Still’s disease is a serious disease manifested by fever, polyarthritis. Transient skin rashes and systemic inflammatory lesions of somatic organs. Pathology is diagnosed using the method of excluding other diseases based on clinical symptoms, laboratory data, results of examination of affected joints, lymphoreticular and cardiopulmonary systems. Treatment is carried out mainly with nonsteroidal anti-inflammatory and glucocorticoids, cytostatics are the reserve drugs.
Meaning
Still’s disease was described in 1897 by the British physician George Still. For a long time it was considered a severe form of juvenile rheumatoid arthritis. It was not until 1971 that Eric Bywaters published numerous observations of this disease in adult patients. According to statistics provided by modern world rheumatology, the prevalence of Still’s disease in recent years is approximately 1 person per 100 thousand population. Female and male persons are equally susceptible to morbidity. The largest number of cases occur in children under the age of 16.
Due to the absence of specific symptoms of the disease, patients often, despite the negative results of bacteriological blood cultures, are diagnosed with sepsis, for which they undergo repeated courses of antibiotic therapy. It is noted that about 5% of cases are initially treated by doctors as “fever of unknown genesis”.
Causes
Numerous studies in the field of the etiology of Still’s disease have not given an answer to the question of its causes. Sudden onset, high fever, lymphadenopathy and leukocytosis of the blood indicate the infectious nature of the disease. However, a single pathogen has not yet been identified. In some cases of Still’s disease, rubella virus was detected in patients, in others — cytomegalovirus. Cases of the disease associated with the parainfluenza virus, Epstein-Barr virus, mycoplasma, Escherichia have been observed.
It is impossible to exclude the presence of a hereditary predisposition to the development of Still’s disease. But the final results confirming the connection of the disease with HLA loci have not yet been obtained. The immunological theory relating disease to autoimmune diseases is confirmed only in some cases, when patients are found to have CEC causing the development of allergic vasculitis.
Symptoms
Fever in Still’s disease is characterized by a rise in temperature to high numbers (39 ° C and above). Unlike most infectious diseases, it is not permanent. The most characteristic is a one-time temperature rise during the day, usually in the evening. Less often there are 2 temperature peaks per day. In most patients, the temperature between peaks decreases to normal figures, which is accompanied by a significant improvement in the general condition. Approximately 20% of patients have no normalization of body temperature.
Rashes in Still’s disease, as a rule, occur at the height of the rise in body temperature and are of an incoming nature: they disappear, then reappear. The elements of the rash are mainly represented by flat pink spots (maculae) or papules located in the proximal parts of the limbs and on the trunk, less often on the face. In 30% of cases of Still’s disease, rashes rise above the general surface of the skin and occur in places of injury or compression of the skin (Kebner phenomenon). Sometimes they are accompanied by itching. The pink color of the rash, its periodic disappearance and the absence of subjective sensations often make the rash invisible to patients. In some cases, to detect a rash, the doctor has to examine the patient immediately after a warm shower or resort to thermal effects on the skin, for example, by applying warm wipes. There are atypical skin manifestations of Still’s disease: petechial hemorrhages, nodular erythema, alopecia.
Articular syndrome. Arthralgias, along with myalgias, at the beginning of Still’s disease are attributed to the general manifestations of the disease caused by a high rise in temperature. At the initial stage, arthritis can affect only one joint. Then the lesion takes on the character of polyarthritis involving the ankle, knee, wrist, elbow, hip, temporomandibular, interphalangeal, metatarsal-phalangeal joints. The most typical for Still’s disease is the development of arthritis of the interphalangeal distal joints of the hand. This feature makes it possible to differentiate the disease from rheumatoid arthritis, rheumatic fever, systemic lupus erythematosus, which are not characterized by the defeat of these joints at a young age.
Damage to the lymphoreticular organs includes hepatosplenomegaly and lymphadenopathy. 65% of patients with Still’s disease have lymphadenitis. In half of the cases of the disease, there is an increase in cervical lymph nodes. Enlarged lymph nodes in Still’s disease retain their mobility, have a moderately dense consistency. Pronounced compaction of the lymph node, its isolated enlargement or solidity with surrounding tissues should be alarming in oncological terms. In atypical cases, lymphadenitis can take on a necrotic character.
Sore throat bothers 70% of patients with Still’s disease and usually manifests itself at the beginning of the disease. It is characterized by a pronounced burning sensation in the throat and is permanent.
Cardiopulmonary manifestations of Still’s disease are most often in the nature of serositis: pleurisy and/ or pericarditis. In 20% of cases, aseptic pneumonitis is observed, often occurring with symptoms of bilateral pneumonia (cough, shortness of breath, high fever), which do not go away against the background of intensive antibiotic therapy. More rare lesions occurring in Still’s disease include: myocarditis, cardiac tamponade, the appearance of valvular vegetations with a clinical picture of infectious endocarditis, respiratory distress syndrome.
Diagnostics
The absence of specific diagnostic signs of Still’s disease makes its diagnosis difficult for a rheumatologist, requiring a certain period of patient observation and often based on the exclusion of other diseases. In a clinical blood test, pronounced leukocytosis and accelerated ESR are noted. The vast majority of patients have ESR above 50 mm/h. The biochemical analysis of blood reveals an increased level of proteins characteristic of the acute inflammatory phase: CRP, ferritin, serum amyloid A. At the same time, despite the clinical signs of pronounced systemic inflammation typical of Still’s disease, rheumatoid and antinuclear factors are not detected in the blood, and the back-up of blood for sterility gives a negative result. Biochemical tests of the liver show an increase in the activity of its enzymes.
X—ray examination of the joints reveals effusion in the joint cavity, swelling of soft tissues, less often – osteoporosis of the bones forming the joint. In patients with a chronic form of Still’s disease, the presence of ankylosis in the wrist joints is typical. During joint puncture, aseptic synovial fluid with inflammatory changes is obtained.
If necessary, patients with Still’s disease undergo a biopsy of the lymph node, which allows to exclude its malignant metastatic lesion. Cardiopulmonary manifestations of Still’s disease require consultation with a cardiologist and pulmonologist, lung x-ray, ultrasound of the pleural cavity, ECG, ultrasound of the heart, etc. Differential diagnosis of Still’s disease is carried out with rheumatoid arthritis, psoriatic arthritis, dermatomyositis, lymphoma, tuberculosis, sarcoidosis, granulomatous hepatitis, infectious endocarditis, systemic vasculitis, etc.
Treatment
In the acute period, prescribing drugs from the group of nonsteroidal anti-inflammatory drugs is sufficient for 25% of patients. Their reception, depending on the clinic of Still’s disease, takes from 1 to 3 months. Changes in the heart and lungs are an indication for glucocorticosteroid therapy with prednisone or dexamethasone. However, these drugs do not always have a sufficient effect. In the chronic course of Still’s disease, methotrexate can be used to reduce the dose of corticosteroids. Cyclophosphamide may be a reserve drug for patients with severe forms of the disease. In some cases of Still’s disease resistant to traditional treatment, infliximab and etanercept may be used.
Forecast
The outcome of Still’s disease may be spontaneous recovery, transition to a recurrent or chronic form. Recovery occurs in 1/3 of patients, usually within 6-9 months from the onset of the disease. The recurrent course of Still’s disease in 2/3 of patients is characterized by the occurrence of only one attack (exacerbation) of the disease, which can occur in the period from 10 months to 10 years. A small part of patients have a cyclical recurrent course of the disease with repeated attacks. The most severe is the chronic form of Still’s disease, which proceeds with severe polyarthritis, leading to limited movement in the joints. Moreover, the earlier appearance of arthritis symptoms is an unfavorable prognostic sign.
Among adult patients with Still’s disease, the five-year survival rate is comparable to that of SLE and is 90-95%. Patients may die from secondary infection, amyloidosis, liver failure, clotting disorders, heart failure, pulmonary tuberculosis, respiratory distress syndrome.