What is a vasculitis?
Vasculitis is an inflammation of the blood vessel walls. This inflammatory process can occur without known cause (primary vasculitis), autoimmune reactions, as a result of an infectious process or in association with another pathological condition (secondary vasculitis).
Causes
In the vasculitis the participation of the immune system is evident: this becomes "hyperactive" and erroneously recognizes the blood vessel cells as foreign and dangerous, then attacks them as if they were a bacterium or a virus. It is not always clear why this happens, but an infection, certain types of cancer and immune system disorders, certain drugs or an allergic reaction often act as a trigger.
The inflammatory process causes changes in the walls of blood vessels. Vasculitis constricts the lumen of vessels, reducing the amount of blood reaching the tissues and organs. In some cases, thrombi or granulomas may form in an affected vessel, which obstruct the blood flow. Other times, instead of reducing the caliber, vasculitis weakens the blood vessel, causing the onset of an aneurysm.
Vasculitis with no known cause (primary vasculitis)
For many forms of vasculitis, the cause is not known.
Secondary vasculitis
Several diseases are associated with the onset of vasculitis.
These conditions include:
- Infections. Some vasculitis occur in response to an infection. For example, many cases of cryoglobulinemic vasculitis are the result of hepatitis C virus infection, while hepatitis B can result in the clinical presentation of polyarteritis nodosa.
- Autoimmune diseases . Vasculitis can also occur as a result of certain diseases of the immune system, such as rheumatoid arthritis, systemic lupus erythematosus, juvenile dermatomyositis and scleroderma.
- Allergic reactions. Exposure to chemicals (insecticides and petroleum products) and drugs such as amphetamines, sulfonamides, beta-lactams, oral contraceptives, NSAIDs, quinolones and some vaccines can cause vasculitis.
- Blood cell tumors . A lymphoproliferative or myeloproliferative neoplasm can cause vasculitis.
Classification
There are different forms of vasculitis and each of these is characterized by a specific clinical picture.
Vasculitis can be classified based on:
- Underlying cause;
- Location of affected vessels:
- Cerebral vasculitis;
- Cutaneous vasculitis;
- Systemic vasculitis.
- Type or size of the affected blood vessels:
- Vasculitis of large blood vessels (such as the aorta and its branches) : polymyalgia rheumatica, Takayasu arteritis and temporal arteritis;
- Vasculitis of medium-sized vessels (arteries and veins) : Buerger's disease, cutaneous vasculitis, Kawasaki syndrome and polyarteritis nodosa;
- Vasculitis of small vessels (capillaries, arterioles, and venules) : Churg-Strauss syndrome, leukocytoclastic vasculitis, essential mixed cryoglobulinemia, Schönlein-Henoch purpura, microscopic polyangioitis and Wegener's granulomatosis.
Vasculitis includes arteritis, inflammation that mainly affects the arteries, such as Takayasu's arteritis, polyarteritis nodosa and temporal arteritis. Some forms, on the other hand, affect arteries and veins of various sizes (Behçet's disease).
Symptoms
Signs and symptoms of vasculitis vary depending on which blood vessels (hence which organs or systems) are involved. The severity of the disease also depends on the form in which it occurs (from mild to disabling). However, there are general signs and symptoms common to most vasculitis, which include:
- Skin eruptions;
- Weakness;
- Temperature;
- Joint and muscle pains;
- Loss of appetite and weight.
Although aspecific manifestations may be present for some time, such as arthralgia and lethargy, the first evident sign of vasculitis is often a skin lesion (purpura, nodules, ulcers, canker sores, etc.).
Presentation based on the caliber of the affected vessels
If a small blood vessel is affected, it can break and produce slight infiltration of blood into the tissue, which appear as small red and purplish spots on the skin. If instead a larger vessel ignites, it can swell and produce a nodule, palpable when it is located near the surface of the skin.
Vasculitis | Small vases | Medium-sized vessels | Large blood vessels |
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Signs and symptoms of specific organs and systems
Additional symptoms may occur depending on the area of the body affected by the vasculitis:
- Heart: myocardial infarction, hypertension and gangrene;
- Joints: arthritis;
- Kidneys: dark urine or hematuria and glomerulonephritis;
- Skin: nodules, ulcers, bruises or hives, purpura and reticular livedo;
- Lungs: shortness of breath and hemoptysis (cough with blood);
- Eyes: redness, itching and burning, photosensitivity, reduced visual acuity and blindness;
- Gastrointestinal tract: mouth ulcers and ulcers, abdominal pain and perforation of the intestine;
- Nose, throat and ears: sinusitis, ulcers, tinnitus and hearing loss;
- Nerves: numbness, tingling, weakness in various parts of the body, loss of sensation or strength in hands and feet, and pain in arms and legs;
- Brain: headache, stroke, muscle weakness and paralysis (inability to move).
Signs and symptoms of some types of vasculitis
- Behçet syndrome: it can cause recurrent and painful oral ulcers, ulcers on the genitals, skin lesions similar to acne and ocular inflammation (uveitis);
- Temporal arteritis : usually affects an artery on the side of the head. Symptoms may include headache, pain in the jaw and scalp, with blurred or double vision up to sudden blindness. This condition is also called giant cell arteritis. It is often associated with another type of inflammatory condition, called polymyalgia rheumatica (PMR), which causes pain and inflammation in the large joints of the body (shoulders, etc.).
- Takayasu arteritis: it affects medium and large arteries (in particular, the aorta and its branches) and manifests itself as a systemic disease. Symptoms of Takayasu's arteritis can include fatigue and sickness, fever, night sweats, joint pain, loss of appetite, numbness, high blood pressure, headache and visual disturbances.
- Buerger's disease: also known as thromboangiitis obliterans, causes inflammation of the blood vessels at the extremities. As a result, less blood flows to the affected tissues, causing pain in the calves and feet when walking or pain in the hands and forearms with activity. In the most serious cases, ulcers can develop on the fingers and gangrene (death or decay of body tissues). Other symptoms include the formation of blood clots in the superficial veins of the limbs; rarely, Buerger's disease can affect the blood vessels of the brain, abdomen and heart. The disease is strongly associated with cigarette smoking.
- Kawasaki disease : rare childhood disease, also known as mucocutaneous lymph node syndrome; in fact, this pathology is typically associated with swollen lymph nodes and redness of the skin and mucous membranes of the eyes and mouth. It can affect any blood vessel in the body.
- Polyarteritis nodosa: it can occur in medium-sized blood vessels, in different parts of the body. It often affects the kidneys, the digestive system, peripheral nerves and the skin. Signs and symptoms include: anemia, fever, feeling sick, weight loss, purpura, skin ulcers, kidney problems, muscle, abdominal and joint pain. Some cases of polyarteritis nodosa appear to be associated with hepatitis B or C.
- Churg-Strauss syndrome: also known as allergic granulomatosis or allergic vasculitis, it can affect many organs, including the lungs, skin, kidneys, nervous system and heart. The symptoms can vary greatly.
- Essential mixed cryoglobulinemia: it is often associated with hepatitis C virus infections, but can also occur together with other diseases. "Cryoglobulinemia" refers to the presence of abnormal proteins in the blood, which precipitate (forming a gel) when a serum sample is placed at a lower temperature than body temperature. Symptoms often include joint pain, palpable purpura, weakness and neuropathy.
- Schönlein-Henoch purpura: inflammation of the smaller blood vessels of the skin, joints, intestines and kidneys. Symptoms may include abdominal pain, blood in the urine, joint pain, signs of kidney damage (hematuria) and purpura on the buttocks, legs and feet. The disease is more common in childhood, but can also affect adolescents and adults. In children, about half of all cases are caused by a viral or bacterial infection of the upper respiratory tract.
- Hypersensitivity vasculitis (or leukocytoclastic): it can be triggered by an allergic reaction (often drug-induced) or by an infection. A common symptom is the appearance of red spots on the skin, usually on the lower part of the legs or back. Drug withdrawal or treatment of the infection usually resolves vasculitis.
- Microscopic polyangiitis: affects small blood vessels, especially kidneys and lungs. Clinical signs are often not specific and can gradually begin with fever, weight loss and muscle pain. Sometimes, symptoms progress suddenly and rapidly, inducing kidney failure. If the lungs are affected, the first symptom may be the emission of blood with cough (hemoptysis).
- Wegener's granulomatosis: it can be located at the level of the nose, paranasal sinuses, throat, lungs, kidneys and other organs. In addition to inflammation of the blood vessels, the affected tissues can also develop abnormal nodules, called granulomas. If these develop in the lungs, they can destroy the lung tissue. The damage can be confused by pneumonia or lung cancer. Signs and symptoms may include fever, weight loss, muscle pain, nasal obstruction, chronic sinusitis and nosebleeds. The kidneys are often affected, although most people do not have any obvious symptoms until the damage is more advanced (kidney failure).
Vasculitis can affect people of both sexes and affect a wide range of ages. Some forms of vasculitis most commonly affect certain groups of people. For example, Kawasaki disease occurs only during childhood; Schönlein-Henoch purpura is more common in children than in adults, while temporal arteritis occurs only over 50 years of age.
In children, the most common forms of vasculitis occur suddenly and usually as a single episode.
Other forms have a slower and more subtle onset that lasts for weeks or months, making diagnosis difficult; this is the case of polyarteritis nodosa, Wegener's granulomatosis and Takayasu's arteritis.
Complications
The complications of vasculitis can be multiple and depend on the underlying cause, type and size of the affected blood vessel. Inflammation can lead to loss of function in the affected tissues, organs or systems of the body.
In general, complications can include:
- Organ damage . The interruption of blood flow, caused by inflammation, can damage the organs supplied by the blood vessel. Some types of vasculitis can cause serious consequences: renal failure, gangrene, pulmonary hemorrhage, subglottic stenosis, arterial or venous thrombosis, etc. Organs involved in vasculitis, such as the heart or lungs, may require specific medical treatment designed to improve their function during the active phase of the disease;
- Recurrent episodes of vasculitis . Even when treatment for vasculitis is initially successful, the condition may recur and require additional therapy. In other cases, the disease may never completely resolve and requires long-term treatment.
Vasculitis: Diagnosis and Treatment »
