Generality
Still 's disease is an inflammatory disease, chronic in nature, very similar to rheumatoid arthritis.
Given that the symptoms resemble those of many other diseases, making a correct and early diagnosis is not simple: before pronouncing, in fact, the doctor must carefully evaluate numerous parameters.
Still's disease is currently incurable, but there are pharmacological treatments that manage to control its symptoms and slow its progression. If the patient follows the doctor's advice, his standard of living is only moderately compromised by the disease.
What is Still's disease
Still's disease - whose full name is Still's disease of adult onset - is a systemic inflammatory disease, chronic in character, which is mainly characterized by high fever, salmon-colored skin rash, sore throat and joint pain.
Due to its resemblance to rheumatoid arthritis, Still's disease is also considered a particular form of arthritis .
NB: for systemic inflammatory disease, it means a pathology characterized by an inflammatory state that affects several parts of the body (one or more systems).
Some diseases similar to Still's disease:
- Rheumatoid arthritis
- Systemic lupus erythematosus
- Crohn's disease
- Lyme disease
YOUTH FORM
Next to the adult form, there is also a juvenile form of Still's disease, characterized by slightly different symptoms and known more as juvenile rheumatoid arthritis (or systemic arthritis), than as juvenile Still's disease.
Epidemiology
Still's disease is an uncommon pathology: in fact, every year, to get sick of it is one person every 100, 000 or so.
According to some statistical surveys, there are two age groups most affected by the disease: one is between the ages of 15 and 25, the other between 36 and 46 years.
There seems to be a marked preference for female individuals, but the reason for this remains to be explained.
It cannot be ruled out that Still's disease is a hereditary disorder, given the small number of times more than one case has been recorded in the same family.
Causes
The causes, which cause Still's disease, are unknown.
According to some theories, it seems that to trigger the disease is some kind of infection, viral or bacterial.
However, there is no evidence on this.
Symptoms and Complications
Still's disease manifests itself in different ways from patient to patient: some face a drastic deterioration; others improve suddenly and without a specific reason; still others alternate periods with symptoms to periods without any pathological disorder.
TYPICAL SYMPTOMS
The most common and characteristic symptoms and signs of Still's disease are:- Sore throat . It is usually the first symptom that appears in the affected individual. The feeling is of a strong burning pain. Furthermore, the appearance of this disorder coincides with the enlargement of the lymph nodes of the neck.
- High fever . It is not constant throughout the day, but rises suddenly, up to 38.5-39 ° C, in the late afternoon or evening; in some cases, two daily rises can also be recorded, separated by a period of time in which the temperature returns to normal. The duration of this disorder is generally one week, but it can last even longer, depending on how the body responds.
- Salmon-colored skin rash . The skin becomes - suddenly and coinciding with the fever peaks - salmon pink. The rash occurs with flat and / or raised spots, usually appears at the level of the trunk, arms and legs, and does not cause any itching. Rubbing of the skin, even involuntary, can trigger the appearance of the rash.
- Pain, swelling and stiffness in the joints . At the beginning, the joints involved are only the knees and wrists; over time, pain and swelling extend to the hips, elbows, hands and shoulders. Especially in the morning, the sense of rigidity is very strong and lasts for several hours. Joint disorders last for at least a couple of weeks.
- Muscle pain . It alternates with fever, or when there is one, there is no other. The painful sensation is so intense that it makes any daily activity difficult, if not impossible.
Less common signs of Still's disease:
- Unexplained weight loss
- General enlarged lymph nodes
- Abdominal pain and swelling
- Respiratory and cough problems
- Enlarged spleen (splenomegaly)
WHEN TO REFER TO THE DOCTOR?
If without a plausible reason you experience symptoms such as high fever associated with a salmon pink rash, joint pain, sore throat and cough, then it is advisable to consult your doctor, for further examinations.
COMPLICATIONS
The complications, linked to Still's disease, arise when the inflammatory state is not treated properly or when the diagnosis is late: in these situations, to pay the worst consequences are the joints or some organs of the body, such as the heart and lungs.
- Irreparable damage to the joints . Prolonged inflammation can permanently damage the joints. Those most at risk are the first that tend to show joint disorders, ie knees and wrists. It is infrequent, but possible, that the joints of the ankles, shoulders and fingers also deteriorate.
- Inflammation of the heart . The generalized inflammatory state can extend to the heart, causing a pericarditis (ie an inflammation of the membrane, the pericardium, which surrounds and protects the heart) or a myocarditis (ie an inflammation of the heart muscle). The main symptoms are chest pain, shortness of breath, dry cough and swelling in the abdomen.
- Pleural effusion . Due to an inflammation of the pleura ( pleuritis ), it is the accumulation of fluid inside the pleural cavity, ie the space between the pleura and the lung.
Diagnosis
It is difficult to diagnose Still's disease, as the symptoms it causes are very similar to those of other diseases and infections, such as some cancers, Crohn's disease or Lyme disease .
To detect the disorder and to exclude similar diseases, various radiological and blood tests are available to the doctor.
EXAMINATION OBJECTIVE
The physical examination represents, as often happens, the first step of the diagnosis path.
The doctor evaluates the patient's state of health, analyzes the symptoms and investigates their medical history . Although, in fact, the causes of Still's disease are still unknown, we know what can cause diseases with similar symptoms, such as Crohn's disease or Lyme disease.
BLOOD TESTS
The collection and analysis of a blood sample can provide useful diagnostic information: in fact, if Still's disease is in progress, blood shows certain characteristics, which, conversely, would not have if it were another disease from similar symptoms.
Here is what peculiarities the blood sample must have:
- High white blood cell count (leukocytes)
- Low number of red blood cells (erythrocytes)
- High levels of C-reactive protein (PCR)
- High erythrocyte sedimentation rate (ESR)
- Control of anti-nucleus antibodies is negative (this, for example, is positive in systemic lupus erythematosus)
- Negative control of rheumatoid factor
- High levels of transaminases
- High levels of ferritin and fibrinogen
RADIOLOGICAL TESTS
The useful radiological examinations, in case of suspected Still's disease, are:
- Ultrasound and CT ( computerized axial tomography ) of the abdomen : show the appearance of the internal organs, contained in the abdominal cavity. Any abnormalities, such as splenomegaly, are detected by these tests.
- Nuclear magnetic resonance ( NMR ) to the most painful joints : it clarifies the origin of joint pain and what degree has reached the associated damage. The injury to the joints caused by a chronic inflammatory state is recognizable to an experienced eye.
- Chest X-ray : provides clear images of the heart and lungs. If present, pericarditis, myocarditis and pleurisy are easily seen through this control.
IMPORTANCE OF EARLY DIAGNOSIS
Some individuals suffering from Still's disease, if they neglect the ailments or if they do not treat themselves properly, may face the aforementioned complications. Conversely, early diagnosis and adequate treatment greatly reduce the risk of these unpleasant consequences.
Treatment
Currently, there is still no cure that completely heals Still's disease. The only countermeasures available are drugs of various types, which serve the following purposes:
- Limit the symptoms
- Slow down the progression of the disease
- Prevent complications
- Improve patients' quality of life
The choice of the most appropriate medicines is up to the doctor, who considers the seriousness with which the disease presents itself, at what time the diagnosis (early or late) was made and how the patient responded to previous treatments.
In general, for chronic inflammatory diseases such as Still's disease, drug treatments are assumed by the patient throughout life: in fact, although they may be modified in the dose or temporarily suspended, they are never definitively interrupted.
NON STEROID IDEAS (NSAID)
NSAIDs, or non-steroidal anti-inflammatory drugs, are usually the first medications prescribed by a doctor when Still's disease is diagnosed.
Their purpose, as the name suggests, is to reduce the generalized inflammatory state, when it is still moderate and does not create particular disorders.
The most used NSAIDs, in these cases, are ibuprofen, naproxen and aspirin (at high doses).
The duration of treatment is not quantifiable, except after having observed how the patient responds to treatment: if the response is positive, the doses taken may gradually decrease or suspend for a few days; vice versa, if the benefits are few, it is necessary to continue with the therapy, if not even replace it with something stronger.
The side effects, due to the use of NSAIDs, affect the liver and its functions. In this regard, the medical advice to be followed as prevention of liver disorders is to undergo periodic blood tests.
CORTICOSTEROIDS
Corticosteroids are the most powerful and best-performing anti-inflammatory drugs.
However, prolonged use can cause unpleasant and, in some cases, serious side effects, such as high blood pressure and diabetes mellitus.
Main side effects of corticosteroids:
- Arterial hypertension and water retention
- Hyperglycemia, insulin resistance and diabetes mellitus
- Osteoporosis
- Increased body weight, due to the increase in fat mass
- Swelling and facial tension
- Susceptibility to infections
- Susceptibility to injuries (reduced tendon and ligament resistance)
- Psychosis
- Morning insomnia
This explains why, before taking them, NSAIDs are administered, and why, once they are taken, they are kept at the minimum effective therapeutic dose.
One of the most used corticosteroids, in patients with Still's disease, is definitely prednisone .
IMMUNOSUPPRESSANTS
Immunosuppressive drugs are another possible therapeutic solution for those suffering from Still's disease.
Their function is to weaken the immune defenses . Normally, these defensive barriers serve to counter external insults, bacterial and viral infections etc; however, after the appearance of certain pathological disorders (including Still's disease), they can "turn over" against the body and become responsible for a generalized inflammatory state.
Immunosuppressive medicines can be taken in combination with others of the same type and for long periods of time.
There are two possible routes of administration: by intravenous injection or by mouth.
Immunosuppressive drugs used against Still's disease:
- Methotrexate
- Idrossiclorichina
- sulfasalazine
- azathioprine
- Cyclophosphamide
- Cyclosporine
- anakinra
- TNF inhibitors (adalimumab, etanercept and infliximab)
For further information: Medications for the Treatment of Still's Disease "
REST AND WARNINGS
During the acute phase of the disease (ie when pathological disorders appear), it is good to observe a period of rest, in which heavy work or situations that are particularly stressful for the physique should be avoided.
Furthermore, the possible cyclical course of Still's disease (cyclically means that it alternates periods with symptoms to periods of remission in which the disorders disappear or become very mild) could lead the patient to think he is cured and to be able to interrupt the treatments . However, there is no more wrong choice than this, as drug intake should be continued even in moments of apparent healing.
Any decision to temporarily interrupt or change the treatment rests solely with the doctor, after appropriate clinical evaluation.
Prognosis
For incurable diseases, such as Still's disease or rheumatoid arthritis, the prognosis can never be positive. In fact, if it is true that they do not cause death, it is also true that they greatly influence the standard of living of the sick: these, in fact, are constantly under treatment (except for temporary suspension of drugs) and suffering from chronic disorders, due to the pathology.
For a better prognosis, it is good to follow the following medical advice:
- Always consult your doctor before making any decisions about which drugs to take. This behavior should be adopted in any situation, that is both when the symptoms get worse and when they suddenly improve.
- Adapt your diet to corticosteroid medications, taking dietary supplements based on calcium and vitamin D. These serve to prevent or, at least, limit osteoporosis.
- Observe a period of rest during the acute phase of the symptoms, but then it is good to remain active and in motion to remedy the joint stiffness typical of Still's disease.
