How does the disease manifest itself?
The most characteristic symptom is a special rhythm of temperature: one or two rises per day, usually in the evening, to high values, followed by a return to normal and relatively good health between attacks. At the peak of the fever, a pink spotty rash appears, often on the torso and limbs, which lasts for several hours and disappears without a trace, so it is often not seen. The joints hurt and swell, most often the wrist, knee and ankle are affected. Many patients report severe sore throat at the onset of the disease in the absence of purulent sore throat.
- Daily high temperatures
- Pink rash at the peak of a fever
- Joint pain and swelling
- Sore throat without purulent plaque
- Enlarged lymph nodes
- Enlarged liver and spleen
- Severe weakness and weight loss
Why is this a diagnosis of exclusion?
There is no test that directly confirms Still's disease. Many conditions give a similar picture: severe bacterial and viral infections, tuberculosis, inflammation of the inner lining of the heart, lymphomas and other tumors, systemic lupus erythematosus, vasculitis, drug reactions. They all require completely different treatment, and some require urgent treatment. Therefore, the doctor consistently excludes these causes, and only after that makes a diagnosis based on a combination of signs. This is why the examination may take time, and starting treatment with hormones before an infection has been ruled out is dangerous.
- There is no specific analysis
- Rule out infections, including tuberculosis
- Rule out lymphomas and other tumors
- Lupus and vasculitis are excluded
- Take into account drug reactions
- Diagnosis is made based on a combination of criteria
Laboratory changes
The tests show a pronounced inflammatory reaction: ESR and C-reactive protein are significantly increased, the number of leukocytes is increased with a predominance of neutrophils, and hemoglobin is often decreased. Ferritin occupies a special place: with this disease it increases tens of times more than with normal inflammation, and although it does not prove the diagnosis in itself, it serves as an important clue. Rheumatoid factor and antinuclear antibodies, characteristic of other rheumatic diseases, are usually negative, and this is also used as a sign. Liver parameters are often elevated.
- Significant increase in ESR and C-reactive protein
- Leukocytosis with neutrophil predominance
- Very high ferritin
- Negative rheumatoid factor and antinuclear antibodies
- Anemia
- Increased liver parameters
Survey
The examination program is broad, because the task is not to miss another cause of fever. They take blood and urine cultures, conduct tests for viral hepatitis, HIV and tuberculosis, perform an X-ray or tomography of the chest, ultrasound of the abdominal cavity, and echocardiography to exclude damage to the heart valves. They examine the joints and, if necessary, do an ultrasound. If the lymph nodes are enlarged, a biopsy may be needed to rule out lymphoma. The decision on the set of studies is made by the doctor, focusing on a specific picture.
- Blood and urine cultures
- Examination for tuberculosis, hepatitis, HIV
- X-ray or tomography of the chest
- Ultrasound of the abdominal cavity and lymph nodes
- Echocardiography
- Lymph node biopsy for suspected lymphoma
- Examination and ultrasound of joints
Treatment and course
Treatment is prescribed by a rheumatologist. In mild cases, they begin with anti-inflammatory drugs, but more often the use of glucocorticoids is required, which quickly relieve fever and pain. To reduce the dose of hormones and maintain remission, basic drugs are used, and if the disease persists, modern genetically engineered drugs are used that block key inflammatory signals. The timing and dosage are selected individually; self-cancellation is unacceptable. The course is of three types: a single episode with recovery, repeated exacerbations and chronic arthritis with gradual damage to the joints. Therefore, observation continues even after improvement.
- Anti-inflammatory drugs for mild cases
- Glucocorticoids as the basis for exacerbation therapy
- Basic drugs to maintain remission
- Genetic engineering therapy for persistent disease
- Gradual dose reduction only under the supervision of a physician
- Observation by a rheumatologist and control of tests
- Timely treatment of infections during therapy