In microscopic polyangiitis, blood vessels become inflamed by autoimmunologic processes. The cause is not yet known but is speculatively associated with immunologic misprogramming after infection. The prognosis for untreated patients is unfavorable, but drug-treated patients do not necessarily have an unfavorable course.
What is microscopic polyangiitis?
Microscopic polyangitis corresponds to polyarteritis nodosa, also known as Kussmaul disease. The condition is classified under the vasculitis group of diseases and thus corresponds to inflammation of the blood vessels that is autoimmunologically caused. The manifestation is classified as an ANCA-negative vasculitis of the medium-sized vessels. Inflammation of the smaller and medium-sized arteries with a pearly arrangement of their inflammatory nodules mainly affects the calves, forearms, and internal organs. The inflammations are triggered by misrecognition of the body’s immune system. Microscopic polyangiitis is an extremely rare disease. Among one million people, the prevalence is estimated at about three new cases per year. Men develop the disease somewhat more frequently than the female sex. In many places, there is even talk of a prevalence in men that is about twice as high. The disease usually manifests in middle age.
Causes
Microscopic polyangiitis is an autoimmune disease. The body’s immune system produces antibodies against body tissues, causing inflammation. To date, the causes of autoimmune diseases are not well understood. Speculatively, there is some talk of misprogramming of the immune system in the context of previous infections. The causes of primary microscopic polyangiitis have also not been definitively determined. In about one third of all cases, however, the disease is associated with chronic hepatitis C of genotype 2 or hepatitis B. In these cases, polyangiitis is considered to be the result of a malfunctioning immune system. In these cases, polyangiitis is considered secondary and merely a symptom of hepatitis, presumably caused by an infection-related overreaction of the immune system. Infections may also play a role in the primary ideopathic form of the disease. However, this connection is not confirmed. The only certainty so far is that the immunological inflammations result in symptomatic necrosis of the vessel walls and are associated with the infiltration of neutrophil granulocytes. Necrosis leads to vasoconstriction with thrombosis or infarction of end-stream pathways.
Symptoms, complaints, and signs
Patients of microscopic polyangiitis suffer from nonspecific symptoms such as fever, muscle pain, joint discomfort, and night sweats or weight loss. About 80 percent of all sufferers are affected by neuropathy, which primarily affects mixed nerves above a certain size. Sometimes the central nervous system is involved. In such a case, visual disturbances, secondary symptoms such as strokes or epileptic seizures occur. Since there is often symptomatic hypertension, these symptoms are considered secondary consequences thereof. In the digestive tract, the disease may manifest as colicky abdominal pain or intestinal infarcts and bleeding. In the kidneys, microaneurysms of the vessels can be observed. In addition, cardiac symptoms such as angina pectoris or myocardial infarction may be present. Polyangiitis may also manifest on the skin, most notably in livedo racemosa or subcutaneous aneurysms. Laboratory analysis reveals elevated CRP and erythrocyte sedimentation rate. Leukocytosis develops.
Diagnosis and course of disease
The diagnosis of microscopic polyangiitis is made according to the ACR criteria and requires at least three of a total of ten characteristic criteria. In addition to a weight loss of more than four kilograms, characteristic criteria include livedo reticularis, testicular pain, muscle weakness and muscle pain, and mono- or polyneuropathies, hypertension, and an elevation of serum creatinine or urea. The same applies to detectable hepatitis B or hepatitis C, angiographic abnormalities, or matching findings from arterial biopsy. Differentially, microscopic polyangiitis should be distinguished from other vasculitides and systemic lupus erythematosus.Untreated, the prognosis is extremely unfavorable. The five-year survival rate is less than 20 percent in untreated patients. Most often, death occurs from a heart attack or stroke. For treated patients, the five-year survival rate increases to 80 percent.
Complications
Various symptoms usually occur with this disease, but in each case they significantly reduce the quality of life of those affected. The patients suffer thereby above all from fever and furthermore also from pain at the joints or at the muscles. It is also not uncommon for sweating to occur at night, which can disturb sleep. This often results in depression or irritability of the affected person. Furthermore, patients also suffer from paralysis or various disorders of sensitivity that can occur over the entire body. Visual disturbances or hearing difficulties can also restrict and complicate the patient’s daily life. Furthermore, abdominal pain or epileptic seizures occur. These are usually associated with severe pain. In the worst case, patients suffer a heart attack and die from it. In general, the life expectancy of the affected person is significantly reduced by the disease. It is also not uncommon for pain to occur in the testicles. The treatment of this disease is carried out with the help of medications. As a rule, the course of the disease is positive. Particular complications do not limit the treatment in this case.
When should you go to the doctor?
People suffering from symptoms such as fever, a general feeling of malaise, pain in the muscles or impairment of the joints should see a doctor. Unwanted weight loss, night sweats, and sleep disturbances should be investigated and treated. If there are disturbances in vision, an increased risk of accidents, and an increased risk of injury, a doctor is needed. If there is abdominal pain, irregularity of the digestive tract, or cramping in the upper abdomen, a doctor’s visit is required. Colic is a special warning sign of the organism. They should be clarified by a doctor as soon as possible. In individual cases, an emergency doctor should be alerted so that emergency medical care can be provided immediately. Disturbances of cardiac activity as well as cardiac rhythm are a cause for concern. Since in severe cases a heart attack is imminent, a visit to the doctor should be made. Heart palpitations, increased blood pressure, hot flashes or inner restlessness should be clarified by a doctor. If there is a feeling of pressure in the chest, a strong feeling of illness or heaviness, a doctor should be consulted. If an existing pain sensation spreads over the chest further into the back or jaw, consultation with a physician is necessary. If there are abnormalities in the appearance of the skin, a visit to the doctor is also advisable.
Treatment and therapy
Polyangiitis is an incurable disease. Since the cause is not known in detail, there can be no causal therapy, only symptomatic therapy. In most cases, symptomatic therapy corresponds primarily to conservative drug treatment with corticosteroids or cyclophosphamide and azathioprine. If hepatitis is present, treatment of this infection may interactively improve the symptoms of polyangiitis. The success of treatment always depends on the severity of polyangiitis in each individual case. In most cases, patients are assigned to a specific severity level using a five-factor score. The mild form corresponds to a score of zero. At this grade, glucocorticoids improve symptoms in half of the cases. However, almost half of the patients with remission suffer a relapse. Such a relapse must be followed by administration of an immunosuppressant. Patients in the more severe grades also receive immunosuppressants such as cyclopshosphamide and azathioprine. Glucocorticoids in high-dose therapy lasting several weeks are also a common treatment step for patients with more severe forms. To reduce symptomatic hypertension and thus mortality from myocardial infarction or stroke, ACE inhibitors are administered.
Outlook and prognosis
Approximately four in one million people develop microscopic polyangiitis each year. One of the reasons for this low number is that the state of scientific research to date has been inadequate. But the fact is that it is an incurable disease.Therapy focuses exclusively on the symptoms and not on the causes. Doctors assign patients to a score when they are diagnosed. A lower score allows a more favorable prognosis than a higher severity score. Basically, quality of life and expectancy have improved in recent years. One of the problems with microscopic polyangiitis is that neoplasms develop. After two years from treatment admission, these are found in a good third of patients. These recurrences can only be detected early by close monitoring. On the other hand, most patients can remain symptom-free for years with suitable therapeutic approaches. Five years after diagnosis, an average of 80 percent of all treated patients are still alive. If, on the other hand, treatment is not undertaken, the risk of death increases significantly. Only one in five is still alive five years after the onset of microscopic polyangiitis.
Prevention
Microscopic polyangiitis cannot yet be prevented because the definitive causes remain unclear.
Follow-up care
Because the treatment of microscopic polyangiitis is complex and lengthy, follow-up care focuses on managing the disease with confidence. Affected individuals should try to build a positive attitude despite the adversity. In doing so, relaxation exercises and meditation can help calm and focus the mind. Microscopic polyangiitis is a very rare disease, which can bring significant complications. Follow-up care is highly dependent on the individual severity of the disease, so symptoms can only be alleviated, but not permanently controlled. A healthy lifestyle with sufficient sleep and exercise in the fresh air strengthen the immune system and can increase well-being. Acute symptoms that occur repeatedly and suddenly should be clarified immediately with the attending physician.
What you can do yourself
Microscopic polyangiitis has no cure to date. Treatment focuses on relieving symptoms and improving the sufferer’s quality of life. The sufferer can support these measures by adapting his or her lifestyle to the condition. This can be achieved, for example, by changing the diet. In principle, a healthy and balanced diet with sufficient carbohydrates is recommended for microscopic polyangiitis. In addition, stimulants such as alcohol, caffeine and nicotine should be avoided. Affected individuals should consume sufficient fluids to counteract bleeding, high blood pressure and other typical symptoms of the disease. Medicinal therapy is also indicated. This can be supported by the patient by keeping a diary of any side effects and interactions. With the help of these notes, the responsible physician can optimally adjust the medication to the individual symptoms and constitution of the patient. If visual disturbances or epileptic seizures occur, a specialist must be consulted. In addition, the patient needs support in everyday life, because the often acute symptoms increase the risk of accidents and falls. In cases of severe polyangiitis, a psychotherapist may also be called in under certain circumstances.
