Allergic subsepsis Wissler-Franconi: etiology, pathogenesis and treatment
Subsepsis allergic Wissler-Franconi (SAVF) is a rare disease that belongs to a special form of rheumatoid arthritis. This pathology is characterized by high, sometimes hectic temperature, polymorphic skin rash, arthralgia or other joint lesions, as well as neurovegetative disorders. Although SAVF can occur at any age, it most often occurs in children between 4 and 12 years of age.
The etiology and pathogenesis of SAVF still remain unclear. However, the provoking factor is an infection, most often a sluggish focal one. Perhaps the pathogenesis is based on an autoimmune process, which leads to damage to various organs and systems.
The clinical picture of SAVF begins acutely with high fever that can last many weeks and months. Characterized by a polymorphic rash on the skin, which is localized on the extremities, less often on the face, chest and abdomen. Arthralgia, paroxysmal abdominal pain, enlarged lymph nodes, spleen and liver are often observed. Neurovegetative disorders may also appear: chills, tachycardia, arrhythmia, sweating and mental disorders.
In the initial period of the disease, hematological changes are characteristic: leukocytosis, with a shift of the formula to the left, and a persistent increase in ESR for several months. Subsequently, there is a tendency towards leukopenia, and the ESR decreases. Characterized by hyperproteinemia, hypoalbuminemia, an increase in gamma globulins, an increase in the level of immunoglobulins M and G, as well as a decrease in serum complement.
The diagnosis of SAVF is based on the history and clinical course. Differential diagnosis includes rheumatism, systemic lupus erythematosus, systemic scleroderma, osteomyelitis, sepsis and typhoid fever.
Treatment of SAVF consists of general measures such as hospitalization and bed rest, as well as antibiotic therapy as indicated. The complex of therapy includes acetylsalicylic acid, which is prescribed 3-4 times a day after meals at a dose of 0.06-0.1 g/kg body weight per day (but not more than 3 grams per day), as well as glucocorticosteroids at a dose of 1-2 mg/kg body weight per day for 3-4 weeks. In the presence of articular manifestations, non-steroidal anti-inflammatory drugs (NSAIDs) are prescribed. In case of development of complications, such as vasoconstrictor collapse, angioedema and others, intensive therapy and symptomatic treatment are carried out.
The prognosis of SAVF depends on the severity of the disease and the timeliness of treatment. In most cases, the prognosis is favorable, but complications are possible, such as arterial hypertension, damage to the cardiovascular and nervous systems, as well as the development of chronic pathology. Therefore, it is important to promptly consult a doctor if you suspect SAVF and begin treatment.