Effects of the COVID-19 Pandemic in Patients with Systemic Autoinflammatory Diseases. An international survey
Main Article Content
Abstract
Background: Early in the COVID-19 pandemic it was recognized that individuals who had worse prognoses had hyperinflammatory reactions associated with SARS CoV-2. This was worrisome for patients with systemic autoinflammatory diseases (SAID) who already have episodic hyperinflammation at baseline.
Aim: To assess the effects of the COVID-19 pandemic and SARS CoV-2 infection on patients with SAID.
Methods: An anonymous survey developed on the Survey Monkey platform was distributed online through social media and support groups of patients with SAID between January 20th and May 17th of 2021.
Results: We analyzed 484 surveys that conveyed data from 593 patients with SAID. Seventy-four percent of the surveys were answered in the United States, US territories, and Canada (n=360/484) and the rest from other countries. Sixty percent (n=330/547) of patients with SAID did not experience changes in the frequency of flares during the first year of the pandemic. Ten percent (n=52/545) of patients had presumed or confirmed COVID-19. Of those, 81% (n=42/52) had mild and moderate symptoms and 4 were hospitalized. Thirty-one percent (n=16/52) experienced flares during or after the infection and 40% (n=21/52) reported post-COVID manifestations. The individuals who had moderate symptoms or were hospitalized for COVID-19 were more likely to report post-COVID manifestations, OR 5.25 (95%CI 1.57-17.6, p=0.0072). Post-COVID integumentary manifestations occurred after asymptomatic and mild COVID-19 whereas neurologic manifestations occurred after acute COVID-19 with moderate symptoms or requiring hospitalization.
Conclusion: During the COVID-19 pandemic, over half of patients with SAID did not experience changes in the frequency of flares despite a decreased exposure to environmental triggers. Few individuals with SAID required hospitalization for COVID-19. The presence and type of post-COVID manifestations were associated with the severity of acute COVID-19 in patients with SAID.
Article Details
How to Cite
NAZZAR, S et al.
Effects of the COVID-19 Pandemic in Patients with Systemic Autoinflammatory Diseases. An international survey.
Medical Research Archives, [S.l.], v. 12, n. 10, oct. 2024.
ISSN 2375-1924.
Available at: <https://esmed.org/MRA/mra/article/view/5990>. Date accessed: 15 nov. 2024.
doi: https://doi.org/10.18103/mra.v12i10.5990.
Section
Research Articles
The Medical Research Archives grants authors the right to publish and reproduce the unrevised contribution in whole or in part at any time and in any form for any scholarly non-commercial purpose with the condition that all publications of the contribution include a full citation to the journal as published by the Medical Research Archives.
References
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20. Walker UA, Luetke Volksbeck S, Kuemmerle-Deschner J. Flare of a Cryopyrin-associated Periodic Syndrome Following Vaccination with Neisseria Meningitidis Polysaccharides. J Rheumatol. 2018;45(6):878-879. doi:10.3899/jrheum.171037
21. Jeyaratnam J, Ter Haar NM, Lachmann HJ, et al. The safety of live-attenuated vaccines in patients using IL-1 or IL-6 blockade: an international survey. Pediatr Rheumatol Online J. 2018;16(1):19. Published 2018 Mar 21. doi:10.1186/s12969-018-0235-z
22. Park SY, Lee KH. Adult-onset Still's Disease after BNT162b2 mRNA COVID-19 Vaccine. J Korean Med Sci. 2021;36(50):e344. Published 2021 Dec 27. doi:10.3346/jkms.2021.36.e344
23. Flare-up of adult-onset Still's disease after receiving a second dose of BNT162b2 COVID-19 mRNA vaccine
24. Peet CJ, Papadopoulou C, Sombrito BRM, Wood MR, Lachmann HJ. COVID-19 and autoinflammatory diseases: prevalence and outcomes of infection and early experience of vaccination in patients on biologics. Rheumatol Adv Pract. 2021;5(2):rkab043. Published 2021 Aug 23.
25. Grasland A, Le Maître F, Pouchot J, Hazera P, Bazin C, Vinceneux P. Maladie de Still de l'adulte après vaccination contre l'hépatite A et B? [Adult-onset Still's disease after hepatitis A and B vaccination?]. Rev Med Interne. 1998;19(2):134-136. doi:10.1016/s0248-8663(97)83425-x
26. Padgett, Christina. “Recurrence of Symptoms Associated with Menstruation in a Patient with a History of Periodic Fevers.” Journal of pediatric and adolescent gynecology vol. 33,4 (2020): 429-431. doi:10.1016/j.jpag.2020.03.008
27. Guzelant, Gul et al. “Exacerbation of Behçet's syndrome and familial Mediterranean fever with menstruation.” Clinical and experimental rheumatology vol. 35 Suppl 108,6 (2017): 95-99.
28. Akar S, Soyturk M, Onen F, Tunca M. The relations between attacks and menstrual periods and pregnancies of familial Mediterranean fever patients. Rheumatol Int. 2006;26(7):676-679. doi:10.1007/s00296-005-0041-z
29. Kumei, Shima et al. “Cold Exposure Related Fever with an Mediterranean Fever (MEFV) Gene Mutation.” Internal medicine (Tokyo, Japan) vol. 56,16 (2017): 2233-2236. doi:10.2169/internalmedicine.8274-16
30. Sharma M, Ferguson PJ. Autoinflammatory bone disorders: update on immunologic abnormalities and clues about possible triggers. Curr Opin Rheumatol. 2013;25(5):658-664. doi:10.1097/BOR.0b013e328363eb08
31. Nakashimai, Hideyuki et al. “Febrile attacks triggered by milk allergy in an infant with mevalonate kinase deficiency.” Rheumatology international vol. 36,10 (2016): 1477-8. doi:10.1007/s00296-016-3522-3
32. Mansfield L, Lapidus S, Nazzar S, Moorthy LN, Adler-Shohet F, Hollander M et al. Increase in pediatric recurrent fever evaluations during the first year of the COVID-19 pandemic in North America. Front. Pediatr. 2023; 11
33. Manson JJ, Crooks C, Naja M, Ledlie A, Goulden B, Liddle T et al. COVID-19-associated hyperinflammation and escalation of patient care: a retrospective longitudinal cohort study. Lancet Rheumatol. 2020; 2: 594-602.
34. MedCalc Software Ltd. Odds ratio calculator. https://www.medcalc.org/calc/odds_ratio.php (Version 22.009; accessed August 8, 2023)
35. Levinsky Y, Butbul Aviel Y, Ahmad SA, Broide M, Gendler Y, Dagan N, et al. PFAPA flares observed during COVID outbreak: can emotional stress trigger PFAPA attacks? A multicenter cohort study. Pediatr Rheumatol Online J. 2022;20(1):46.
36. Wu N, Zhao M, Wu D, Yu K, Shen M. COVID-19 pandemic and systemic autoinflammatory diseases management: a cross-sectional survey. Rheumatol Int. 2021; 41: 1541-1543
37. Haslak F, Yildiz M, Adrovic A, Sahin S, Koker O, Aliyeva A, et al. Management of childhood-onset autoinflammatory diseases during the COVID-19 pandemic. Rheumatol Int. 2020; 40: 1423-1431.
38. Pablos JL, Galindo M, Carmona L, Lledó A, Retuerto M, Blanco R, et al. Clinical outcomes of hospitalised patients with COVID-19 and chronic inflammatory and autoimmune rheumatic diseases: a multicentric matched cohort study. Ann Rheum Dis. 2020; 79: 1544-1549.
39. Bourguiba R, Delplanque M, Vinit C, Ackermann F, Savey L, Grateau G, et al. Clinical course of COVID-19 in a cohort of 342 familial mediterranean fever patients with a long-term treatment by colchicine in a french endemic area. Ann Rheum Dis. 2021; 80: 539-540.
40. Welzel T, Samba SD, Klein R, van den Anker JN, Kuemmerle-Deschner JB. COVID-19 in autoinflammatory diseases with immunosuppressive treatment. J Clin Med. 2021; 10: 605
41. FAI2R /SFR/SNFMI/SOFREMIP/CRI/IMIDIATE consortium and contributors. Severity of COVID-19 and survival in patients with rheumatic and inflammatory diseases: data from the french RMD COVID-19 cohort of 694 patients. Ann Rheum Dis. 2020; 80: 527-538.
42. Meyts I, Bucciol G, Quinti I, et al. Coronavirus disease 2019 in patients with inborn errors of immunity: An international study. J Allergy Clin Immunol. 2021;147(2):520-531. doi:10.1016/j.jaci.2020.09.010
43. Shields AM, Burns SO, Savic S, Richter AG; UK PIN COVID-19 Consortium. COVID-19 in patients with primary and secondary immunodeficiency: The United Kingdom experience. J Allergy Clin Immunol. 2021;147(3):870-875.e1. doi:10.1016/j.jaci.2020.12.620
44. Alhumaid S, Al Mutared KM, Al Alawi Z, et al. Severity of SARS-CoV-2 infection in children with inborn errors of immunity (primary immunodeficiencies): a systematic review. Allergy Asthma Clin Immunol. 2023; 19: 69
45. Davis HE, Assaf GS, McCorkell L, et al. Characterizing long COVID in an international cohort: 7 months of symptoms and their impact. EClinicalMedicine. 2021; 38: 101019.
46. Taquet M, Dercon Q, Luciano S, Geddes JR, Husain M, Harrison PJ. Incidence, co-occurrence, and evolution of long-COVID features: a 6-month retrospective cohort study of 273,618 survivors of COVID-19. PLoS Med. 2021; 18: 1003773.
2. van der Hilst, Jeroen C H et al. “Long-term follow-up, clinical features, and quality of life in a series of 103 patients with hyperimmunoglobulinemia D syndrome.” Medicine vol. 87,6 (2008): 301-310. doi:10.1097/MD.0b013e318190cfb7
3. Kishida, Dai et al. “Triggering factors for febrile attacks in Japanese patients with familial Mediterranean fever.” Clinical and experimental rheumatology vol. 38 Suppl 127,5 (2020): 76-79.
4. Shenavandeh S, Asis M, Eftekhari MH, et al. The Patients' Beliefs Regarding the Role of Food, Mucosal Trauma, Menstruation, and Psychological Stress in the Recurrence of Behçet's Disease Symptoms. J Med Life. 2020;13(2):164-169. doi:10.25122/jml-2019-0153
5. Karadag O, Tufan A, Yazisiz V, et al. The factors considered as trigger for the attacks in patients with familial Mediterranean fever. Rheumatol Int. 2013;33(4):893-897. doi:10.1007/s00296-012-2453-x
6. Schwabe, Peters. Familial Mediterranean Fever in Armenians. Analysis of 100 cases. Medicine 1974;53(6):453
7. G. Yenokyan, H.K. Armenian. Triggers for attacks in Familial Mediterranean Fever: application of the case-crossover design. Am J Epidemiol, 175 (2012), pp. 1054-1061
8. Avagyan, Amaryan et al. Influence of Some Environmental Factors on Manifestation of Familial Mediterranean Fever in Children: Clinical and Genetic Aspects. Caucasus Journal of Health Sciences and Public Health, June 2018;2(2)
9. Korkmaz, Cengiz et al. “Familial Mediterranean fever: the molecular pathways from stress exposure to attacks.” Rheumatology (Oxford, England) vol. 59,12 (2020): 3611-3621. doi:10.1093/rheumatology/keaa450
10. Volle G, Fraison JB, Gobert D, et al. Dietary and Nondietary Triggers of Oral Ulcer Recurrences in Behçet's Disease. Arthritis Care Res (Hoboken). 2017;69(9):1429-1436. doi:10.1002/acr.23155
11. Lachmann HJ, Papa R, Gerhold K, et al. The phenotype of TNF receptor-associated autoinflammatory syndrome (TRAPS) at presentation: a series of 158 cases from the Eurofever/EUROTRAPS international registry. Ann Rheum Dis. 2014;73(12):2160-2167. doi:10.1136/annrheumdis-2013-204184
12. Maggio, Maria Cristina, and Giovanni Corsello. “FMF is not always "fever": from clinical presentation to "treat to target".” Italian journal of pediatrics vol. 46,1 7. 15 Jan. 2020, doi:10.1186/s13052-019-0766-z
13. Christiansen, Mette et al. “XIAP deficiency and MEFV variants resulting in an autoinflammatory lymphoproliferative syndrome.” BMJ case reports vol. 2016 bcr2016216922. 28 Sep. 2016, doi:10.1136/bcr-2016-216922.
14. Stoffels, Monique et al. “TLR2/TLR4-dependent exaggerated cytokine production in hyperimmunoglobulinaemia D and periodic fever syndrome.” Rheumatology (Oxford, England) vol. 54,2 (2015): 363-8. doi:10.1093/rheumatology/keu341
15. Jia J, Shi H, Liu M, et al. Cytomegalovirus Infection May Trigger Adult-Onset Still's Disease Onset or Relapses. Front Immunol. 2019;10:898. Published 2019 Apr 24. doi:10.3389/fimmu.2019.00898
16. Yu X, Zheng H. Refractory Genital HPV Infection and Adult-Onset Still Disease: A Case Report and Literature Review. Medicine (Baltimore). 2016;95(15):e3169. doi:10.1097/MD.0000000000003169
17. Torres-Aguilar H, Sosa-Luis SA, Aguilar-Ruiz SR. Infections as triggers of flares in systemic autoimmune diseases: novel innate immunity mechanisms. Curr Opin Rheumatol. 2019;31(5):525-531. doi:10.1097/BOR.0000000000000630
18. Ozcifci G, Aydin T, Atli Z, et al. The incidence, clinical characteristics, and outcome of COVID-19 in a prospectively followed cohort of patients with Behçet's syndrome. Rheumatol Int. 2022;42(1):101-113. doi:10.1007/s00296-021-05056-2
19. McPeake JR, Hirst WJ, Brind AM, Williams R. Hepatitis A causing a second episode of virus-associated haemophagocytic lymphohistiocytosis in a patient with Still's disease. J Med Virol. 1993;39(2):173-175. doi:10.1002/jmv.1890390216
20. Walker UA, Luetke Volksbeck S, Kuemmerle-Deschner J. Flare of a Cryopyrin-associated Periodic Syndrome Following Vaccination with Neisseria Meningitidis Polysaccharides. J Rheumatol. 2018;45(6):878-879. doi:10.3899/jrheum.171037
21. Jeyaratnam J, Ter Haar NM, Lachmann HJ, et al. The safety of live-attenuated vaccines in patients using IL-1 or IL-6 blockade: an international survey. Pediatr Rheumatol Online J. 2018;16(1):19. Published 2018 Mar 21. doi:10.1186/s12969-018-0235-z
22. Park SY, Lee KH. Adult-onset Still's Disease after BNT162b2 mRNA COVID-19 Vaccine. J Korean Med Sci. 2021;36(50):e344. Published 2021 Dec 27. doi:10.3346/jkms.2021.36.e344
23. Flare-up of adult-onset Still's disease after receiving a second dose of BNT162b2 COVID-19 mRNA vaccine
24. Peet CJ, Papadopoulou C, Sombrito BRM, Wood MR, Lachmann HJ. COVID-19 and autoinflammatory diseases: prevalence and outcomes of infection and early experience of vaccination in patients on biologics. Rheumatol Adv Pract. 2021;5(2):rkab043. Published 2021 Aug 23.
25. Grasland A, Le Maître F, Pouchot J, Hazera P, Bazin C, Vinceneux P. Maladie de Still de l'adulte après vaccination contre l'hépatite A et B? [Adult-onset Still's disease after hepatitis A and B vaccination?]. Rev Med Interne. 1998;19(2):134-136. doi:10.1016/s0248-8663(97)83425-x
26. Padgett, Christina. “Recurrence of Symptoms Associated with Menstruation in a Patient with a History of Periodic Fevers.” Journal of pediatric and adolescent gynecology vol. 33,4 (2020): 429-431. doi:10.1016/j.jpag.2020.03.008
27. Guzelant, Gul et al. “Exacerbation of Behçet's syndrome and familial Mediterranean fever with menstruation.” Clinical and experimental rheumatology vol. 35 Suppl 108,6 (2017): 95-99.
28. Akar S, Soyturk M, Onen F, Tunca M. The relations between attacks and menstrual periods and pregnancies of familial Mediterranean fever patients. Rheumatol Int. 2006;26(7):676-679. doi:10.1007/s00296-005-0041-z
29. Kumei, Shima et al. “Cold Exposure Related Fever with an Mediterranean Fever (MEFV) Gene Mutation.” Internal medicine (Tokyo, Japan) vol. 56,16 (2017): 2233-2236. doi:10.2169/internalmedicine.8274-16
30. Sharma M, Ferguson PJ. Autoinflammatory bone disorders: update on immunologic abnormalities and clues about possible triggers. Curr Opin Rheumatol. 2013;25(5):658-664. doi:10.1097/BOR.0b013e328363eb08
31. Nakashimai, Hideyuki et al. “Febrile attacks triggered by milk allergy in an infant with mevalonate kinase deficiency.” Rheumatology international vol. 36,10 (2016): 1477-8. doi:10.1007/s00296-016-3522-3
32. Mansfield L, Lapidus S, Nazzar S, Moorthy LN, Adler-Shohet F, Hollander M et al. Increase in pediatric recurrent fever evaluations during the first year of the COVID-19 pandemic in North America. Front. Pediatr. 2023; 11
33. Manson JJ, Crooks C, Naja M, Ledlie A, Goulden B, Liddle T et al. COVID-19-associated hyperinflammation and escalation of patient care: a retrospective longitudinal cohort study. Lancet Rheumatol. 2020; 2: 594-602.
34. MedCalc Software Ltd. Odds ratio calculator. https://www.medcalc.org/calc/odds_ratio.php (Version 22.009; accessed August 8, 2023)
35. Levinsky Y, Butbul Aviel Y, Ahmad SA, Broide M, Gendler Y, Dagan N, et al. PFAPA flares observed during COVID outbreak: can emotional stress trigger PFAPA attacks? A multicenter cohort study. Pediatr Rheumatol Online J. 2022;20(1):46.
36. Wu N, Zhao M, Wu D, Yu K, Shen M. COVID-19 pandemic and systemic autoinflammatory diseases management: a cross-sectional survey. Rheumatol Int. 2021; 41: 1541-1543
37. Haslak F, Yildiz M, Adrovic A, Sahin S, Koker O, Aliyeva A, et al. Management of childhood-onset autoinflammatory diseases during the COVID-19 pandemic. Rheumatol Int. 2020; 40: 1423-1431.
38. Pablos JL, Galindo M, Carmona L, Lledó A, Retuerto M, Blanco R, et al. Clinical outcomes of hospitalised patients with COVID-19 and chronic inflammatory and autoimmune rheumatic diseases: a multicentric matched cohort study. Ann Rheum Dis. 2020; 79: 1544-1549.
39. Bourguiba R, Delplanque M, Vinit C, Ackermann F, Savey L, Grateau G, et al. Clinical course of COVID-19 in a cohort of 342 familial mediterranean fever patients with a long-term treatment by colchicine in a french endemic area. Ann Rheum Dis. 2021; 80: 539-540.
40. Welzel T, Samba SD, Klein R, van den Anker JN, Kuemmerle-Deschner JB. COVID-19 in autoinflammatory diseases with immunosuppressive treatment. J Clin Med. 2021; 10: 605
41. FAI2R /SFR/SNFMI/SOFREMIP/CRI/IMIDIATE consortium and contributors. Severity of COVID-19 and survival in patients with rheumatic and inflammatory diseases: data from the french RMD COVID-19 cohort of 694 patients. Ann Rheum Dis. 2020; 80: 527-538.
42. Meyts I, Bucciol G, Quinti I, et al. Coronavirus disease 2019 in patients with inborn errors of immunity: An international study. J Allergy Clin Immunol. 2021;147(2):520-531. doi:10.1016/j.jaci.2020.09.010
43. Shields AM, Burns SO, Savic S, Richter AG; UK PIN COVID-19 Consortium. COVID-19 in patients with primary and secondary immunodeficiency: The United Kingdom experience. J Allergy Clin Immunol. 2021;147(3):870-875.e1. doi:10.1016/j.jaci.2020.12.620
44. Alhumaid S, Al Mutared KM, Al Alawi Z, et al. Severity of SARS-CoV-2 infection in children with inborn errors of immunity (primary immunodeficiencies): a systematic review. Allergy Asthma Clin Immunol. 2023; 19: 69
45. Davis HE, Assaf GS, McCorkell L, et al. Characterizing long COVID in an international cohort: 7 months of symptoms and their impact. EClinicalMedicine. 2021; 38: 101019.
46. Taquet M, Dercon Q, Luciano S, Geddes JR, Husain M, Harrison PJ. Incidence, co-occurrence, and evolution of long-COVID features: a 6-month retrospective cohort study of 273,618 survivors of COVID-19. PLoS Med. 2021; 18: 1003773.