hereditary disease of unknown etiology.
prevalence of periodic disease
Periodic disease refers to a group of hereditary diseases of the autosomal-recessive type, the main manifestations of it are short-term, recurrent acute occurring attacks (attacks) with articular-abdominal symptoms, fever and / or painful symptoms withside of the chest, ending in complete recovery.
Periodic disease is most common in people of Caucasian nationalities and residents of the Mediterranean region (Armenians, Turks, Jews, Arabs, and so on), so it is also called the Armenian disease, Mediterranean fever, benign familial fever, etc.But often the disease occurs in other regions of the world, as is the family, hereditary.More common in muzhchi, and the first signs of it already appear in childhood or adolescence.
Features of periodic disease
- cause of the disease is an inborn error of metabolism predominantly serous tissues (they form the outer shell of the majority of human organs and blood vessels, as well as in
the synovial membrane of the joints), accompanied by an increase in vascular permeability, prone to swelling (exudation) and the development of connective tissue deposition in her abnormal metabolic products (amyloid).
- disease can longer period of time asymptomatic, not worsen.But under the influence of unfavorable external and internal factors (their nature is not yet fully studied) there is a benign inflammation of serous membranes with the release in this period various tissue enzymes, mediators, as well as the development of the cell, the body's immune responses.All this is accompanied by fever, severe pain, and later - and the pathological deposition of amyloid protein in organs and tissues (mainly in the kidneys).
- After the attacks subsided, which usually lasts a few days, the patient feels better and in the interictal period, he feels relatively well.Periods of remission (open spaces between exacerbations) are different in all patients and usually do not exceed 3-7 days.
- But with each attack increased amyloid deposition, leading to rising kidney impairment.Over time, 25-40% of patients develop chronic renal failure before fatal even at a young age (before 40 years).But now the treatment not only helps to alleviate the condition of patients, improve kachestvozhizni, but also to increase its duration.
main manifestations of periodic disease
- Typically, each period of exacerbation is accompanied by fever, but there is a special form of febrile disease, which flows by type of malaria - with periodic during the day the temperature rises to 40 degrees.
- In 85-95% of cases observed symptoms of peritonitis (inflammation of the peritoneum), due to which the patients are often hospitalized in the surgical department
- -abdominalnaya form of the disease in more than half the time (sometimes up to 80%) accompanied by a worsening of symptoms of arthritis - articulardisease.
- In 30-60% of patients with recurrent disease observed thoracic shape in the form of pleurisy, sudden attacks of pain in the chest when breathing, bronchitis, etc.
- Very often a combination of several forms of the disease.This may be a nodal failure, enlarged spleen, skin rash that looks like a face.Sometimes it can develop aseptic meningitis.