Immune complex vasculitis refers to a form of blood vessel inflammation. It is one of the subgroups of vascular inflammation.
What is immune complex vasculitis?
Immune complex vasculitis is a type of vascular inflammation (vasculitis). In vasculitis, inflammation of the walls of blood vessels occurs. We speak of immune complex vasculitis when the vascular inflammation is caused by an immunological reaction. Immune complexes are deposited on the walls of numerous smaller blood vessels. Physicians then also speak of a leukocytoplastic vasculitis, a vasculitis allergica or a hypersensitivity vasculitis. Doctors distinguish between cutaneous and systemic immune complex vasculitis:
In cutaneous immune complex vasculitis, affected individuals suffer from inflammation of the small blood vessels near the skin. The vasculitis is preceded by a bacterial infection, which is mostly caused by streptococci, or a viral disease. If, on the other hand, the vasculitis is caused by antibodies and takes a chronic course, this can usually be attributed to hepatitis C. Systemic immune complex vasculitis is said to occur when not only the skin is affected by the vascular inflammation, but also the kidneys as well as the complete central nervous system.
Causes
Immune complex vasculitis is caused by the deposition of larger immune complexes, which originate from the blood, on the walls of blood vessels. Usually this occurs due to hypersensitivity of the immune system to drugs or components of germs. Sometimes leukocytoplastic vasculitis also presents as part of systemic immune complex vasculitis. The formation of smaller immune complexes occurs, among other things, when a smaller wound is infected with bacteria. These are bound in the wound by specific antibodies of the organism. The complexes can be easily dissolved in the blood. Their degradation takes place in the liver. However, if larger accumulations of foreign substances and corresponding antibodies meet, this leads to the formation of large immune complexes. This process usually occurs in the context of infections, in which a larger number of germs enters the blood. The most common triggers include throat infections caused by viruses or streptococcus bacteria, and infections with hepatitis B or hepatitis C viruses. Antibodies to medications such as certain antibiotics, painkillers, the birth control pill, or diuretics are also produced by some individuals. If large quantities of germs or drugs against which antibodies already exist enter the blood, this results in the formation of huge immune complexes. Since these are difficult to dissolve in the blood, they are deposited as solid substances on the walls of the blood vessels. This is especially true for blood vessels where there is slow blood flow, such as very small veins. Once the immune complexes have deposited on the vessel walls, this leads to a migration of leukocytes (white blood cells) from the blood into the vessel walls. This in turn triggers an inflammatory response. The leukocytes attempt to break down the immune complexes by releasing certain substances, but this is only partially successful. Inadvertently, so to speak, the aggressive ingredients can penetrate into neighboring tissues, resulting in further damage to the blood vessel walls.
Symptoms, complaints, and signs
Typical symptoms of immune complex vasculitis include many small hemorrhages that are visible on the skin. At first, they appear on the lower legs in the form of red dots that can reach a maximum size of three millimeters. After a few days, the dots turn blue-red. In addition, further red hemorrhages appear in the lower and upper leg region. However, itching or burning occurs only occasionally. In case of a stronger inflammatory reaction, the dark red nodules reach a diameter of several millimeters. If the immune complex vasculitis is severe, the uppermost skin layer in the center of the nodules dies off, which is noticeable by a light gray discoloration or blistering. Furthermore, superficial skin defects form, which weep. In the worst case, tissue necrosis is also possible, which is manifested by soluble black nodules and painful small wounds.
Diagnosis and course of the disease
During the examination, the doctor will ask the patient whether he suffered from an infection some time ago or is taking any new medications. He also wants to know if the individual is suffering from any underlying chronic diseases. Finally, the patient’s skin is checked. The physician pays particular attention to red spots. To confirm the diagnosis, two tissue samples (biopsies) are taken. The first sample is microscopically checked in a laboratory for vascular inflammation. In the second sample, immunofluorescence can be used to detect immune complexes deposited on the walls of the vessels. If immune complex vasculitis is not treated or if there is involvement of the central nervous system, the disease usually takes a negative course.
Complications
Immune complex vasculitis causes hemorrhages in the skin. These hemorrhages become visible to the affected person as small dots and may become discolored and larger as the disease progresses. In most cases, there is also severe itching and burning pain in the affected regions. Not infrequently, the pain also occurs in the form of pain at rest and can thus lead to considerable difficulty sleeping and to irritability on the part of the patient. In severe cases, the upper layers of skin in the respective regions may also die off completely, which can lead primarily to the formation of scars and blisters. This manifests itself in decreased aesthetics and, consequently, in decreased self-esteem. In the treatment of immune complex vasculitis, there are no particular complications in most cases. First and foremost, the underlying disease responsible for the immune complex vasculitis is always treated. If inflammation has already occurred, antibiotics can be taken to counteract it. Life expectancy is not reduced by the disease and no further symptoms occur after treatment.
When should you go to the doctor?
Anyone who suddenly notices bleeding on the skin should see a doctor. The skin changes indicate a skin disease that needs to be clarified and, if necessary, treated. Whether this is immune complex vasculitis can only be determined by a doctor. Therefore, medical advice should be sought at the first signs of disease. Clear warning signs, which should be clarified immediately if possible, are blue-red discolorations on the skin, often associated with itching or burning. A visit to the doctor is indicated at the latest when the inflammations become larger or the skin areas die off. The disease often occurs after an infection with hepatitis B or hepatitis C viruses. Taking painkillers or antibiotics can also lead to immune complex vasculitis. Anyone who is affected by these factors should have the symptoms clarified quickly. In addition to the family doctor, a dermatologist or an immunologist can be consulted. Patients with chronic symptoms should consult a therapist, as the disease often has an impact on the psyche.
Treatment and therapy
Treatment of immune complex vasculitis depends on the triggering causes. If drugs or a pathogen are responsible for the hypersensitivity, it is important to eliminate the trigger. As a rule, the inflammatory symptoms soon disappear. If bacteria are involved in the vascular inflammation, the administration of antibiotics may be useful. If the cause is a specific medication, it is discontinued or exchanged for another preparation. Wearing special compression stockings is considered helpful. In this way, the blood flows faster in the small vessels, which prevents new immune complexes from being deposited on the vessel walls. In most cases, cortisone therapy is also given.
Outlook and prognosis
Immune complex vasculitis requires comprehensive treatment. If nonbacterial inflammation of the blood vessels is not treated, the prognosis is unfavorable. The various gastrointestinal and neurologic symptoms cause numerous complications, such as epilepsies or intestinal infarcts, which can be fatal. If the heart is involved, angina pectoris may result, leading to myocardial infarction. The patients’ quality of life is severely restricted by the condition and they have to take a wide variety of medications.Nevertheless, well-being is reduced, which can also result in psychological complaints in the long term. Even comprehensive treatment does not guarantee recovery. If the central nervous system is involved, a fatal course is likely. Those affected generally have a reduced life expectancy. Factors such as early treatment and, except for immune complex vasculitis, good physical condition improve the prospects for recovery. With comprehensive medical therapy, the condition can be treated causally in a period of three months to several years. Accompanying symptomatic therapy is necessary. The prognosis is worse in children, the elderly and patients with immune deficiency. In most cases, these patients die from typical endothelial swelling or other complications in the first weeks to months after the onset of immune complex vasculitis.
Prevention
Preventive measures against immune complex vasculitis are not known. Therefore, it is all the more important to start therapy quickly if the disease occurs.
Follow-up
Affected individuals do not have any special options for aftercare in most cases of immune complex vasculitis, so the first priority in this disease should be to obtain a rapid diagnosis. With an early diagnosis and also an early initiation of treatment, there is usually a positive course of the disease. Self-cure cannot occur with this disease. As a rule, those affected by immune complex vasculitis must discontinue certain medications or replace them with others. However, a doctor should always be consulted. When taking medication, it is always important to pay attention to the correct dosage and also to take it regularly in order to alleviate the symptoms properly and permanently. If there are any questions or uncertainties, it is advisable to consult a doctor first. Often, wearing compression stockings can also have a positive effect on the course of immune complex vasculitis. Due to the disease, many patients also need the help of their own family or friends in their daily lives. Loving conversations with those affected often have a positive effect on the course of the disease and can sometimes prevent the emergence of depression or other psychological complaints. Possibly, the disease reduces the life expectancy of the affected person.
What you can do yourself
Unfortunately, immune complex vasculitis cannot be prevented directly. When the disease occurs, a doctor should always be consulted immediately for this reason. The earlier the disease is treated, the higher the chances of a positive course of the disease. Unfortunately, the possibilities for self-help are also severely limited in the case of this disease. If the doctor prescribes antibiotics to the patient, they should be taken. When taking antibiotics, the corresponding instructions must be followed. The intake of other medications should also be checked. Furthermore, the symptoms of immune complex vasculitis can be limited by wearing compression stockings. These can usually be prescribed by a physician. Therapy with cortisone also has a positive effect on the course of the disease. The affected person should pay attention in self-experimentation to whether certain medications intensify the symptoms of immune complex vasculitis. If this is the case, the drugs can possibly be discontinued or replaced by other drugs after consultation with the attending physician. Skin discomfort or scars can also be limited and avoided with the help of caring ointments or creams. However, in severe cases, surgical intervention is suitable to avoid aesthetic discomfort.
