Pediatric Allergic Subseptic Syndrome
Introduction
Introduction to children with allergic subseptic syndrome Allergic subseptic syndrome, ie allergic subsepticemia, is also known as Wissler-Fanconi syndrome, Wissler syndrome, subacute allergic fever, and the like. It is a rare infection-allergy syndrome characterized by long-term intermittent persistent fever, rash, joint pain, leukocytosis, and negative blood culture. Many people consider that the clinical manifestations, laboratory tests, and disease progression of the intrinsic are similar to those of juvenile rheumatoid arthritis, and are therefore considered to be a clinical type or a clinical stage of systemic rheumatoid disease and not a unique disease. It is also considered to be an allergic disease between rheumatic fever and rheumatoid disease and is associated with autoimmunity. basic knowledge The proportion of illness: 0.005% Susceptible people: children Mode of infection: non-infectious Complications: myocarditis, atrioventricular block, iridocyclitis
Cause
Pediatric allergic subseptic syndrome etiology
(1) Causes of the disease
The cause is not clear. Wissler believes that the bacterial infection and allergic reaction are comprehensive. Fanconi has taken the specimen from the patient's infected alveolar and cultured the streptococcus and recovered it after the injection of the self-bacterial vaccine. Therefore, it is believed that the bacteria have a certain disease principle. Significance, as for the source of infection, in addition to streptococcal and staphylococcal infections, others speculate that it is associated with infections such as mycoplasma and viruses (rubella or lentivirus).
(two) pathogenesis
Many people are sure that the pathogenesis of this disease has an immune mechanism involved, the evidence is as follows:
1. Lymphocytes and plasma cells infiltrated in the synovial membrane with follicle formation.
2. Immunofluorescence electron microscopy of synovial cells, see IgG, IgM, rheumatoid factor and complement.
3. The presence of soluble antigen-antibody complex in synovial fluid is accompanied by a decrease in complement. The increase of leukocytes in synovial fluid without the presence of bacteria also suggests the presence of an immune response.
4. There is a decrease in serum rheumatoid factor with complement.
5. Metabolic abnormalities The concentration of sugar in joint fluid decreased, serum arginine, tyrosine, histidine and glutamine decreased; tryptophan metabolite excretion increased, etc. Pearson believed that antigen-antibody complex was phagocytized by leukocytes, white blood cells Dissolve, release hydrolase and cause local and systemic manifestations. Others such as history of trauma before onset, positive family history (ie, genetic relationship) are still unknown, and others believe that the disease and adult rheumatoid arthritis are both The same, but different, suggesting that both have the same cause, only the human body's response is different.
Prevention
Pediatric allergic sepsis syndrome prevention
The cause of the disease has not yet been clarified. At present, there is no systematic prevention method. The prevention measures for allergic diseases should be referred to, and the prevention and treatment of various infectious diseases should be emphasized.
Complication
Pediatric allergic sepsis syndrome complications Complications Myocarditis atrioventricular block iridocyclitis
Other clinical manifestations such as systolic murmur sometimes occur in the heart, electrocardiogram can be changed by myocarditis, myocardial damage, ventricular hypertrophy and atrioventricular block, occasionally pericarditis and pleurisy, brain symptoms can be seen in some cases, and EEG abnormalities In addition, a small number of patients can be seen with iridocyclitis, subcutaneous nodules, and renal amyloidosis.
Symptom
Symptoms of allergic sub-sepsis syndrome in children Common symptoms Repeated hyperthermia lymphadenopathy, relaxation, heat, intermittent heat, chills, heat, joint pain, hepatosplenomegaly, joint swelling, abdominal pain
Fever
Repeated high fever is a prominent complaint, fever is more rapid, often preceded by joint symptoms ranging from several weeks to several years, the heat type is mostly intermittent heat, but also can be relaxation heat, heat retention, recurrence heat, cycle heat, double peak heat, etc. A variety of thermal types, of which the most characteristic of continuous intermittent heat, the same day temperature fluctuations can be greater than 2 ° C, or even 3 ~ 4 ° C, high fever can have chills, sick children with fever when the mind is clear, the general situation is still good, hot When you retreat, you will be treated with other people. If you have other diseases, such as respiratory infections, chickenpox or surgery, you may often cause re-heating.
2. Rash
About 20% to 70% of patients have rashes, most of which occur in the first few months of arthritis, several years or with arthritis, and a few occur in arthritis 1 week to 9 years, the rash is scattered in the punctate rash. It is not painful or itchy, generally not more than 5mm, even can be fused into a sheet, the edge is irregular, the larger rash is pale in the center, the rash can be multi-shaped erythema, scarlet fever or measles-like, and the shape changes, often one Over-the-sex, the short-term disappears in a few hours, occasionally for one or several days, the rash often appears with fever, recurring, when the fever and other inflammatory manifestations are relieved, the rash disappears, and when the disease recurs, the rash often It was an early appearance. Grislain reported a boy who had 36 episodes in 3 years and 2 months after the first onset, of which 34 had irregular high fever and 31 had rash.
Anti-allergic drugs, hormones and other rashes are not effective. After injecting hyaluronidase 500U/0.5ml into the rash, the rash can be small, indicating that the rash can be inhibited by hyaluronidase, suggesting that there is a substance similar to hyaluronic acid or Chondroitin sulfate is present.
3. Joint symptoms
20% of patients can have no signs of arthritis at the beginning of the disease. Joint pain can be roughly divided into 4 cases: 1 acute extensive pain; 2 pain and tenderness during exercise; 3 no conscious pain but tenderness; 4 joint swelling may be no or With tenderness, joint pain is more fixed, occasionally wandering, the earliest and most susceptible to knee joints, wrists, elbows, ankles, hips and other joints are often involved, older children can sometimes involve the cervical and ankle joints, with Age can affect small joints. Joint symptoms are generally consistent with high fever episodes, and heat-removal arthritis is also relieved. There are also multiple cycles of episodes and remissions. One patient has 30 episodes in 12 years, and a few patients have fever. Although controlled, the joint disease activity continued, and then converted to chronic polyarthritis.
4. Lymph node enlargement
At the beginning of the disease, about 60% of patients have swollen lymph nodes on the neck, underarms, groin and trochlear, no pain, clear edges, sometimes misdiagnosed as lymphoma or hematological malignant lesions, and if the mesenteric lymph nodes can cause abdominal pain Often accompanied by significant systemic symptoms such as fever, hepatosplenomegaly and arthritis, lymph nodes can disappear after systemic symptoms are relieved, and lymphadenopathy in individual patients can continue until other systemic symptoms disappear.
Examine
Examination of children with allergic subseptic syndrome
There is currently no specific diagnostic method for the intrinsic, and the following experimental tests are available:
Blood routine examination
It can be seen that eosinophils increase, hemoglobin volume decreases, and low hemoglobinemia can be seen. The total number of white blood cells increases with the left shift of the nucleus. The white blood cells are generally 10,000 to 20,000, most of which are 50,000, and some >50,000, with up to 100,000.
2. Blood test
(1) Protein electrophoresis: albumin is mostly reduced, and 2 and gamma globulin are significantly higher.
(2) Determination of rheumatoid factor: mostly negative, a few positive, children with late onset or persistent disease, severe arthritis are more positive, and more with subcutaneous nodules, a small number of patients initially negative, then positive, negative The prognosis is poor.
(3) Lupus cells: rarely positive, and short-lived.
(4) Determination of antinuclear antibodies: acute onset, systemic symptoms were negative, only a few patients were positive and low titers, positive patients were often associated with rheumatoid factor positive or gamma globulin increased, female patients, polyarthritis patients and Young children are more likely to be positive.
(5) immunoglobulin determination: serum IgG, IgA and IgM can be increased, especially in the active period, Bianco said, such as patients with increased IgG and IgM is not high, with serum complement increased, the course of disease is mostly benign and self-limiting, good prognosis.
(6) Serum complement determination: normal or increased, especially C3, C4, C3a, while C1q is unchanged, the cause of increased complement is excessive consumption and increased synthesis and overcompensation, (7) ESR: mostly increased However, it has nothing to do with the level of fever.
(8) Anti-streptolysin "O": 1/5 ~ 1/2 people have increased anti-"O", most of the previous infection history, the intrinsic anti-"O" measurement results are not higher than the control group, so this One trial lacked specificity.
(9) Others: shortened red blood cell life, poor iron treatment, mucin can be increased, C-reactive protein is often positive during active period, and some people think that plasma mucin tyrosine can be used as a reference indicator of inflammatory activity.
3. Bone marrow examination showed a hyperplastic change in the bone marrow.
4. Blood culture examinations all received negative results.
5. Urine examination showed thermal proteinuria or persistent egg hematuria. In patients with amyloidosis, proteinuria was longer and more severe.
6. Joint biopsy showed synovial cell hyperplasia, synovial hypertrophy and edema, small blood vessels increased with vascular endothelial swelling, mainly infiltration of plasma cells and lymphocytes, but also neutrophils, often accompanied by cellulose deposition.
7. Skin rash biopsy showed significant edema of collagen fibers with perivascular neutrophil infiltration, as well as lymphocytes and plasma cells.
8. Do X-ray chest X-ray, ECG, B-ultrasound, etc.
1. X-ray examination of bone fragments showed soft tissue swelling, osteoporosis, osteophyte growth, bone erosion and joint stiffness, chest radiographs can be found in pleural inflammatory changes.
2. ECG examination may show changes in myocarditis, myocardial damage, ventricular hypertrophy and atrioventricular block, and some may find pericarditis.
Diagnosis
Diagnosis and diagnosis of allergic subseptic syndrome in children
diagnosis
It is generally considered that the following conditions can be diagnosed:
1. Long-term high heat and long-term intermittent continuous high fever, which may be relaxation heat or heat retention.
2. The rash and joint symptoms recurred with transient pleomorphic rash and joint symptoms, accompanied by lymph nodes and hepatosplenomegaly.
3. Laboratory examination of peripheral blood shows increased white blood cells and neutrophils, rapid blood sedimentation, negative blood culture.
4. Therapeutic response Antibiotic treatment is ineffective, but corticosteroids and other immunosuppressive treatments can alleviate the condition.
Differential diagnosis
This disease is easily misdiagnosed as other diseases. Of the 229 cases of Grossma, 109 cases have been misdiagnosed as rheumatic fever, sepsis and lymphoma. In the differential diagnosis, sepsis, liver abscess, miliary tuberculosis, malaria, typhoid, Brucella should be considered. Disease, drug fever, lupus erythematosus, lymphoma, nausea and malignant granuloma.
