Panarteritis nodosa or polyarteritis nodosa is a rheumatic disease that causes inflammation of the blood vessels. Although the disease is life-threatening without treatment, with treatment, patients can usually achieve freedom from symptoms.
What is panarteritis nodosa?
Panarteritis nodosa, also called classic polyarteritis nodosa, is one of the rheumatic diseases in the vasculitides group. These rheumatic diseases are characterized by inflammation of the blood vessels. In panarteritis nodosa, the medium-sized arteries are mostly affected. In particular, the arteries of the gastrointestinal tract, the nervous system, the musculoskeletal system and the kidneys are affected. All wall layers of the affected arteries can become necrotic and inflamed and occasionally nodular changes occur. There is a risk of vessel bulging, so-called aneurysms, which can lead to the formation of thrombi. Furthermore, panarteritis nodosa leads to a poorer blood supply in the affected areas of the body due to the destruction of the vessels. As a result, panarteritis nodosa can lead to the death of tissue supplied by the affected arteries.
Causes
The causes of panarteritis nodosa disease are not yet fully understood. However, it is thought to be an autoimmune disease that breaks out because of previous infectious diseases. An autoimmune disease is a disease in which the immune system is directed against the body’s own structures and attacks them. Some of the patients suffered from an infection with hepatitis B prior to the disease. It has been shown that these patients can develop deposits of antibodies on the walls of the blood vessels. These deposits trigger immune reactions there that lead to the aforementioned inflammatory processes and their consequences. In addition to hepatitis B, pathogens such as Coxsackie, Epstein-Barr, cytomegalovirus, and a human herpes virus, for example, are suspected of being able to trigger panarteriitis nodosa.
Symptoms, complaints, and signs
Panarteritis nodosa also presents with very nonspecific symptoms in almost all affected individuals. For example, fever, weight loss, night sweats, and muscle and joint pain occur in approximately 95 percent of those affected. The symptoms are thus reminiscent of the flu. Four out of five people affected eventually suffer from neuropathy due to this form of vasculitis. This can be expressed, among other things, in visual disturbances and seizures. There is also an undersupply of blood to the nerves. In many patients, the digestive system is also involved, with severe and intermittent pain being the main symptom. Nausea and vomiting occur. In severe cases, there may also be infarction of the liver, pancreas, or spleen. Infarction of individual segments of the intestine may also occur. In men, testicular pain may occur due to poorer blood flow. In both sexes, angina frequently develops. Panarteritis nodosa also has a negative effect on the mobility of the affected person. Movements of the joints quickly lead to pain. In addition, skin changes may occur. In particular, more or less bizarre skin markings occur, which can be attributed to slow blood flow. The risk of heart attack is increased.
Diagnosis and course
Symptoms in panarteritis nodosa disease can be quite varied. For example, there may be high fever, loss of appetite, weight loss, weakness, and night sweats. Other symptoms depend on the area in which panarteritis nodosa occurs:
Severe abdominal pain suggests disease of an artery in the gastrointestinal tract. If the musculoskeletal system is involved, muscle and joint pain can be expected. In the cardiac region, there is radiating pain to the chest, pressure behind the breastbone, or the sensation of heartburn. If panarteritis nodosa in the area of the heart leads to a blockage of the vessel, a heart attack is imminent. In the brain, panarteritis nodosa can cause headaches, dizziness, vomiting, paralysis, speech disorders or even a stroke. If an artery of the kidneys is affected, there is initially increased blood pressure. Later, renal insufficiency is imminent. Because of the wide range of symptoms, it is not easy to make a diagnosis.An X-ray image of the blood vessels is helpful here, as the changes are visible here. Ultrasound examinations can detect vascular constrictions, and imaging techniques such as MRI, X-ray or CT reveal foci of disease in the body. Suspicion of panarteritis nodosa can ultimately be confirmed by taking and examining tissue samples from the affected areas.
Complications
As a rule, self-healing does not occur in panarteritis nodosa. Since this is a life-threatening disease, treatment by a physician is definitely necessary. If panarteritis nodosa is not treated, in most cases the affected person will die. Patients primarily suffer from severe weight loss in this disease. High fever and fatigue can also occur, significantly reducing the patient’s quality of life. Furthermore, there is also stomach discomfort and intestinal discomfort, so that the affected person also suffers from nausea and vomiting. Likewise, the joints can be painful and swollen, so that it comes to movement restrictions and thus to restrictions in the everyday life of the patient. At night, it is not uncommon for those affected to suffer from sleep problems and night sweats. This can also lead to depression and irritability in the patient. Without treatment, panarteritis nodosa leads to renal insufficiency and thus to the death of the patient. The disease can be treated with the help of medication. Complications do not occur. With proper and early treatment, there is also no decreased life expectancy of the patient.
When should you go to the doctor?
If flu-like symptoms persist, a visit to the doctor is advisable. If the affected person suffers from fever, a general feeling of illness, internal weakness, or an unwanted loss of weight, the symptoms should be clarified. Night sweats, pain or problems with the joints should be presented to a doctor. Complaints of the musculoskeletal system, restrictions of the locomotor system as well as disturbances of the digestive tract indicate a disease that needs to be treated. The affected person needs medical care for functional disorders or a seizure disorder. Without treatment, a life-threatening condition may result. In acute cases, the emergency medical services must be alerted. Relapsing pain is reported by patients as characteristic of panarteritis nodosa. In order to avoid risks, patients should refrain from taking pain medication until they have consulted their physician. Nausea and vomiting as well as irregularities of the skin appearance should also be presented to a physician. If men suffer from pain in the testicles and sexual dysfunction, a comprehensive examination is advisable. Disturbances of the blood circulation or the cardiovascular system are considered worrisome and must be examined by a physician as soon as possible to avoid complications. If there are impairments in coping with everyday life or if participation in usual leisure activities and sports activities must be restricted, a physician is needed.
Treatment and therapy
Treatment of panarteritis nodosa is initially with very high doses of corticosteroids to allow the inflammatory responses to subside. Then, the dose of corticosteroids is slowly reduced until the smallest possible dose that is still effective is found. With this dose, the treatment is continued for a longer period of time. It is also possible to additionally or alternatively give an immunosuppressant to stop the immune system‘s reaction against its own body. To prevent severe damage, treatment should be given as soon as possible. It may also be helpful to have treatment performed at a center specializing in panarteritis nodosa. If an infectious disease is evident as the cause of panarteritis nodosa, this must of course also be treated. With the correct and early treatment, patients can usually achieve complete freedom from symptoms. Without treatment, however, a large proportion of those affected die within five years.
Outlook and prognosis
Whereas polyarteritis nodosa was more frequently fatal 25 years ago, the quality of life and prognosis of those affected has clearly improved in recent years with the proliferation of high-dose glucocorticoid-cytostatic combinations. This is because, while relapses are more frequent in other forms of vasculitis, they are now rare in polyarteritis nodosa. With successful treatment in specialized centers, remission over several years or even complete remission can usually be achieved within the first three years. Therefore, a good prognosis can be assumed, especially since the five-year survival rate is over 80% with appropriate therapy with glucocorticoids and the use of cytostatic drugs. However, since it is an incurable autoimmune disease, only the symptoms, not the causes, are treated. If the digestive organs, kidneys, nerves or brain are already more severely affected or if hepatitis B is also present, however, only a rather poor prognosis can be assumed. Therefore, early diagnosis and combination treatment are all the more important. In untreated disease, however, the five-year survival rate is only 13%. Without therapy, the main causes of death in these cases are myocardial infarction, stroke, or renal failure.
Prevention
Since it is an autoimmune disease, prevention is not possible. However, at least infectious diseases such as hepatitis B should be prevented by vaccination.
Follow-up care
In most cases, the person affected by panarteritis nodosa has very few or even limited options for aftercare, since it is a relatively rare disease. In order to avoid other complications or complaints in the further course of the disease, the affected person should in any case consult a doctor at an early stage. A doctor should be contacted at the first signs or symptoms of the disease. The treatment of the disease is usually carried out with the help of various medications. The affected person should always pay attention to a correct dosage and also to the regular intake of the medication, so that it does not come in the further course to complications or to other complaints. In the event of side effects or uncertainties, a doctor should always be consulted first, and he or she should also be consulted if there are any questions. In many cases, those affected by panarteritis nodosa are also dependent on the help and care of their own family. This mainly alleviates or prevents depression and other psychological upsets. Whether there is a reduced life expectancy of the affected person due to panarteritis nodosa cannot be universally predicted in this context.
Here’s what you can do yourself
In the case of panarteritis nodosa, everyday care and self-help are limited primarily to combating the accompanying symptoms. The most important thing a patient with this condition can do is to take it easy on himself. It is advisable that sufferers get plenty of rest to counteract the symptoms of exhaustion. Despite the recommendation that sufferers take it easy, it seems important to do regular motor exercises. These should be approached at rest, as stress or overwork are counterproductive. In order to keep the muscles in shape despite periods of rest, leisurely walks are particularly suitable. But everyday motor skills should also be trained. This is important so that any effects on the nervous system can be counteracted preventively. In addition, possible effects on the nervous system are noticed more quickly. Furthermore, the diet should be adjusted. Although panarteritis nodosa is associated with weight loss, which can be compensated for by diet if necessary, it is also important to control blood pressure. In the case of high blood pressure, fish and different types of vegetables are particularly suitable, while alcohol should be avoided above all.
