Guidelines for the diagnosis and treatment of Steele’s disease in adults (draft)
Source: Chinese Medical Association, Division of Rheumatology
Overview】 Steele’s disease originally refers to juvenile chronic arthritis of systemic origin, but a similar disease can also occur in adults, and is called adult onset still’s disease AOSD. This disease was once called “allergic subseptic disease”, but since 1987 it has been unified as adult Still’s disease. Du Mingrui, Department of Rheumatology, The First Affiliated Hospital of Henan College of Traditional Chinese Medicine The cause of this disease is still unknown. Clinical features are fever, arthralgia and/or arthritis, rash, myalgia, sore throat, enlarged lymph nodes, neutrophilia, and thrombocytosis, with systemic damage in severe cases. Since there are no specific diagnostic methods or criteria, infections, tumors, and other connective tissue diseases need to be excluded before considering the diagnosis. In some patients, even if the diagnosis of adult Still’s disease is made, close follow-up during treatment is required to further exclude the possibility of these diseases. The prevalence of the disease is similar in men and women and is scattered throughout the world with no geographical differences. The age of prevalence is 16-35 years old, and the onset of the disease can be seen at an advanced age. (1) Fever is the most common and earliest symptom of the disease. Other manifestations such as rash, joint and muscle symptoms, and increased peripheral blood leukocytes may appear weeks or even months after the onset of fever. 80% or more of patients have a typical remittent fever, usually with a sudden rise in body temperature of 39℃ or more in the evening, with or without chills, but the temperature may drop to normal on its own in the morning of the next day without heat remission. Usually the temperature peak is once a day, but twice a day is rare.
(2) Rash is another major manifestation of the disease, seen in more than 85% of patients, the typical rash is orange-red macular rash or maculopapular rash, sometimes the rash form is variable, may be urticaria-like rash. The rash is mainly distributed on the trunk and extremities, but can also be seen on the face. The rash is often accompanied by a fever, often appearing in the evening when the fever starts, and disappearing the next morning when the fever subsides. Another skin abnormality is the diffuse erythema of the skin with mild itching due to mechanical irritation such as folding of clothes, bedding, scratching or hot baths, a phenomenon known as Koebner’s phenomenon, seen in about 1/3 of patients. (3) Joints and muscles Almost 100% of patients have joint pain and arthritis in more than 90%. The knee and wrist joints are most commonly involved, followed by the ankle, shoulder and elbow joints. The number of joints involved is small in the early stages of the disease, but may increase later to become polyarthritic. In many patients, the cartilage and bone tissue of the affected joints may be damaged by erosion, so joint stiffness and deformity may occur in the late stage. Muscle pain is common, accounting for more than 80% of cases. Most patients have varying degrees of muscle pain with fever, and some patients have muscle weakness and mildly increased muscle enzymes. (4) Sore throat Most patients have sore throat in the early stage of the disease, sometimes present throughout the course of the disease, and the sore throat appears or worsens during fever and relieves after the fever subsides. There may be pharyngeal congestion, lymphatic follicle hyperplasia in the posterior pharyngeal wall and tonsillar enlargement, and negative pharyngeal swab culture. (5) Other clinical manifestations There may be peripheral lymph node enlargement, hepatosplenomegaly, abdominal pain (a few resemble acute abdomen), pleurisy, pericardial effusion, myocarditis, pneumonia. Less common are renal, central nervous abnormalities, and peripheral nerve damage. A few patients may develop acute respiratory failure, congestive heart failure, pericardial tamponade, constrictive pericarditis, diffuse intravascular coagulation (DIC), severe anemia, and necrotizing lymphadenopathy. 2, laboratory tests (1) blood routine In the active phase of the disease, more than 90% of patients have increased neutrophils, about 80% of patients have blood leukocyte count ≥ 15 × 109 / L. About 50% of patients have elevated platelet count, eosinophils do not change. Orthocytic orthochromic anemia may be combined. Almost 100% of patients have increased hematocrit. (2) Liver enzymes are mildly elevated in some patients. (3) Blood bacterial culture is negative. (4) Negative rheumatoid factor and antinuclear antibodies, only a few may be low titer positive. Blood complement level is normal or high. (5) Serum ferritin SF The SF level is increased in this disease, and its level is positively correlated with the disease activity. Therefore, SF not only contributes to the diagnosis of the disease, but also has some significance in determining whether the disease is active and evaluating the effect of treatment. (6) The synovial fluid and plasma cavity effusion has increased leukocytes, showing inflammatory changes, among which neutrophils are mainly increased. Radiological manifestations In patients with arthritis, there may be periarticular soft tissue swelling and osteoporosis at the end of the joint bones. As the disease progresses, there may be destruction of articular cartilage and narrowing of the joint space, and such changes are most likely to occur in the wrist joint. The subchondral bone may also be destroyed, which may eventually lead to joint stiffness and deformity. Diagnosis and Differential Diagnosis】 1. If the following clinical manifestations and positive laboratory test results occur, the disease should be suspected. (1) Fever is the most prominent symptom of the disease and appears at the earliest, and the typical fever pattern is flaccid fever, usually once a day. (2) The rash is common on the trunk and extremities, but can also be seen on the face as an orange-red rash or maculopapular rash, usually accompanied by fever, and is transient. (3) There is usually arthralgia and/or arthritis, with early oligoarthritis or polyarthritis developing. Myalgic symptoms are also common. (4) Peripheral blood leukocytes are significantly elevated, mainly neutrophils, and blood cultures are negative. (5) Serological examination: most patients are negative for rheumatoid factor and antinuclear antibodies. (6) A variety of antimicrobial therapy is ineffective, while glucocorticoids are effective. There is no specific diagnostic method for this disease, and many diagnostic or classification criteria have been developed at home and abroad, but there is still no unified standard. However, there is no unified standard yet. We recommend the American Cush criteria and the Japanese criteria, which are more commonly used. (1) Cush criteria Required conditions Fever ≥ 39℃ Arthralgia or arthritis Rheumatoid factor