Still's disease in adults and children: symptoms, treatment, chances of recovery
Still's disease is a severe multi-system pathology, the causes of its origin and development are still unclear.In this disease joints, lymphoreticular, cardiovascular and respiratory systems of a person are affected.
For the first time, Still's disease was described at the end of the 19th century, and for many years it was considered as one of the varieties of juvenile rheumatoid arthritis.By the 70s of the last century, a lot of information about the possibility of developing this pathology in adults, although the bulk of patients really are children and adolescents under 16 years old. Treatment is in the competence of rheumatologists .For therapy, mainly NSAIDs and preparations of glucocorticoid hormones are used, and cytostatics are used for the reserve.Differential diagnosis is carried out using an exclusion method.Table of contents:
The exact causes of the disease have not been established to date.For Still's disease, a sharp start is characteristic, suggesting an infectious etiology.However, a common infection agent for all cases has not been identified to date.
Pathogenic microorganisms that were detected in different patients:
- Yersinia( Yersinia enterocolitica);
- mycoplasmas( Mycoplasma pneumonia);
- parainfluenza virus.
According to some experts, there is a family( genetically determined) predisposition to the disease .According to the genetic theory, there is a possible connection of Still's disease with disorders in the tissue compatibility complex.Some researchers tend to regard this pathology as autoimmune.
Symptoms of Styla disease
Leading symptoms of Still's disease:
- articular syndrome;
- transient skin rashes;
- febrile reaction( characterized by a "high" fever);
- cardiopulmonary pathology;
- lesion of the lymphoreticular system( lymphadenopathy and hepatosplenomegaly).
When a laboratory study of a patient's blood reveals a significant leukocytosis with an increase in the level of neutrophilic granulocytes and an increase in the rate of erythrocyte sedimentation.
Note: manifestations of the disease are unspecific, so patients are often mistakenly diagnosed with "sepsis" and prescribe long courses of antibiotics.
The articular syndrome is manifested by arthralgia and myalgia at the peak of temperature( joint and muscle pain). At the initial stage of the development of the Still disease, an isolated inflammatory lesion of only one joint - arthritis can be noted.Further, other moving joints are involved in the process, that is, polyarthritis develops.The defeat of the interphalangeal distal joints of the hands is very characteristic.The symptom allows differential diagnostics with SLE, rheumatic fever and rheumatoid arthritis, in which these joints in young patients practically do not suffer.
The temperature rises to high figures - 39 ° C or more, but it does not hold permanently as in most infectious pathologies.It is typical to have a one-time increase during the day, usually in the evening.In some cases, 2 temperature peaks are recorded during the day.Between them, the temperature returns to normal physiological values;In parallel, the well-being improves.
Important: in approximately 1 in 5 patients, hyperthermia does not disappear, i.e., fever persists.
Transient skin rashes appear at the peak of the fever, and spontaneously disappear as the general condition improves.The most common are papules( small dense nodules) and maculae( spots of pink color). Primary localization of the rash - proximal parts of the arms and legs and trunk;In some patients, cutaneous elements are identified on the face.In about 30% of cases, they develop with compression or trauma to the skin( the so-called Köbner phenomenon is observed).In some cases, patients may complain of skin itching, but more often unpleasant sensations are absent.The rash often goes unnoticed;In order to reliably confirm its presence in the course of diagnosis, a specialist has to resort to provoking thermal effects.
Atypical skin manifestations:
- hair loss;
- petechiae( pinpoint hemorrhage);
- erythema nodosum.
Manifestations from the lymphoreticular system
With Still's disease, lymphoreticular organs suffer.Almost 70% of patients are diagnosed with lymphadenopathy;In 50% of cases, cervical lymph nodes increase.They retain mobility, and with palpation a moderately dense consistency is determined.
Important: adhesion of lymph nodes to surrounding tissues requires additional research to exclude cancer.
In rare cases( if atypical course), necrotic lesions of the lymph nodes are possible.
Many patients are diagnosed with hepatosplenomegaly - a significant increase in the liver and spleen.
Lesion of other organs
When the pathological process of the respiratory system and the heart is involved, inflammation of the serous membranes of the organs - the pleura and pericardium - is most often developed.Often pleurisy and pericarditis develop in parallel.Each fifth patient is diagnosed with aseptic pneumonitis, which is symptomatic of bilateral pneumonia.Hyperthermia, cough and shortness of breath do not disappear with the use of antibiotics.
Possible inflammation of the heart muscle;Tamponade of the heart is not excluded.
Important: 2/3 of the patients complain of a constant burning sensation in the throat.This symptom appears at the very beginning of the disease.With Still's disease, severe damage to the organs of vision is possible - complicated cataracts, ribbon-like degeneration of the cornea, or iridocyclitis.
The diagnosis of "Still's disease" presents certain difficulties;It in most cases requires a long observation of the patient and the exclusion of other pathologies that have similar clinical manifestations. The study of laboratory data is of great importance.
Changes in blood tests:
- significant neutrophilic leukocytosis;
- increase in ESR( over 50 mm / hour);
- presence of "acute phase proteins"( ferritin, C-reactive);
- increased activity of liver enzymes.
Note: result of bacterial blood culture is negative, which allows to exclude sepsis.Antinuclear and rheumatoid factor are not detected, despite the symptoms inherent in a number of systemic inflammatory diseases.
Additional diagnostic methods
In the course of X-ray diagnostics in patients suffering from Still's disease, the presence of effusion in the articular cavities is revealed.Sometimes there is a change in the structure of bone tissue - osteoporosis.If the pathology acquires a chronic course, a picture typical of ankylosis is seen on the x-ray of the hands.
During joint puncture, a synovial fluid with signs of an inflammatory reaction is obtained;Bacteria are not found in it.
To exclude malignant tumor lesions of lymph nodes, they are punctured with fence and subsequent cytological and histological study of tissue samples.
If there is cardiopulmonary symptoms, additional consultation of specialists in the field of cardiology and pulmonology is required, as well as ultrasound scanning of the thorax and the study of the electrocardiogram.
Pathologies with which differential diagnosis is performed:
- systemic vasculitis;
- arthritis( rheumatoid and psoriatic);
- granulomatous hepatitis;
- endocarditis of infectious genesis.
Treatment of the disease Stylla
The medical tactics depend on the clinical course of the disease. In the acute phase, pharmacotherapy is performed using non-steroidal anti-inflammatory drugs. They give a positive effect in every fourth case.The duration of the course of treatment varies from 1 to 3 months.
In the presence of pathological changes on the part of the heart and respiratory system, glucocorticoids - Dexamethasone or Prednisolone - are shown.
If Still's disease takes a chronic course, then the cytostatic methotrexate, which is a structural analogue of folic acid, is prescribed to reduce the dose of hormones.A reserve cytostatic agent prescribed in severe cases is cyclophosphamide.
If the pathology can not be treated according to the standard scheme, the immunosuppressant Infliximab and the powerful immunomodulator Etanercept can be used.
Prediction: recovery from Still's disease
Possible outcomes of Still's disease can be spontaneous recovery.It occurs in about 30% of patients for 6-9 months.From the appearance of the first symptoms. Often the disease passes into a chronic or recurrent form.
In a recurrent form, as a rule, there is a single exacerbation;Less repeated attacks are fixed.
The most severe is the chronic form;It is characterized by severe polyarthritis symptoms with a significant limitation of the amplitude of movements in the affected joints.
If pathology develops in adults, a five-year survival forecast is 90-95%, which is comparable to that of systemic lupus erythematosus. Causes of death include coagulation disorders, respiratory distress syndrome, amyloidosis, or secondary bacterial infections( including pulmonary tuberculosis).
Совинская Елена, medical reviewer