraecken, E. Hoitsma, C. P. M. Van Der Grinten, et al., “Sleep disturbances associated with periodic leg movements in chronic sarcoidosis,” Sarcoidosis Vasc. Diffus. Lung Dis., vol. 21, no. 2, pp. 137–146, 2004.
(16) M. Polydefkis, R. P. Allen, P. Hauer, et al., “Subclinical sensory neuropathy in late-onset restless legs syndrome.,” Neurology, vol. 55, no. 8, pp. 1115–1121, Oct. 2000, doi: 10.1212/wnl.55.8.1115.
(17) Y.-M. Lim, S.-E. Chang, S. Chung, et al., “Small fiber function in drug naïve patients with idiopathic restless legs syndrome.,” J. Clin. Neurosci. Off. J. Neurosurg. Soc. Australas., vol. 19, no. 5, pp. 702–705, May 2012, doi: 10.1016/j.jocn.2011.07.043.
(18) F. Gemignani, F. Brindani, F. Vitetta, et al., “Restless legs syndrome in diabetic neuropathy: a frequent manifestation of small fiber neuropathy.,” J. Peripher. Nerv. Syst., vol. 12, no. 1, pp. 50–53, Mar. 2007, doi: 10.1111/j.1529-8027.2007.00116.x.
(19) M. Andréasson, N. Lagali, R. A. Badian, et al., “Parkinson’s disease with restless legs syndrome-an in vivo corneal confocal microscopy study.,” NPJ Park. Dis., vol. 7, no. 1, p. 4, Jan. 2021, doi: 10.1038/s41531-020-00148-5.
(20) G. Devigili, S. Rinaldo, R. Lombardi, et al., “Diagnostic criteria for small fibre neuropathy in clinical practice and research,” Brain, vol. 142, no. 12, pp. 3728–3736, 2019, doi: 10.1093/brain/awz333.
(21) H. J. Michielsen, J. De Vries, and G. L. Van Heck, “Psychometric qualities of a brief self-rated fatigue measure: The Fatigue Assessment Scale,” J. Psychosom. Res., vol. 54, no. 4, pp. 345–352, 2003, doi: 10.1016/S0022-3999(02)00392-6.
(22) C. Hendriks, M. Drent, M. Elfferich, et al., “The Fatigue Assessment Scale: Quality and availability in sarcoidosis and other diseases,” Curr. Opin. Pulm. Med., vol. 24, no. 5, pp. 495–503, 2018, doi: 10.1097/MCP.0000000000000496.
(23) W. I. Campbell and S. Lewis, “Visual analogue measurement of pain.,” Ulster Med. J., vol. 59, no. 2, pp. 149–154, 1990.
(24) R. Rago, F. Forfori, G. Materazzi, et al., “Evaluation of a preoperative pain score in response to pressure as a marker of postoperative pain and drugs consumption in surgical thyroidectomy,” Clin. J. Pain, vol. 28, no. 5, pp. 382–386, 2012, doi: 10.1097/AJP.0b013e3182326495.
(25) A. S. Walters, C. LeBrocq, A. Dhar, et al., “Validation of the International Restless Legs Syndrome Study Group rating scale for restless legs syndrome,” Sleep Med., vol. 4, no. 2, pp. 121–132, 2003, doi: 10.1016/S1389-9457(02)00258-7.
(26) D. E. Broadbent, P. F. Cooper, P. FitzGerald, et al., “The cognitive failures questionnaire (CFQ) and its correlates,” Br. J. Clin. Psychol., vol. 21, no. 1, pp. 1–16, 1982, doi: 10.1111/j.2044-8260.1982.tb01421.x.
(27) R. Ponds, M. Van Boxtel, and J. Jolles, “De ‘Cognitive Failure Questionnaire’ Als Maat Voor Subjectief Cognitief Functioneren,” Tijdschr. voor Neuropsychol., vol. 2, no. 1, pp. 37–45, 2006.
(28) A. Singha, M. Kirkland, W. Drake, et al., “The influence of age and sex in sarcoidosis.,” Curr. Opin. Pulm. Med., vol. 28, no. 4, pp. 307–313, Jul. 2022, doi: 10.1097/MCP.0000000000000882.
(29) S. A. Johnson, K. Shouman, S. Shelly, et al., “Small fiber neuropathy incidence, prevalence, longitudinal impairments, and disability,” Neurology, vol. 97, no. 22, pp. E2236–E2247, 2021, doi: 10.1212/WNL.0000000000012894.
(30) J. De Vries, H. Michielsen, G. L. Van Heck, et al., “Measuring fatigue in sarcoidosis: the Fatigue Assessment Scale (FAS).,” Br. J. Health Psychol., vol. 9, no. Pt 3, pp. 279–291, Sep. 2004, doi: 10.1348/1359107041557048.
(31) M. H. Silber, M. J. Buchfuhrer, C. J. Earley, et al., “The Management of Restless Legs Syndrome: An Updated Algorithm,” Mayo Clin. Proc., vol. 96, no. 7, pp. 1921–1937, 2021, doi: 10.1016/j.mayocp.2020.12.026.
(32) M. R. L. A. D. V. J. Drent M, “Association between physical functions and quality of life in sarcoidosis.,” Sarcoidosis Vasc Diffus. Lung Dis, vol. 31, no. 2, pp. 117–28, Jul. 2014.
(33) N. Carrigan and E. Barkus, “A systematic review of cognitive failures in daily life: Healthy populations.,” Neurosci. Biobehav. Rev., vol. 63, pp. 29–42, Apr. 2016, doi: 10.1016/j.neubiorev.2016.01.010.
(34) S. Mackie and J. W. Winkelman, “Restless Legs Syndrome and Psychiatric Disorders,” Sleep Med. Clin., vol. 10, no. 3, pp. 351–357, 2015, doi: 10.1016/j.jsmc.2015.05.009.
(35) A. Guay, M. Houle, J. O’Shaughnessy, et al., “Current Evidence on Diagnostic Criteria, Relevant Outcome Measures, and Efficacy of Nonpharmacologic Therapy in the Management of Restless Legs Syndrome (RLS): A Scoping Review,” J. Manipulative Physiol. Ther., vol. 43, no. 9, pp. 930–941, 2020, doi: 10.1016/j.jmpt.2020.05.004.
(36) E. E. Lower, S. Harman, and R. P. Baughman, “Double-blind, randomized trial of dexmethylphenidate hydrochloride for the treatment of sarcoidosis-associated fatigue,” Chest, vol. 133, no. 5, pp. 1189–1195, 2008, doi: 10.1378/chest.07-2952.
(37) E. E. Lower, A. Malhotra, V. Surdulescu, et al., “Armodafinil for sarcoidosis-associated fatigue: A double-blind, placebo-controlled, crossover trial,” J. Pain Symptom Manage., vol. 45, no. 2, pp. 159–169, 2013, doi: 10.1016/j.jpainsymman.2012.02.016.
(38) R. Vis, E. M. W. van de Garde, B. Meek, et al., “Randomised, placebo-controlled trial of dexamethasone for quality of life in pulmonary sarcoidosis,” Respir. Med., vol. 165, Apr. 2020, doi: 10.1016/j.rmed.2020.105936.
(39) V. Kahlmann, C. C. Moor, S. J. van Helmondt, et al., “Online mindfulness-based cognitive therapy for fatigue in patients with sarcoidosis (TIRED): a randomised controlled trial,” Lancet Respir. Med., vol. 11, no. 3, pp. 265–272, 2023, doi: 10.1016/S2213-2600(22)00387-3.
(40) R. P. Allen, D. L. Picchietti, D. Garcia-Borreguero, et al., “Restless legs syndrome/Willis-Ekbom disease diagnostic criteria: Updated International Restless Legs Syndrome Study Group (IRLSSG) consensus criteria - history, rationale, description, and significance,” Sleep Med., vol. 15, no. 8, pp. 860–873, 2014, doi: 10.1016/j.sleep.2014.03.025.
(41) S. Tesfaye, A. J. M. Boulton, P. J. Dyck, et al., “Diabetic neuropathies: Update on definitions, diagnostic criteria, estimation of severity, and treatments,” Diabetes Care, vol. 33, no. 10, pp. 2285–2293, 2010, doi: 10.2337/dc10-1303.
(42) G. Devigili, V. Tugnoli, P. Penza, et al., “The diagnostic criteria for small fibre neuropathy: From symptoms to neuropathology,” Brain, vol. 131, no. 7, pp. 1912–1925, 2008, doi: 10.1093/brain/awn093.