21/07/2024
Daily Syndrome : Periodic Fever Syndrome: The term “periodic fever syndrome” refers to several different autoinflammatory diseases that have similar symptoms–the primary symptom being a recurrent fever for which no infectious cause can be found. The patient just keeps getting fevers, often accompanied by a range of other symptoms. These are primarily genetic conditions and are not contagious. Autoinflammatory diseases are caused by a malfunction in the innate immune system.
The periodic fever syndromes include, but are not limited to: The Cryopryin-Associated Periodic syndromes (CAPS): Familial Cold Autoinflammatory Syndrome (FCAS), Muckle-Wells Syndrome (MWS), and Neonatal-Onset Multisystem Inflammatory Disease (NOMID or CINCA) Familial Mediterranean Fever (FMF) Tumour Necrosis Factor (TNF)-Associated Periodic Syndrome (TRAPS) Mevalonate Kinase Deficiencies: Hyper-IgD with Periodic Fever Syndrome (HIDS), and Mevoalonate Aciduria (MA) Periodic Fever, Aphthous Stomatitis, Pharyngitis, & Cervical Adenitis (PFAPA syndrome)
Many autoinflammatory diseases tend to have frequent recurring fevers as the most obvious symptom. The fevers are often cyclic in nature, coming and going in some cases erratically and in some cases more predictably.These are some of the most common symptoms that may be noted during disease flares in various periodic fever syndromes: Fever (recurrent, and accompanied by some of the following symptoms) Rash Headache Vomiting Diarrhea Constipation Conjunctivitis Mouth ulcers Swollen lymph nodes Abdominal pain Joint pains, or arthritis Fatigue Abnormal blood test results during flares of symptoms, including: high white blood count (WBC) and high inflammatory markers CRP and ESR Serositis (pericarditis, pleurisy)
Age of Onset Most patients start presenting with symptoms in childhood, either as an infant, toddler, or by the age of 5. However, some patients may not develop their first symptoms until they are older (school age or adolescence) or even as adults. In some cases, the disease may go into remission for months to years at a time.Once a patient is suspected of having a periodic fever syndrome, it is necessary to do a clinical evaluation to try to determine which syndrome(s) could be a possibility. This includes an evaluation of the patient’s most common symptoms, blood tests (during flares of symptoms, and during times with no obvious symptoms), and genetic testing for these diseases. If the patient has a rash, a skin biopsy by a dermatologist should be done as well.
