Still's disease

Introduction to Still's disease Still's disease is a seronegative polyarthritis that is a type of juvenile rheumatoid arthritis. Still disease (AOSD) is a relatively rare inflammatory disease with a clinical and laboratory profile similar to systemic youth arthritis (Still disease). It is characterized by a daily peak fever, arthritis and a typical rash that occurs throughout the fever period. basic knowledge The proportion of illness: 0.002% Susceptible people: no specific population Mode of infection: non-infectious Complications: atelectasis pneumonia, adult respiratory distress syndrome, peptic ulcer

Cause of Still's disease

(1) Causes of the disease

The cause of the disease is still unclear and is generally thought to be related to infection, genetic and immune abnormalities.

Infection (35%)

Most patients have a history of upper respiratory tract infection before onset. There are pharyngitis and gingivitis on the onset of the disease. The serum anti-O is elevated in the laboratory test. Some patients have staphylococcal growth in the pharyngeal test, and the disease is relieved after the preparation of the self-vaccination. Still's disease is associated with streptococcal infection. In addition, antibodies against Yersinia, anti-Rubella virus antibodies and anti-mumps virus antibodies are found in the serum of some patients, and staphylococcal A immune complexes are present in some patients. Therefore, some people think that the incidence of adult Steel's disease has a certain relationship with infection, but in addition to the culture of the pharyngeal test, bacteria and viruses have never been isolated in other diseased tissues, so the role of infection in the pathogenesis cannot be determined.

Genetics (25%)

It has been reported that adult Still's disease is associated with class I antigens and class II antigens in human leukocyte antigens, including HLAB8, Bw35, B44, DR4, DR5 and DR7, suggesting that the disease is genetically related, but the above-mentioned HLA-positive sites and clinical No significant correlation was found between the performance, diagnosis and treatment of the drug, and it was of no special significance in supporting clinical diagnosis.

Immune abnormality (15%)

Some studies have suggested that immune abnormalities are associated with this disease, and patients with adult Still's disease have abnormal cellular and humoral immunity.

1 The blood levels of tumor necrosis factor, interleukin-1, interleukin-2, interleukin-2 receptor and interleukin-6 are elevated in the patient's blood.

2T helper cells decrease, T inhibits cell growth and total T lymphocyte count decreases, T cell receptor- phenotype-positive T lymphocytes (TCR-gamma delta T cells) increase, and serum ferritin and C-reactive protein is closely related, T cell receptor- phenotype-positive T lymphocytes are a newly discovered T cell subset, which has the function of secreting various cytokines and cytotoxic activity.

3 Some patients have some autoantibodies during disease activities, such as anti-histone antibodies and anti-cardiolipin antibodies, and some patients have anti-erythrocyte antibodies and anti-platelet antibodies.

4 serum total complement, C3 and C4 can be reduced,

5 circulating immune complexes are elevated, during the disease activity, serum immunoglobulin is elevated, and hyperglobulinemia occurs. Pregnancy and the use of estrogen may have the effect of inducing the disease.

The above studies suggest that adult Still's disease may be due to the excessive immune response of susceptible individuals to certain foreign antigens such as viral or bacterial infections, resulting in abnormal cellular and humoral immune regulation, resulting in fever rash, joint pain and elevated peripheral blood cells. A series of inflammatory clinical manifestations.

(two) pathogenesis

The pathogenesis of this disease is still unclear. There is no specific pathological change in this disease. The synovial membrane is characterized by non-specific synovitis, mild to moderate hyperplasia of synovial cells, vascular congestion, lymphocyte and plasma cell infiltration with follicular formation. The synovial lining cells were positive for IgG, IgM and rheumatoid factor, and the lymph nodes were non-specific inflammation. Some lymph nodes showed T-cell tumor-like immunogenic hyperplasia, sometimes with lymph node necrosis, and the skin showed skin collagen fiber edema around the blood vessels. Inflammatory cells infiltrate, muscles show muscle edema and non-specific inflammation, liver shows hepatocyte degeneration, necrosis, inflammatory cell infiltration in portal and portal areas, cardiac manifestations of interstitial myocarditis, cellulose exudative pericarditis and heart valves Inflammatory lesions, renal biopsy showed thickening of the glomerular basement membrane, tubular atrophy and interstitial cell infiltration.

Still's disease prevention

1. Eliminate and reduce or avoid the disease factors, improve the living environment, improve the development of good habits, prevent infection, pay attention to food hygiene, and rational diet.

2. Pay attention to exercise, increase the body's ability to resist disease, do not fatigue, excessive consumption, quit smoking and alcohol.

3. Early detection and early diagnosis and early treatment, establish confidence in the fight against disease, adhere to treatment.

Currently there are no related content description. Also pay attention to a reasonable diet.

Stil's disease complications Complications, atelectasis, pneumonia, adult respiratory distress syndrome, peptic ulcer

Atelectasis, pulmonary hemorrhage, interstitial pneumonia and amyloidosis, or adult respiratory distress syndrome, as well as individual patients with peptic ulcer, appendicitis or pancreatitis.

Stil's disease symptoms Common symptoms Lymph node enlargement Hepatosplenomegaly Atmospheric blood loss and papular rash Joint pain Relaxation Heat heat retention Yellow jaundice fever

Both men and women can be ill, more common in children 2 to 10 years old.

1. Fever is a relaxation type, generally above 39 ~ 40 ° C, body temperature fluctuations can reach 2 ~ 3 ° C per day, the patient's body temperature may be high, but the symptoms of poisoning are slightly light, generally good, after 1 to 2 weeks Repeated fever, sometimes with heat, can last for months to years.

2. The rash fever and rash often exist at the same time. The rash has the characteristics of polymorphism and change, and can be characterized by rash, papules, urticaria, scarlet fever-like erythema, measles-like erythema, erythema multiforme and nodular erythema. The morphology, the size varies, the distribution is uncertain, and there may be pigmentation after regression, often with repeated rashes.

3. Joint symptoms can affect the joints of the limbs and the joints, showing pain, swelling is mild, can be migratory pain, the symptoms are aggravated during fever, and the heat can be relieved by itself.

4. Others Pericarditis, myocarditis, pleurisy, hepatosplenomegaly and jaundice, superficial lymph nodes, more common in the neck, underarms and groin, tenderness is not significant.

Stil's disease check

White blood cell count was significantly increased (10 ~ 45) × l09 / L, classification of neutrophils increased, nuclear left shift; ESR increased, blood culture was negative.

X-ray findings: X-ray findings of the disease are non-specific, early soft tissue swelling and osteoporosis near the joints, repeated or persistent arthritis can be seen in articular cartilage destruction and bone erosion, common under the periosteum near the affected joint Linear new bone, joint space stenosis, joint stiffness and joint subluxation in the late stage, often involving the wrist, knee and ankle joints, a small number of patients with cervical involvement report, the characteristic radiological changes are the wrist and Non-erosive stenosis of the intercarpal joint can lead to bony rigidity. Compared with rheumatoid arthritis, the incidence is 6 times and 11 times higher. No infection or neoplastic lesions were found in various laboratory and imaging examinations.

Diagnosis and identification of Still's disease

Diagnostic criteria

Diagnosis can generally be made based on typical clinical performance and laboratory tests.

TCM pathogenesis and syndrome type: This disease is congenital endowment insufficiency or vain internal injury, qi and blood weakness, resulting in yin and yang qi and blood disorders, the evil gas multiplied by this disease.

Main card: There is a scarlet fever-like rash on the back of the chest. There is a tendency to fuse, the color fades, the systemic symptoms have fever, joint pain, fatigue, weakness, loss of appetite, red tongue, little or no moss, and fine pulse.

Dialectical syndrome: Yin deficiency heat, blood and blood loss.

Differential diagnosis

1. The symptoms of sepsis poisoning are heavy, the general condition is poor, the rash is mostly hemorrhagic, the blood culture is positive, and there is no intermittent development, and antibiotic treatment is effective.

2. Rheumatic fever rash is mainly ring erythema and subcutaneous nodules, often accompanied by endocarditis.

3. Rheumatoid disease is characterized by invasion of small joints, pain and joint deformity, and more rheumatoid factor.

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