Allergic subsepsis is a symptom complex of infectious-allergic genesis with unclear etiology, mainly affecting children and similar in its characteristics to autoimmune collagenoses. Symptoms are a sudden developing severe fever lasting from 2-3 days to several months, erythematous or coroid skin rashes, pain and swelling of the joints (polyarthritis). Diagnosis is made on the basis of the clinical picture of the disease and the results of laboratory tests (blood test, biochemical blood test). The treatment is complex, includes the use of antibacterial agents, nonsteroidal anti-inflammatory drugs, in complex cases, glucocorticoids and cytostatics can be used.
ICD 10
T78.9 Adverse reaction, unspecified
General information
Allergic subsepsis, or Wissler-Fanconi disease, was isolated into a separate nosological unit in the 40s of the XX century (in 1943 by G. Wissler and, independently of him, in 1946 by G. Fanconi). The etiology and many stages of the pathogenesis of the syndrome are currently insufficiently studied. Usually the disease affects children aged 3-8 years, but cases of pathology in infants in the first weeks of life have been described. There are no exact data on the prevalence of subsepsis, mainly due to the complexity of its differential diagnosis with some forms of reactive arthritis, Still’s syndrome and true sepsis. Some authors believe that the Wissler-Fanconi syndrome is one of the stages of the development of true collagenosis.
Causes
The etiology of the disease is currently not precisely established, specialists in the field of clinical allergology, immunology, infectology and a number of other medical areas are studying it. Despite the clinical picture characteristic of sepsis, the blood cultures of patients almost always turn out to be sterile, which indicates that the infection is only a trigger factor for an immunological reaction. A number of patients with allergic subsepsis have a history of frequent episodes of intolerance to pollen, household dust, certain foods and medicines. It is not known whether an allergic reaction is a link in the pathogenesis of the disease or simply indicates an altered reactivity of the patient’s body and a disturbed state of his immune system.
In the blood of some individuals with Wissler-Fanconi disease, antinuclear antibodies and other indicators of autoimmune pathological processes are determined. This combines the disease with a number of autoimmune pathologies, such as Still’s syndrome, rheumatism, reactive polyarthritis. The fact that subsepsis is prone to spontaneous disappearance with the recovery of the patient contradicts its consideration as the initial stage of collagenoses. There is a hypothesis that this symptom complex may manifest several diseases of different genesis. Ambiguity in the development of pathology complicates the development of effective etiotropic therapy, therefore, treatment is carried out symptomatically, through the complex use of drugs from different groups.
Pathogenesis
Due to the poor knowledge of the etiology of allergic subsepsis, there are many gaps in understanding the pathogenesis of this condition. According to the most common theory of the development of Wissler-Fanconi syndrome, a systemic perverted immunological reaction occurs under the influence of a focus of bacterial infection of an acute or chronic nature. It is assumed that the development of the reaction is provoked by septicemia, in which antigens of the pathogen enter the bloodstream en masse. Due to the peculiarities of reactivity, the complement system is activated (mainly in the tissues of the skin and articular membranes). This leads to the appearance of a characteristic rash and arthralgia. In the cells of the epidermis, an increased number of complement components are detected.
The pathogenesis of fever and hyperthermia in subsepsis is presumably explained by the activation of macrophages and their release of many pyrogens. Aspects of the appearance of antinuclear antibodies, neutrophilia and pronounced leukocytosis characteristic of true sepsis remain unclear. It is believed that the appearance of antibodies to the cell nucleus indicates the transformation of allergic subsepsis into true collagenosis, which worsens the prognosis of the disease. The pathogenesis of the development of heart lesions (myocarditis and pericarditis) in this condition is unknown. They are noted in a small percentage of patients, extremely rarely lead to complications, and are prone to spontaneous healing.
Symptoms of allergic subsepsis
In most cases, the disease begins acutely, against the background of complete health, without any prerequisites or prodromal phenomena. In rare cases, allergic subsepsis occurs shortly after a sore throat or respiratory tract disease. The first manifestation of pathology is a high temperature (up to 38-39 ° C), fever has a continuous or intermittent character with periodic sharp rises and falls in temperature with a frequency of 2-3 days. The decrease in temperature after the peak lasts 10-18 hours and is accompanied by profuse sweating. The total duration of the hyperthermia period can be up to several weeks or months.
In the first hours after an increase in temperature, erythematous or urticarian rashes appear on the skin. Frequent localization of the rash is the skin of the legs and arms, less often they are registered on the skin of the chest, face, abdomen. Skin elements may become less pronounced and reappear, the frequency of rashes often coincides with periods of rising and falling temperature and feverish manifestations. Patients complain of moderate skin itching in the area of rashes. In some patients, subcutaneous seals in the form of nodules are found, similar to similar manifestations in rheumatism. At the height of the attack, there is an increase in various groups of lymph nodes – mainly inguinal, axillary, cervical.
Allergic subsepsis is characterized by various articular manifestations in severity – from non-intense pain to significant swelling and redness. The joints of the hands (interphalangeal, wrist, elbow) are more often affected, in some cases the joints of the spine and lower extremities may become inflamed. The localization of joint lesions may change with each attack of pathology. Against the background of high temperature, abdominal pain and muscle pain sometimes occur, with the development of myocarditis, pain in the heart area appears. With fever, disorders of the central nervous system are possible: sleep disorders, anxiety, confusion.
Complications
Complications of allergic subsepsis are recorded quite rarely. The most serious consequence of the disease is the development of true collagenosis – juvenile rheumatoid arthritis or rheumatism. Among other complications, heart failure due to the development of myocarditis or pericarditis, liver damage is extremely rare. Fever can provoke an exacerbation of the patient’s chronic diseases of various organs. Younger patients (3-4 years old) have a high risk of developing repeated episodes after a period of remission. High fever sometimes causes febrile seizures.
Diagnostics
When diagnosing allergic subsepsis, an immunologist or pediatrician pays attention to the clinical picture of the pathology, the results of a laboratory blood test. Radiography and electrocardiography are prescribed as additional studies. It is important to eliminate true sepsis as soon as possible, which requires urgent antibiotic therapy and other emergency measures. Also, as part of the diagnosis of Wissler-Fanconi disease, methods are used to differentiate this condition with typical collagenoses – for example, juvenile rheumatoid arthritis. The following diagnostic measures are carried out:
- Examination and anamnesis collection. Hyperthermia is detected, rash on the hands and feet of various nature, it is possible to identify seals in the skin similar to rheumatic nodules. Palpation of the lymph nodes determines their enlargement and soreness, some joints are painful when pressed, the skin in their projection is hyperemic. Anamnesis often indicates the presence of allergic diseases.
- Laboratory examination of blood. In the blood test, a sharp leukocytosis is detected, a slight neutrophilia with a weak neutrophil shift to the left and an increase in the number of eosinophils is possible. At fever altitude, ESR increases significantly. Blood biochemistry confirms hyperglobulinemia (due to an increase in the concentration of IgG and IgM), a decrease in the level of albumins and complement components, sometimes antinuclear antibodies are registered.
- Instrumental research. When heart pathology is attached, signs of myocarditis are recorded on the ECG – irregular rhythm, ST segment changes, various types of blockages of the conduction system. During the chest X-ray, an increase in the pulmonary pattern, an overlay on the pleura and signs of pleurisy are determined. Abdominal ultrasound indicates an increase in the size of the liver (at the height of fever) and, less often, the spleen.
Differential diagnosis of allergic subsepsis is carried out with true sepsis, juvenile rheumatoid arthritis and other types of collagenosis – for this purpose, patients are prescribed a number of additional studies. Sowing blood on nutrient media shows its sterility, which excludes bacteremia and systemic infectious damage. To exclude rheumatoid arthritis, a biopsy of the synovial membrane of the affected joints is performed with further histochemical examination.
Treatment of allergic subsepsis
There is no single generally accepted treatment regimen for pathology due to insufficiently studied etiology and pathogenesis. Usually, complex therapy is used, aimed at combating the infectious factor, reducing the activity of inflammatory processes. In severe cases, drugs are prescribed to reduce the activity of the immune system. Traditional antipyretics (paracetamol, acetylsalicylic acid) do not help much in eliminating fever with allergic subsepsis, but as part of a complex treatment they can have a beneficial effect. The main components of therapy are:
- Antibacterial therapy. They use drugs of a wide spectrum of action, mainly of the penicillin series. The effectiveness and validity of the use of antibiotics in this allergic pathology is disputed by some experts, but statistically proven better results of treatment with their appointment than without it.
- Anti-inflammatory therapy. Various drugs from the group of nonsteroidal anti-inflammatory drugs are used – acetylsalicylic acid, diclofenac sodium, indomethacin, ibuprofen. In complex severe cases and with severe hyperthermia, glucocorticoids can be prescribed – only they are able to reduce body temperature for a short time with allergic subsepsis.
- Immunosuppressive therapy. In particularly severe cases, short-term use of cytostatics is possible to reduce the severity of immune reactions. They are especially often used for heart lesions and severe inflammation of the joints.
Some doctors consider the appointment of salicylates in this pathology ineffective and prefer a combination of antibiotics with glucocorticoids. Individual cases of effective use of antihistamines have been noted. Patients are shown bed rest, during periods of lowering body temperature – walking in the fresh air. Careful monitoring of laboratory blood parameters is necessary for early detection of signs of rheumatoid arthritis and other collagenoses. Auxiliary treatment is aimed at reducing the side effects of prolonged use of antibiotics and NSAIDs (protection of the gastric mucosa, restoration of intestinal microflora), restoration of water-salt metabolism, impaired as a result of increased sweating.
Prognosis and prevention
The prognosis of allergic subsepsis is usually favorable, but careful monitoring of the condition of patients is required to avoid the development of complications. The defeat and inflammation of the joints in the absence of rheumatoid arthritis does not lead to a restriction of their mobility after arresting the attack. Myocarditis and inflammation of the serous membranes, as a rule, are also cured without consequences. Fever attacks can last up to 2-4 months, after which they can spontaneously disappear. In the future, new episodes of subsepsis may occur, but as the child grows, they become more fleeting and flow easier. In the absence of complications, there are no consequences in adults. Due to poorly understood causes and pathogenesis of the disease, preventive measures have not been developed.
