The most important role in the treatment of a rare disease is played by diagnostics, which makes it possible to differentiate the disease from a number of other he alth disorders. The treatment of Still's disease is long, but the prognosis for patients who turn to a specialist in time is good in most cases.
Case history
The disease was first known in 1897 thanks to the doctor George Still. But at this time, Still's disease was considered only one form of rheumatoid arthritis. It wasn't until 1971 that Eric Bywaters published studies distinguishing the disease from a range of problems with similar symptoms.
Medical statistics show that both men and women can get sick with a rare disease equally. However, most patients who see a doctor with symptoms of Still's disease and receive a confirmatory diagnosis are children under 16 years of age. Given such a young age of most patients, the diagnosis is complicated, since it is difficult for parents who first notice the symptoms in a child to believe in the presence of such an "age"problems.
Causes of disease development
Until now, the etiology of the development of the disease is unclear. Numerous studies aimed at identifying the factor that provoked Still's disease in adults and children have not yielded results. According to the generally accepted hypothesis, the disease is the result of exposure to infectious or viral agents, but there is no evidence to support this statement.
Doctors tried to associate the disease with pregnancy, the use of medications, including female hormones, stress and other factors. According to some reports, Still's disease refers to autoimmune disorders. It has only been reliably proven that in the active phase the disease is characterized by changes in the concentration of cytokines. Perhaps in the future, medicine will be able to identify the cause of the disease, thereby simplifying its diagnosis and speeding up the recovery of patients.
Symptoms of Still's disease in adults
Still's disease, the symptoms of which are similar to a number of other diseases, is diagnosed by several indicators:
- The fever associated with Still's disease differs from the fever associated with a number of infectious diseases in its inconstancy. Traditionally, the temperature during the day is kept within the normal range, but twice a day it rises to 39 degrees and above. At the same time, in a fifth of patients, there is no decrease in temperature indicators and improvement in the patient's well-being, which significantly complicates the diagnosis.
- At the top of the risetemperature, the patient develops skin rashes, represented by pink papules or maculae. Most often, the rash is located on the trunk and in the proximal parts of the limbs, less often - the picture of the rash is presented on the face. In a third of patients, the rash rises above the skin cover, occurring mainly in places of friction and compression. This symptom is called the Koebner phenomenon. The rash is not always accompanied by itching, and its rather pale pink color and periodic disappearance make the symptom invisible to the patient. Doctors, in order to make an accurate diagnosis, are forced to expose the patient to heat, which activates the manifestation of rashes. This may be a warm shower or the application of warm towels. Atypical manifestations of the disease are: alopecia, erythema nodosum, petechial hemorrhages. But in practice, such symptoms are extremely rare.
- The initial stage of Still's disease is characterized by the appearance of myalgia and arthralgia. In this case, only one joint is initially affected. Over time, the disease covers other joints, taking on the character of polyarthritis. First of all, the knee, ankle, wrist, hip, temporomandibular, metatarsophalangeal joints are affected. But the feature of the disease, the most typical for most cases, is the development of arthritis of the distal interphalangeal joints of the hands. This is what differentiates Still's disease from rheumatic fever, systemic lupus erythematosus, or rheumatoid arthritis.
- In 65% of patients, on the background of the disease,lymphadenopathy. Half of the patients have enlarged cervical lymph nodes. In some cases, lymphadenitis takes on a necrotic character.
- At the onset of the disease, patients notice a pronounced burning sensation in the throat, which is permanent.
- Still's disease is also characterized by cardiopulmonary manifestations, such as aseptic pneumonitis, cardiac tamponade, valvular vegetations, respiratory distress syndromes.
- Patients also have ophthalmic disorders. These are complicated cataracts, ribbon-like degeneration of the cornea of the eye, iridocyclitis.
Still's disease in children
Symptoms of the disease in children do not differ from those in adults. However, the manifestations of Still's disease in childhood can be blurred, which leads to delayed diagnosis and untimely treatment. Sometimes polyarthritis in childhood leads to disability. To avoid this, parents should be extremely attentive to the physical condition of the child. And at the first symptoms of the disease, contact a qualified specialist. Advanced Still's disease in children can provoke disproportionate growth of the limbs, which will require surgical intervention.
Diagnosis of disease
Since there are practically no specific symptoms of the disease, diagnosis is difficult. In every twentieth case, Still's disease is treated as a fever of an unexplained nature. In some cases, a diagnosis of sepsis is made. And only after the seriesunsuccessful courses of antibiotic therapy and a number of additional tests, the doctors come to the conclusion that this is adult Still's disease. Treatment and rehabilitation are long-term procedures that require constant medical supervision. The same applies to children.
When making a diagnosis, both symptoms in the form of fever, swelling of the joints, swollen lymph nodes and inflammation of the throat, as well as other indicators obtained using an echocardiogram, computed tomography and ultrasound, are taken into account. A blood test is also required, showing the level of platelets and leukocytes. With Still's disease, the patient is characterized by a decrease in the level of red blood cells. In adult patients, C-reactive protein and ferritin are elevated. However, tests for antinuclear antibodies and rheumatoid factor are usually negative.
Treatment during remission
Complex and phased treatment is carried out both in the active phase of the disease and in remission. In the first case, the procedures are performed in a hospital, in the second case, patients receive the necessary therapy while on outpatient treatment or in sanatoriums and resorts. Therapy includes medication, physiotherapy, exercise therapy and massage.
Treatment during an exacerbation of the disease
During the exacerbation of the disease, patients take NSAIDs, immunosuppressants, glucocorticoids. Treatment is always long. That is why both the patient himself and his relatives need to stock up on a large dose of patience. Still's disease inadults and children - the disease is serious, and it is possible to cope with it only with timely diagnosis and well-prescribed treatment.
Forecast
After treatment, three forms of the development of the disease are possible. The best of them is spontaneous recovery, observed in a third of patients with a rare diagnosis. Another third of patients have a periodically relapsing form of the disease. The most difficult option is chronic Still's disease in adults. Treatment, recovery and rehabilitation in this case may include not only traditional therapy, but also arthroplasty, which allows to restore the joints destroyed by the disease.
