Skip to main content
SpringerLink
Log in

Autoinflammatory Diseases in the Geriatric Population

  • Chapter
  • First Online:
Rheumatic Disease in Geriatrics

  • 573 Accesses

Abstract

Autoinflammatory disorders are inherited diseases of innate immunity leading to uncontrolled activation of the interleukin-1 pathway. The disorders are characterized by unprovoked recurrent inflammation affecting primarily the skin, as well as the serosal and synovial membranes. As innate immunity is the culprit, there are no autoantibodies or autoreactive T-cells to aid in the diagnosis and reactive amyloidosis is a potential severe long-term complication [1, 2].

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 69.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 89.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 109.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

Bibliography

  1. Ricci P, Stella A, Settimo E, et al. The grandfather’ s fever. 2019.

    Google Scholar 

  2. Russo RAG, Katsicas MM. Autoinflammatory diseases. Medicina (B Aires). 2016;76(3):166–72. https://doi.org/10.1016/j.imlet.2013.12.013.

    Article  CAS  Google Scholar 

  3. Fonnesu C, Cerquaglia C, Giovinale M, et al. Familial Mediterranean fever: a review for clinical management. Jt Bone Spine. 2009;76(3):227–33. https://doi.org/10.1016/j.jbspin.2008.08.004.

    Article  Google Scholar 

  4. Livneh A, Langevitz P, Zemer D, et al. Criteria for the diagnosis of familial Mediterranean fever. Arthritis Rheum. 1997;40(10):1879–85. https://doi.org/10.1002/art.1780401023.

    Article  CAS  PubMed  Google Scholar 

  5. Consortium FF. A candidate gene for familial Mediterranean fever. Nat Genet. 1997;17(1):25–31. https://doi.org/10.1038/ng0997-25.

    Article  Google Scholar 

  6. Ben-Chetrit E, Touitou I. Familial mediterranean fever in the world. Arthritis Care Res. 2009;61:1447–53. https://doi.org/10.1002/art.24458.

    Article  CAS  Google Scholar 

  7. Samuels J, Aksentijevich I, Torosyan Y, et al. Familial Mediterranean fever at the millennium clinical spectrum, ancient mutations, and a survey of 100 American referrals to the national institutes of health. Medicine (Baltimore). 1998;77:268–97. https://doi.org/10.1097/00005792-199807000-00005.

    Article  CAS  Google Scholar 

  8. Touitou I. The spectrum of familial Mediterranean fever (FMF) mutations. Eur J Hum Genet. 2001;9(7):473–83. https://doi.org/10.1038/sj.ejhg.5200658.

    Article  CAS  PubMed  Google Scholar 

  9. Migita K, Uehara R. Nakamura Y, et al, vol. 91. Med (United States): Familial mediterranean fever in Japan; 2012. p. 337–43. https://doi.org/10.1097/MD.0b013e318277cf75.

    Book  Google Scholar 

  10. Grossman C, Kassel Y, Livneh A, Ben-Zvi I. Familial Mediterranean fever (FMF) phenotype in patients homozygous to the MEFV M694V mutation. Eur J Med Genet. 2019;62(6):103532. https://doi.org/10.1016/j.ejmg.2018.08.013.

    Article  PubMed  Google Scholar 

  11. Ben-Chetrit E, Lerer I, Malamud E, Domingo C, Abeliovich D. The E148Q mutation in the MEFV gene: is it a disease-causing mutation or a sequence variant? Hum Mutat. 2000;15:385–6. https://doi.org/10.1002/(SICI)1098-1004(200004)15:4<385::AID-HUMU22>3.0.CO;2-A.

    Article  CAS  PubMed  Google Scholar 

  12. Tamir N, Langevitz P, Zemer D, et al. Late-onset familial Mediterranean fever (FMF): a subset with distinct clinical, demographic, and molecular genetic characteristics. Am J Med Genet. 1999;87:30–5. https://doi.org/10.1002/(SICI)1096-8628(19991105)87:1<30::AID-AJMG6>3.0.CO;2-B.

    Article  CAS  PubMed  Google Scholar 

  13. Sayarlioglu M, Cefle A, Inanc M, et al. Characteristics of patients with adult-onset familial Mediterranean fever in Turkey: analysis of 401 cases. Int J Clin Pract. 2005;59:202–5. https://doi.org/10.1111/j.1742-1241.2004.00294.x.

    Article  CAS  PubMed  Google Scholar 

  14. Ozdogan H, Ugurlu S. Familial Mediterranean fever. Press Medicale. 2019;48(1):e61–76. https://doi.org/10.1016/j.lpm.2018.08.014.

    Article  Google Scholar 

  15. Kriegshäuser G, Enko D, Hayrapetyan H, Atoyan S, Oberkanins C, Sarkisian T. Clinical and genetic heterogeneity in a large cohort of Armenian patients with late-onset familial Mediterranean fever. Genet Med. 2018;20:1583–8. https://doi.org/10.1038/gim.2018.46.

    Article  PubMed  Google Scholar 

  16. Zemer D, Revach M, Pras M, et al. A controlled trial of colchicine in preventing attacks of familial Mediterranean fever. N Engl J Med. 1974;291:932–4. https://doi.org/10.1056/NEJM197410312911803.

    Article  CAS  PubMed  Google Scholar 

  17. Wason S, Faulkner RD, Davis MW. Are dosing adjustments required for colchicine in the elderly compared with younger patients? Adv Ther. 2012;29(6):551–61. https://doi.org/10.1007/s12325-012-0028-6.

    Article  CAS  PubMed  Google Scholar 

  18. Leibovitz A, Lidar M, Baumoehl Y, Livneh A, Segal R. Colchicine therapy and the coginitive status of elderly patients with familial Mediterranean fever. Isr Med Assoc J. 2006;8(7):469–72.

    CAS  PubMed  Google Scholar 

  19. Ter Haar N, Lachmann H, Özen S, et al. Treatment of autoinflammatory diseases: results from the Eurofever Registry and a literature review. Ann Rheum Dis. 2013;72:678–85. https://doi.org/10.1136/annrheumdis-2011-201268.

    Article  PubMed  Google Scholar 

  20. Ben-Chetrit E, Backenroth R. Amyloidosis induced, end stage renal disease in patients with familial Mediterranean fever is highly associated with point mutations in the MEFV gene. Ann Rheum Dis. 2001;60(2):146–9. https://doi.org/10.1136/ard.60.2.146.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Varan O, Kucuk H, Babaoglu H, et al. Chronic inflammation in adult familial Mediterranean fever patients: underlying causes and association with amyloidosis. Scand J Rheumatol. 2019;48(4):315–9. https://doi.org/10.1080/03009742.2018.1558282.

    Article  CAS  PubMed  Google Scholar 

  22. Shinar Y, Tohami T, Livneh A, et al. Acquired familial Mediterranean fever associated with a somatic MEFV mutation in a patient with JAK2 associated post-polycythemia myelofibrosis. Orphanet J Rare Dis. 2015;10(1):1–6. https://doi.org/10.1186/s13023-015-0298-6.

    Article  Google Scholar 

  23. Stoffels M, Kastner DL. Old dogs, new tricks: monogenic autoinflammatory disease unleashed. Annu Rev Genomics Hum Genet. 2016;17(1):245–72. https://doi.org/10.1146/annurev-genom-090413-025334.

    Article  CAS  PubMed  Google Scholar 

  24. Dodé C, André M, Bienvenu T, et al. The enlarging clinical, genetic, and population spectrum of tumor necrosis factor receptor-associated periodic syndrome. Arthritis Rheum. 2002;46(8):2181–8. https://doi.org/10.1002/art.10429.

    Article  CAS  PubMed  Google Scholar 

  25. McDermott MF, Aksentijevich I, Galon J, et al. Germline mutations in the extracellular domains of the 55 kDa TNF receptor, TNFR1, define a family of dominantly inherited autoinflammatory syndromes. Cell. 1999;97:133–44. https://doi.org/10.1016/S0092-8674(00)80721-7.

    Article  CAS  PubMed  Google Scholar 

  26. Bazzoni F, Beutler B. The tumor necrosis factor ligand and receptor families. N Engl J Med. 1996;334:1717–25. https://doi.org/10.1056/NEJM199606273342607.

    Article  CAS  PubMed  Google Scholar 

  27. Aganna E, Martinon F, Hawkins PN, et al. Association of mutations in the NALP3/CIAS1/PYPAF1 gene with a broad phenotype including recurrent fever, cold sensitivity, sensorineural deafness, and AA amyloidosis. Arthritis Rheum. 2002;46:2445–52. https://doi.org/10.1002/art.10509.

    Article  CAS  PubMed  Google Scholar 

  28. 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;73:2160–7. https://doi.org/10.1136/annrheumdis-2013-204184.

    Article  CAS  PubMed  Google Scholar 

  29. Alexandra J, Dode C, Papo T, Jacobelli S, Andre M. Failure of anti-TNF therapy in TNF-receptor-1-associated periodic syndrome (TRAPS). Rheumatology (Oxford). 2007;46(7):1211–2. https://doi.org/10.1093/rheumatology/kel298.

    Article  CAS  PubMed  Google Scholar 

  30. Ravet N, Rouaghe S, Dodé C, et al. Clinical significance of P46L and R92Q substitutions in the tumour necrosis factor superfamily 1A gene. Ann Rheum Dis. 2006;65:1158–62. https://doi.org/10.1136/ard.2005.048611.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Cantarini L, Rigante D, Merlini G, et al. The expanding spectrum of low-penetrance TNFRSF1A gene variants in adults presenting with recurrent inflammatory attacks: clinical manifestations and long-term follow-up. Semin Arthritis Rheum. 2014;43(6):818–23. https://doi.org/10.1016/j.semarthrit.2013.12.002.

    Article  CAS  PubMed  Google Scholar 

  32. Menon SG, Efthimiou P. Tumor necrosis factor-associated periodic syndrome in adults. Rheumatol Int. 2018;38(1):3–11. https://doi.org/10.1007/s00296-017-3820-4.

    Article  PubMed  Google Scholar 

  33. Kontzias A, Zarabi SK, Calabrese C, et al. Somatic mosaicism in adult-onset TNF receptor-associated periodic syndrome (TRAPS). Mol Genet Genomic Med. 2019;7(8):3–7. https://doi.org/10.1002/mgg3.791.

    Article  CAS  Google Scholar 

  34. Cantarini L, Rigante D, Lucherini OM, et al. Role of etanercept in the treatment of tumor necrosis factor receptor-associated periodic syndrome: personal experience and review of the literature. Int J Immunopathol Pharmacol. 2010;23:701–7. https://doi.org/10.1177/039463201002300303.

    Article  CAS  PubMed  Google Scholar 

  35. Nedjai B, Hitman GA, Quillinan N, et al. Proinflammatory action of the antiinflammatory drug infliximab in tumor necrosis factor receptor-associated periodic syndrome. Arthritis Rheum. 2009;60:619–25. https://doi.org/10.1002/art.24294.

    Article  CAS  PubMed  Google Scholar 

  36. Bulua AC, Mogul DB, Aksentijevich I, et al. Efficacy of etanercept in the tumor necrosis factor receptor-associated periodic syndrome: a prospective, open-label, dose-escalation study. Arthritis Rheum. 2012;64:908–13. https://doi.org/10.1002/art.33416.

    Article  CAS  PubMed  Google Scholar 

  37. Brizi MG, Galeazzi M, Lucherini OM, Cantarini L, Cimaz R. Successful treatment of tumor necrosis factor receptor-associated periodic syndrome with canakinumab. Ann Intern Med. 2012;156:907–8. https://doi.org/10.7326/0003-4819-156-12-201206190-00027.

    Article  PubMed  Google Scholar 

  38. Vaitla PM, Radford PM, Tighe PJ, et al. Role of interleukin-6 in a patient with tumor necrosis factor receptor-associated periodic syndrome: assessment of outcomes following treatment with the anti-interleukin-6 receptor monoclonal antibody tocilizumab. Arthritis Rheum. 2011;63:1151–5. https://doi.org/10.1002/art.30215.

    Article  PubMed  Google Scholar 

  39. International Study Group for Behçet’s Disease. Criteria for diagnosis of Behcet’s disease. Lancet. 1990. https://doi.org/10.1016/0140-6736(90)92643-V.

  40. Gürler A, Boyvat A, Türsen Ü. Clinical manifestations of Behçet’s disease: an analysis of 2147 patients. Yonsei Med J. 1997;38:423–7. https://doi.org/10.3349/ymj.1997.38.6.423.

    Article  PubMed  Google Scholar 

  41. Zouboulis CC, Vaiopoulos G, Marcomichelakis N, et al. Onset signs, clinical course, prognosis, treatment and outcome of adult patients with Adamantiades-Behçet’s disease in Greece. Clin Exp Rheumatol. 2003;21(4 Suppl 30):S19–26.

    CAS  PubMed  Google Scholar 

  42. Sungur G, Hazirolan D, Hekimoglu E, Kasim R, Duman S. Late-onset Behçet’s disease: demographic, clinical, and ocular features. Graefes Arch Clin Exp Ophthalmol. 2010;248(9):1325–30. https://doi.org/10.1007/s00417-010-1399-5.

    Article  PubMed  Google Scholar 

  43. Cheon JH, Kim WH. An update on the diagnosis, treatment, and prognosis of intestinal Behçet’s disease. Curr Opin Rheumatol. 2015;27:24–31. https://doi.org/10.1097/BOR.0000000000000125.

    Article  CAS  PubMed  Google Scholar 

  44. Davatchi F, Chams-Davatchi C, Ghodsi Z, et al. Diagnostic value of pathergy test in Behcet’s disease according to the change of incidence over the time. Clin Rheumatol. 2011;30:1151–5. https://doi.org/10.1007/s10067-011-1694-5.

    Article  PubMed  Google Scholar 

  45. Davatchi F, Shahram F, Chams-Davatchi C, et al. Behcet’s disease: from east to west. Clin Rheumatol. 2010;29:823–33. https://doi.org/10.1007/s10067-010-1430-6.

    Article  PubMed  Google Scholar 

  46. Cho S, Cho S, Bang D. New insights in the clinical understanding of behçet’s disease. Yonsei Med J. 2012;53(1):35–42. https://doi.org/10.3349/ymj.2012.53.1.35.

    Article  CAS  PubMed  Google Scholar 

  47. Zouboulis CC. Epidemiology of Adamantiades-Behcet’s disease. Ann Med Interne (Paris). 1999;150(6):488–98.

    CAS  Google Scholar 

  48. Ziadé N, Awada H. Late onset Behçet’s disease. Joint Bone Spine. 2006;73(5):567–9. https://doi.org/10.1016/j.jbspin.2006.06.002.

    Article  PubMed  Google Scholar 

  49. 山口昌子, 横田憲治. ベーチェット病とStreptococcus sanguisとの関係. 札幌医学雑誌 = Sapporo Med J. 1991;60(4):331–338.

    Google Scholar 

  50. Wallace GR, Niemczyk E. Genetics in ocular inflammation-basic principles. Ocul Immunol Inflamm. 2011;19:10–8. https://doi.org/10.3109/09273948.2010.543306.

    Article  CAS  PubMed  Google Scholar 

  51. Yilmaz S, Cimen KA. Familial Behçet’s disease. Rheumatol Int. 2010;30:1107–9. https://doi.org/10.1007/s00296-009-1036-y.

    Article  PubMed  Google Scholar 

  52. Koné-Paut I, Geisler I, Wechsler B, et al. Familial aggregation in Behcet’s disease: high frequency in siblings and parents of pediatric probands. J Pediatr. 1999;135:89–93. https://doi.org/10.1016/S0022-3476(99)70333-1.

    Article  PubMed  Google Scholar 

  53. Shen Y, Ma HF, Luo D, Cai JF, Zou J, Guan JL. High incidence of gastrointestinal ulceration and cytogenetic aberration of trisomy 8 as typical features of Behçet’s disease associated with myelodysplastic syndrome: a series of 16 consecutive Chinese patients from the Shanghai Behçet’s disease database. Biomed Res Int. 2018;2018:1–8. https://doi.org/10.1155/2018/8535091.

    Article  CAS  Google Scholar 

  54. Koguchi-Yoshioka H, Inokuma D, Kanda M, Kondo M, Kikuchi K, Shimizu S. Behçet’s disease-like symptoms associated with myelodysplastic syndrome with trisomy 8: a case report and review of the literature. Acta Derm Venereol. 2014;94(3):355–6. https://doi.org/10.2340/00015555-1706.

    Article  PubMed  Google Scholar 

  55. Usselman CWNSSJRB. 乳鼠心肌提取 HHS public access. Physiol Behav. 2017;176(3):139–48. https://doi.org/10.1016/j.physbeh.2017.03.040.

    Article  CAS  Google Scholar 

  56. Wouters JM, van der Veen J, van de Putte LB, de Rooij DJ. Adult onset Still’s disease and viral infections. Ann Rheum Dis. 1988;47:764–7. https://doi.org/10.1136/ard.47.9.764.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  57. Chen DY, Chen YM, Lan JL, Tzang BS, Lin CC, Hsu TC. Significant association of past parvovirus B19 infection with cytopenia in both adult-onset Still’s disease and systemic lupus erythematosus patients. Clin Chim Acta. 2012;413:855–60. https://doi.org/10.1016/j.cca.2012.01.027.

    Article  CAS  PubMed  Google Scholar 

  58. Fujii T. Cytokine and immunogenetic profiles in Japanese patients with adult Still’s disease. Association with chronic articular disease Rheumatology. 2001;40:1398–404. https://doi.org/10.1093/rheumatology/40.12.1398.

    Article  CAS  PubMed  Google Scholar 

  59. Choi JH, Suh CH, Lee YM, et al. Serum cytokine profiles in patients with adult onset Still’s disease. J Rheumatol. 2003;30(11):2422–7.

    CAS  PubMed  Google Scholar 

  60. Chen DY, Hsieh TY, Hsieh CW, Lin FJ, Lan JL. Increased apoptosis of peripheral blood lymphocytes and its association with interleukin-18 in patients with active untreated adult-onset Still’s disease. Arthritis Care Res. 2007;57:1530–8. https://doi.org/10.1002/art.23088.

    Article  Google Scholar 

  61. Kötter I, Wacker A, Koch S, et al. Anakinra in patients with treatment-resistant adult-onset Still’s disease: four case reports with serial cytokine measurements and a review of the literature. Semin Arthritis Rheum. 2007;37:189–97. https://doi.org/10.1016/j.semarthrit.2007.04.002.

    Article  CAS  PubMed  Google Scholar 

  62. Magadur-Joly G, Billaud E, Barrier JH, et al. Epidemiology of adult Still’s disease: estimate of the incidence by a retrospective study in West France. Ann Rheum Dis. 1995;54:587–90. https://doi.org/10.1136/ard.54.7.587.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  63. Larson EB. Adult Still’s disease - recognition of a clinical syndrome and recent experience. West J Med. 1985;142(5):665–71.

    CAS  PubMed  PubMed Central  Google Scholar 

  64. Crispín JC, Martínez-Baños D, Alcocer-Varela J. Adult-onset still disease as the cause of fever of unknown origin. Medicine (Baltimore). 2005;84(6):331–7. https://doi.org/10.1097/01.md.0000188009.47085.76.

    Article  Google Scholar 

  65. Takahashi A, Abe K, Yokokawa J, et al. Clinical features of liver dysfunction in collagen diseases. Hepatol Res. 2010;40:1092–7. https://doi.org/10.1111/j.1872-034X.2010.00707.x.

    Article  PubMed  Google Scholar 

  66. Kadavath S, Efthimiou P. Adult-onset Still’s disease-pathogenesis, clinical manifestations, and new treatment options. Ann Med. 2015;47(1):6–14. https://doi.org/10.3109/07853890.2014.971052.

    Article  CAS  PubMed  Google Scholar 

  67. Kurasawa M, Kotani K, Kurasawa G, Shida K, Yamada S, Tago T. Adult-onset Still’s disease in a patient over 80 years old successfully treated with low-dose methotrexate therapy. Age Ageing. 2007;36(1):104–6. https://doi.org/10.1093/ageing/afl128.

    Article  PubMed  Google Scholar 

  68. Stoica GS, Cohen RI, Rossoff LJ. Adult Still’s disease and respiratory failure in a 74 year old woman. Postgrad Med J. 2002;78(916):97–8. https://doi.org/10.1136/pmj.78.916.97.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  69. Tamura K, Kubota K, Kurabayashi H, Take H, Shirakura T. Elderly onset of adult Still’s disease: report of a case. Clin Rheumatol. 1994;13:117–8. https://doi.org/10.1007/BF02229879.

    Article  CAS  PubMed  Google Scholar 

  70. Wouters JMGW, Van Rijswijk MH, Van de Putte LBA. Adult onset Still’s disease in the elderly: a report of two cases. J Rheumatol. 1985;12(4):791–3.

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Rheumatology Unit, Autoimmune Disease Center, Sheba Medical Center, Ramat Gan, Israel

    Michal Brodavka & Merav Lidar

  2. Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel

    Merav Lidar

Authors
  1. Michal Brodavka
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Merav Lidar
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Merav Lidar .

Editor information

Editors and Affiliations

  1. Rheumatology Unit, Bnai Zion Medical Center, Haifa, Israel

    Gleb Slobodin

  2. Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Israel

    Yehuda Shoenfeld

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Brodavka, M., Lidar, M. (2020). Autoinflammatory Diseases in the Geriatric Population. In: Slobodin, G., Shoenfeld, Y. (eds) Rheumatic Disease in Geriatrics . Springer, Cham. https://doi.org/10.1007/978-3-030-44234-7_23

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-44234-7_23

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-44233-0

  • Online ISBN: 978-3-030-44234-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation