pediatrics
Familial Mediterranean Fever
Symptoms and Signs of Familial Mediterranean Fever
Onset of FMF is usually between the ages of 5 and 15 years but may be much later or earlier, even during infancy. Attacks have no regular pattern of recurrence. They usually last 12 to 72 hours but may last longer. Frequency ranges from 2 attacks/week to 1 attack/year (most commonly, once every 2 to 6 weeks). Physical and emotional stressors (eg, physical trauma, infection, menstruation) may trigger attacks ( Symptoms and signs references ). Severity and frequency tend to decrease during pregnancy and in patients with C, usually accompanied by Peritonitis , is the major manifestation. Abdominal pain (usually starting in one quadrant and spreading to the whole abdomen) occurs in about 95% of patients and can vary in severity with each attack. Decreased bowel sounds, distention, guarding, and rebound tenderness are likely to occur at the peak of an attack and cannot be differentiated from a perforated viscus by physical examination. Consequently, some patients undergo urgent laparotomy before the correct diagnosis is made. With pleural involvement, dyspnea due to pleuritic pain may occur. Other manifestations of FMF include arthritis (in 25%), usually involving the knee, ankle, and hip; an Erysipelas -like rash of the lower leg; and scrotal swelling and pain caused by inflammation of the tunica vaginalis of the testis. Despite the severity of symptoms during acute attacks, most patients recover swiftly and remain free of illness until their next attack. CAPS = cryopyrin-associated periodic syndromes; FMF = familial Mediterranean fever; NOMID = neonatal-onset multisystem inflammatory disease; PFAPA = periodic fevers with aphthous stomatitis, pharyngitis, and adenitis; TRAPS = tumor necrosis factor receptor–associated periodic syndrome. Adapted fromSag E, Bilginer Y, Ozen S: Autoinflammatory diseases with periodic fevers.Curr Rheumatol Rep19(7):41, 2017. doi: 10.1007/s11926-017-0670-8 The most significant long-term complication of FMF is Chronic renal failure caused by deposition of amyloid protein in the kidneys Amyloid may also be deposited in the gastrointestinal tract, liver, spleen, heart, testes, and thyroid. FMF may cause infertility or spontaneous abortion in up to one third of untreated women because peritoneal pelvic adhesions form, interfering with conception. FMF increases the risk of other inflammatory disorders, such as Ankylosing Spondylitis , 1.Yenokyan G, Armenian HK: Triggers for attacks in familial Mediterranean fever: application of the case-crossover design.Am J Epidemiol175(10):1054-1061, 2012. doi: 10.1093/aje/kwr460 2.Ben-Chetrit E, Yazici H: Familial Mediterranean fever: different faces around the world.Clin Exp Rheumatol37 Suppl 121(6):18-22, 2019. PMID: 31694745 3.Balcı-Peynircioğlu B, Kaya-Akça Ü, Arıcı ZS, et al: Comorbidities in familial Mediterranean fever: Analysis of 2000 genetically confirmed patients.Rheumatology (Oxford)59(6):1372–1380, 2020. doi: 10.1093/rheumatology/kez410 