It is a rare disease. Its essence is to damage the walls of blood vessels by inflammation developing in the body.
It is only 5 percent. all forms of vasculitis, but it has serious consequences. Men are twice as likely to suffer from it than women. The disease can develop at any age, but the so-called the peak incidence is between the ages of 40 and 60. In polyarteritis nodosa, medium and small arteries are damaged. The arteries and capillaries remain intact. The disease is also insidious in that it can affect individual organs, such as the skin, kidneys, gastrointestinal tract, muscles, joints, the central nervous system, peripheral nerves, heart, testes and epididymis.
How does it start?
Vasculitis occurs when white blood cells, or leukocytes, attack and damage the blood vessel wall. The consequence of this process is bleeding or ischemia of our body tissues.
The disease can progress rapidly and affect many organs at once, putting the patient’s life at risk. As a rule, however, symptoms build up slowly (from several weeks to several months) and are easily confused with other ailments. There is pain or muscle weakness, weight loss, fatigue, high temperature and lack of appetite. When the disease likes one of the organs, the symptoms are more pronounced.
On the skin – there is elevated purpura, ulcerations (around the fingers, ankles and on the front of the lower leg), lumps under the skin and a blue skin.
In the ponds – pain in the joints of the knees, elbows, wrists and ankles, accompanied by swelling and redness of the skin above the joint, are characteristic.
The nervous system – patients experience severe headaches, high mood swings, sometimes wrist or foot dropping without the possibility of keeping the hand or foot in a certain position.
Kidneys – when the disease affects the kidneys, arterial hypertension occurs, sometimes the urine turns brown-red, and rarely there may be a sudden, sharp pain in the lumbar region.
Digestive tract – there are recurrent, quite severe abdominal pain, nausea and vomiting.
Heart – an attack of the heart is manifested by fatigue and chest pain.
None of the symptoms should be taken lightly, because although the disease is rare, it is life-threatening.
Only suspicions
In nodular arteritis, characteristic immune complexes build up in the walls of blood vessels in different parts of the body. Under the influence of various stimuli, white cells enter these complexes and provoke the inflammatory process, and under the influence of substances released by leukocytes, the vascular wall of the organ is damaged.
The causes of the disease have not been fully understood yet, but polyarteritis nodosa is considered an autoimmune disease, that is, provoked by the host’s immune system. Researchers suspect that genes, hepatitis B and C infection, and certain bacterial infections such as streptococcal infections may be causing the trouble.
The sooner the better
Since polyarteritis nodosa is a disease that occurs rarely and often has non-specific symptoms, you should consult your doctor when you notice the first disturbing changes. He or she will probably order tests that will rule out other, more common diseases, and, if necessary, will refer the patient to a specialist.
The tests recommended usually include a blood test with ESR. and CRP, the so-called liver function tests; and urine tests for protein and blood. If the disease affects the skin or nerves, a fragment is taken for analysis.
If the doctor suspects polyarteritis nodosa, he or she may refer the patient to a hospital for visceral arteriography or an internal organ biopsy.
Visceral arteriography is performed to check for lesions in the vessels that supply blood to the kidneys and intestines.
A contrast agent is administered through a catheter placed in the femoral artery. A series of X-ray images are then taken to show the contrast flowing through the vessels. The day before the test, you need to drink plenty of water to protect your kidneys from contrast damage. The test is performed on an empty stomach. After the examination, you also need to drink a lot to rinse the contrast out of the body as quickly as possible. One day after the examination, it is necessary to lie down, because immediately after the examination, a compression dressing is applied to the femoral artery, which prevents the artery from bleeding. The contraindication to perform the test is allergy to iodine, severe arterial hypertension and chronic renal failure.
A biopsy, i.e. taking a piece of tissue for examination, in the case of cutaneous form of nodular arteritis, may be performed on an outpatient basis. Internal organ biopsy must be performed in a hospital, as complications may arise.
Silence the immune system
In the treatment of polyarteritis nodosa, the idea is to silence the immune system so that it does not provoke or aggravate inflammation, and therefore does not damage subsequent blood vessels. After diagnosis, the patient must take intravenous glucocorticosteroids in combination with cyclophosphamide. Once the immune system is under control, the patient can take oral medications, which is more convenient for him. Basic therapy lasts about a year. In chronic polyarteritis nodosa, cyclophosphamide is replaced by other immunosuppressants, e.g. methotrexate.
With more serious infections, patients must take antibiotics. If, on diagnosis, polyarteritis nodosa developed after hepatitis B virus (HBV) infection, an antiviral drug must also be included in therapy.
Cutaneous forms of nodular arteritis are treated with oral glucocorticosteroids.
In any case of treatment of the disease, if the infection has not yet developed with HBV, it is necessary to perform preventive vaccinations to eliminate the additional risk. The need to treat ailments that may accompany nodular arteritis, e.g. hypertension, should not be forgotten either. Controlling additional diseases allows you to save and maintain in good condition the strategic organs of the body, such as the kidneys or the heart, for longer.
Unfortunately, we do not know how to prevent this disease. Vaccination against hepatitis B can only be recommended. We know that after the introduction of the vaccine, in areas where there were many cases of polyarteritis nodosa, the most likely cause of which was HBV infection, the number of cases decreased.
Some patients find that treating polyarteritis nodosa is cumbersome. Even if this is the subjective opinion of patients, it is worth taking the trouble of therapy, because if left untreated, the disease leads to death. When properly treated, it usually enters a state of complete remission, i.e. complete relief of symptoms. This condition may persist for many years, which gives you a chance for a long and full-fledged life.
Text: Anna Jarosz