Episodic Fever and Lymphadenopathy

  • Romina Dieli-Crimi
  • Theresa Español


Majority of patients with familial Mediterranean fever (FMF) become symptomatic before 10 years of age and up to 90% experience attacks before the age of 20

Incomplete FMF attacks are common, when recurrent painful attacks are associated with fever below 38 °C, resolution of attacks in less than 12 hours or more than 4 days or when fewer than 3 episodes have occurred.

Hyper-IgD syndrome (HIDS) can also closely mimic symptoms of familial relapsing fevers, with febrile episodes, abdominal pain and mucocutaneous manifestations in form of papules, urticaria, erythema, and petechiae

Recurrent episodes of systemic inflammation in FMF are associated with increased SAA, which is also elevated during symptom-free episodes.

The most serious complication of FMF is renal amyloidosis however, which gives rise to nephrotic syndrome


Hyper-IgD syndrome Familial Mediterranean fever Serum amyloid A 


  1. 1.
    Livneh A, Langevitz P, Zemer D, Zaks N, Kees S, Lidar T, Migdal A, Padeh S, Pras M. Criteria for the diagnosis of familial Mediterranean fever. Arthritis Rheum. 1997;40(10):1879–85.CrossRefGoogle Scholar
  2. 2.
    Zadeh N, Getzug T, Grody WW. Diagnosis and management of familial Mediterranean fever: integrating medical genetics in a dedicated interdisciplinary clinic. Genet Med. 2011;13(3):263–9.CrossRefGoogle Scholar
  3. 3.
    Medlej-Hashim M, Petit I, Adib S, Chouery E, Salem N, Delague V, Rawashdeh M, Mansour I, Lefranc G, Naman R, Loiselet J, Lecron JC, Serre JL, Megarbane A. Familial Mediterranean fever: association of elevated IgD plasma levels with specific MEFV mutations. Eur J Hum Genet. 2001;9(11):849–54.CrossRefGoogle Scholar
  4. 4.
    Su F, Bai F, Zhang Z. Inflammatory cytokines and Alzheimer’s disease: a review from the perspective of genetic polymorphisms. Neurosci Bull. 2016;32(5):469–80.CrossRefGoogle Scholar
  5. 5.
    Aksu K, Keser G. Coexistence of vasculitides with familial Mediterranean fever. Rheumatol Int. 2011;31(10):1263–74.CrossRefGoogle Scholar

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Romina Dieli-Crimi
    • 1
  • Theresa Español
    • 1
  1. 1.Inmunóloga clínica, Facultativo Especialista Inmunología Servicio de Inmunología Hospital Universitario Vall d’Hebron, Edificio laboratorios clínicosBarcelonaSpain

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