Steele’s disease is originally a juvenile form of arthritis with a systemic origin, but a similar disease can also occur in adults and is called Adult Steele’s disease (AOSD). The name “allergic subseptic disease” was once used.
It is characterized by fever, arthralgia and/or arthritis, rash, myalgia, sore throat, enlarged lymph nodes, leukocytosis and neutrophilia, and thrombocytosis, with severe systemic damage. Because there are no specific diagnostic criteria, the diagnosis is often considered only after excluding infections, tumors and other connective tissue diseases. In some patients, even if the diagnosis of adult Still’s disease is made, close follow-up during treatment is required to further exclude these diseases. The prevalence of the disease is similar in men and women and is scattered worldwide without geographical differences. The age of onset is between 16 and 35 years, but it can also be seen at an advanced age.
I. Key points of medical history taking
1. History of present illness
(1) Describe in detail the onset of the disease: the urgency of the onset, the time of fever, the range of fluctuation, the fever type, the accompanying symptoms; the rash form, distribution, duration, and relationship with fever; the presence of polyarticular pain and muscle pain, the location, degree, duration and relationship with fever; the presence of sore throat. The results of tests such as routine examinations, serological examinations, X-rays, etc., the treatment used (especially antibiotics and glucocorticoids) and its response to treatment.
(2) Symptoms and manifestations of visceral involvement: yellowing of the skin and sclera, chest tightness, shortness of breath, palpitations, edema, abdominal pain and other symptoms.
(3) Current symptoms and general condition of the patient.
(2) Past history: history of medication use before the rash.
(3) Family history: History of autoimmune disease in the immediate family.
II. Key points of physical examination
1. Skin and mucous membranes: characteristics, distribution, extent and degree of rash, presence of koebner’s phenomenon, etc. The presence of pale and yellowish skin and mucous membranes.
2. System examination: presence of pharyngeal congestion, enlarged tonsils, abnormal secretions, etc. The liver, spleen and lymph nodes are large. Lung involvement can be heard in the woven P-brain whoosh Jianxu clip pregnancy mu caries伎晌偶靶陌Σ烈簟
3, limb joints: whether there is joint swelling, pressure pain, deformity and functional limitation, whether there is muscle pressure pain, whether muscle strength is abnormal, whether the tendon reflex is normal and symmetrical. Whether the sensation is normal.
4. Pay attention to finding foci of infection, such as paranasal sinuses, tonsils, liver, gallbladder and urinary system, etc.
Auxiliary examinations
1.Laboratory examination
(1) Blood, urine and stool routine, biochemistry, ESR, CRP, eosinophil count, Ig, protein electrophoresis, etc. In the active stage of the disease, more than 90% of patients have increased neutrophils, and about 80% of patients have blood leukocyte count ≥ 15×109/L.
(2) Immunological tests: anti-nuclear antibody, anti-ENA seven, RF, anti-mDNA, ANCA, anti-adhesion point antibody, cold globulin, ASO, etc.
(3) Serum ferritin (SF): significantly increased and its level correlates with disease activity.
(4) Infection-related tests: blood (or bone marrow), middle urine and pharyngeal swab culture; anti-EBV/CMV/fine virus antibodies, anti-mycoplasma/chlamydia/legionella antibodies, Fertilizer extracellular reaction, etc.
2.Special tests
(1)PPD test.
(2)X-ray examination: lung and diseased joints.
(3)Ultrasound: liver, bile, pancreas, spleen, both kidneys and abdominal lymph nodes.
(4) Bone marrow smear examination, including routine classification and looking for parasites (bone marrow culture if necessary).
(5) Lymph node or rash biopsy, joint cavity aspiration and joint fluid culture (if necessary).
IV. Diagnostic and differential diagnosis points
1.Diagnostic points
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. The more commonly used American Cush criteria (see Table 11-1), Japanese criteria (see Table 11-2) and the diagnostic criteria developed by the American College of Rheumatology (see Table 11-3) are recommended.
Table 11-1 Cush criteria
Required conditions
Fever ≥ 39°C
Arthralgia or arthritis
Rheumatoid factor <1:80
Antinuclear antibodies <1:100
Any two of the following must also be present
Blood leukocytes ≥15×109/L
Skin rash
Pleurisy or pericarditis
Hepatomegaly or splenomegaly or lymph node enlargement
Table 11-2 Japanese preliminary diagnostic criteria
Major conditions
Fever ≥39℃ and lasting for more than 1 week
Arthralgia lasting for more than 2 weeks
Typical skin rash
Leukocytes ≥15×109/L
Secondary conditions
Sore throat
Lymph nodes and/or splenomegaly
Abnormal liver function
Negative rheumatoid factor and antinuclear antibodies
This criterion requires exclusion of: infectious diseases, malignancies, other rheumatic diseases. A diagnosis is made when five or more conditions (including at least two major conditions) are met.
Table 11-3 Diagnostic criteria established by the American College of Rheumatology (1987)
Major conditions
Persistent or intermittent fever
Transient orange-red rash or maculopapular rash
Poly/oligoarthritis
Elevated white blood cells or neutrophils
Secondary conditions
Sore throat, abnormal liver function, enlarged lymph nodes, splenomegaly and other organ involvement with the 4 primary conditions listed above will confirm the diagnosis. The presence of 2 of the primary conditions of fever and rash, plus 1 or more of the secondary conditions, may lead to suspicion of the disease.
It is important to emphasize that adult sclerosis is a diagnosis of exclusion and there is still no specific unified diagnostic criteria.
2.Common differential diagnosis
(1) Infectious diseases: viral infections (hepatitis B virus, rubella, microvirus, coxsackievirus, EBV, cytomegalovirus, human immunodeficiency virus, etc.), infective endocarditis, meningococcal bacteremia, gonococcal bacteremia and other bacterial-induced bacteremia or sepsis, tuberculosis, Lyme disease, syphilis, and rheumatic fever, etc.
(2) Malignancies: leukemia, lymphoma, immunoblast lymphadenopathy.
(3) Connective tissue diseases: systemic lupus erythematosus, primary dry syndrome, mixed connective tissue disease and other vasculitis-like nodular polyarteritis, Wegener’s granulomatosis, thrombotic thrombocytopenic purpura, aortitis, etc.
(4) Other diseases: serum sickness, nodular disease, primary granulomatous hepatitis, Crohn’s disease, drug allergy.
V. Treatment plan
1.Drug treatment
(1) Non-steroidal anti-inflammatory drugs (NSAIDs): NSAIDs can be used first in the acute febrile inflammatory period, and generally require larger doses, which should be continued for 1~3 months after remission, and then gradually reduced. Patients with gastrointestinal disease should be given priority to selective COX-2 inhibitors.
(2) Adrenal glucocorticosteroids: Glucocorticosteroids should be used for patients who do not respond to NSAIDs alone, whose symptoms are not well controlled, or who have relapsed with dose reduction, or who have systemic damage and are in serious condition. Prednisone is commonly used at 0.5 mg to 1 mg/kg per day, and the dosage can be gradually reduced after one month of symptom control and disease stabilization, and maintained at the minimum effective amount. For severe cases, methylprednisolone shock therapy can be used.
(3) Improve the condition of anti-rheumatic drugs (DMARDs): hormones still can not control fever or hormone reduction relapse; or arthritis manifestation should be added as soon as possible DMARDs. chronic systemic lesions in milder cases, such as fever, weakness, rash, pluritis, etc. can be used hydroxychloroquine. Methotrexate (MTX) at a dose of 7.5 to 20 mg/week is preferred in those with significant joint lesions. If MTX is not tolerated by the patient, it can be replaced by leflunomide (LEF), which can also be combined with other DMARDs on top of LEF. In more persistent cases, cyclophosphamide, azathioprine and cyclosporine A may be considered, and when the chronic phase is characterized by arthritis, the combination of rheumatoid arthritis DMARDs may be used. MTX+CTX can also be used when remission is difficult with multiple drug therapy.
(4) Botanical preparations: In the chronic phase of the disease, when arthritis is the main manifestation, they can also be used under observation, such as tretinoin 30 mg～60 mg/day; cyanophylline 20 mg～80 mg, 3 times/day; white medicine total glucoside 600 mg 2-3 times/day.
(5) Other drugs Anti-tumor necrosis factor – has been applied abroad. Intravenous injection of gammaglobulin is still controversial.
2.Surgical treatment
If there is joint erosion and destruction or deformity, the patient should refer to the surgical treatment of rheumatoid arthritis and perform arthroplasty, soft tissue disintegration or repair and joint fusion, but postoperative medication is still needed.
Prognosis estimation
The patient’s condition and disease course are diverse. A small percentage of patients have remission after one episode, but most patients are prone to recurrent episodes after remission. 40%-50% of patients have a tendency to self-limit, but there are also types of chronic persistent activity, and those without rash and HLA-B35 positive have milder disease. 20% have chronic arthritis, with cartilage and bone destruction. The causes of death are varied, mainly due to secondary infections, adverse reactions to glucocorticoids, liver failure and other multi-organ damage. The presence of Still’s disease, persistent RF, ANA positivity, and HLA-DR6 positivity at a young age suggest a poor prognosis.