Vasculitis: Symptoms, Causes, Therapy

Brief overview

  • What is Vasculitis? An inflammatory disease of the blood vessels resulting from a defective immune response.
  • Causes: In primary vasculitis, the cause is unknown (e.g., giant cell arteritis, Kawasaki syndrome, Schönlein-Henoch purpura). Secondary vasculitis is caused by other diseases (such as cancer, viral infection) or drugs.
  • Diagnosis: taking the medical history, physical examination, laboratory tests, tissue samples, imaging procedures such as magnetic resonance imaging (MRI), computer tomography (CT), X-ray or ultrasound, further examinations if necessary.
  • Treatment: depending on the form of vasculitis, e.g. with drugs that suppress the immune system (immunosuppressants) and non-steroidal anti-inflammatory drugs. In secondary vasculitides: Treatment of the underlying disease.

Vasculitis: Description

All these forms of vasculitis have one thing in common: The vascular inflammation is caused by certain immune system defense substances attacking the vessel walls. Thus, vasculitis belongs to the autoimmune diseases. These are diseases in which the immune defense is directed against the body’s own structures.

In addition, vasculitides belong to the rheumatic diseases because they are often accompanied by pain in joints or muscles and sometimes also by joint swelling.

In some types of vasculitis, tissue nodules composed of various cells (such as epithelioid cells, giant cells) may form. These so-called non-infectious granulomas are found, for example, in a

  • Granulomatosis with polyangiitis (Wegener’s disease)
  • Eosinophilic granulomatosis with polyangiitis (Churg-Strass syndrome)
  • Giant cell arteritis
  • Takayasu arteritis

Our vascular system

There are different types of vessels in the body. First, we distinguish between arteries and veins:

  • Veins return the blood to the heart.

The transition between arteries and veins is formed by the so-called capillaries (hairpin blood vessels). These are the smallest blood vessels in the body. They form a vascular network through which the exchange of substances takes place in the respective organ: The cells take up nutrients and oxygen from the blood in the capillaries and release waste products to them.

Since the entire body is permeated by blood vessels, vasculitis can occur virtually anywhere.

Types of vasculitis

In very general terms, a distinction is made between primary and secondary vasculitides.

Primary vasculitides

Giant cell arteritis

Giant cell arteritis is the most common form of vasculitis. The inflammation here affects large blood vessels – in the vast majority of cases the temporal artery. Such cases are referred to as temporal arteritis.

The disease occurs more often in women than in men and preferably at an older age (50 years and older). It is often associated with the inflammatory rheumatic disease polymyalgia rheumatica.

Kawasaki syndrome

This rare form of vasculitis usually affects small children: medium-sized vessels become inflamed, for example coronary vessels. This can lead to life-threatening complications.

Read more about this form of primary vasculitis in the article Kawasaki syndrome.

Granulomatosis with polyangiitis

You can read everything important about this form of vasculitis in the article Granulomatosis with polyangiitis (formerly Wegener’s disease).

Vasculitis anaphylactoides (Purpura Schönlein-Henoch)

This form of primary vasculitis, which occurs mainly in children, is associated with inflammation of small blood vessels and capillaries. As a result, there are punctate hemorrhages in the skin and mucous membranes (petechiae).

You can read more about this form of vasculitis in the article Purpura Schönlein-Henoch.

Outside of the categorization as noted in the table above, there are other primary vasculitides such as:

  • Thrombangitis obliterans (endangiitis obliterans): It affects mainly small and medium-sized vessels in the legs. It mostly affects young men (< 40 years), especially heavy smokers.
  • Cerebral vasculitis: It is also called primary CNS vasculitis and affects only vessels in the brain and spinal cord.
  • Hypocomplementemic urticarial vasculitis syndrome: It affects the skin and is manifested by the formation of erythema (skin redness) or wheals that persist for more than 24 hours. Another name for this condition is urticarial vasculitis.

Secondary vasculitides

Vasculitis: Symptoms

The symptoms of vasculitis depend on the form and extent of the disease.

General symptoms

In most cases, vasculitis begins with nonspecific symptoms: many patients initially feel fatigued and tired. In addition, there is a slight fever, usually below 38.5 degrees Celsius (subfebrile temperature). Some patients report severe night sweats and unwanted weight loss.

In addition to these rather vague vasculitis symptoms, rheumatic complaints may also occur: Some patients complain of joint pain, sometimes associated with swelling. Others suffer from muscle pain (myalgias) and report unusually severe muscle catarrh.

If the vasculitis progresses further and affects the organs, more severe symptoms appear. They vary depending on the form of vasculitis.

Vasculitis symptoms in inflammation of small vessels

  • Eye redness and visual disturbances in inflammation of small vessels in the eye
  • Mucosal damage in the mouth with the formation of painful aphthae (small blisters) at the edge of the tongue or on the inside of the lips in the case of inflammation of small vessels in the mouth area
  • recurrent sinusitis and a blocked, occasionally bleeding nose in the case of vasculitis of the small vessels in the area of the nose and sinuses
  • Shortness of breath and coughing up blood as small vessel vasculitis damages lungs

Other possible vasculitis symptoms, depending on the region of the body in which small vessels are inflamed, include, for example, bloody diarrhea or bloody urine, chest pain (if myocardium or pericardium are affected), tingling, or sensations of discomfort (paresthesias).

Vasculitis symptoms in inflammation of medium-sized vessels

  • Heart attack
  • Stroke
  • Intestinal infarction
  • Renal infarction

Vasculitis symptoms in inflammation of large vessels

If vasculitis affects large arteries in the head, patients usually suffer from severe headaches. Some see abruptly worse or even go completely blind.

The large vessels in the arms and legs can also become blocked due to vasculitis, resulting in severe pain.

Symptoms of different forms of vasculitis

Takayasu arteritis: symptoms

Inflammation of the aorta and its vascular branches characterizes this form of vasculitis. The initial stage (preocclusive stage, prepulseless stage) presents insidiously with mild fever, fatigue, joint pain, headache, and weight loss.

Later (occlusive stage, pulseless phase), other vasculitis symptoms develop.

  • In some sufferers, the arms ache and the fingers become pale and cold in fits and starts (Raynaud’s syndrome).
  • If brain vessels are inflamed, visual disturbances, dizziness with fainting or a stroke may occur.
  • Takayasu vasculitis near the heart can lead to symptoms of coronary artery disease (CAD). These include, for example, an uncomfortable feeling of pressure in the chest (angina pectoris).

Panarteritis nodosa: symptoms

This form of vasculitis affects men about three times more often than women. It can damage various organs, which is why vasculitis symptoms can vary widely.

In most cases, coronary vessels are inflamed. Affected people then often feel pressure or pain in the chest (angina pectoris) and may eventually suffer a heart attack (even younger patients). Other possible symptoms include:

  • Fever, night sweats, weight loss
  • crampy abdominal pain (colics), possibly intestinal infarctions
  • testicular pain
  • stroke (also in young patients)
  • paraesthesia, numbness (polyneuropathy; mononeuritis multiplex), epileptic seizures, psychoses
  • Vascular outpouchings (aneurysms)

In numerous patients, vascular inflammation also damages the kidneys, although not the fine renal corpuscles (no glomerulonephritis).

Eosinophilic granulomatosis with polyangiitis: symptoms.

This form of vasculitis is also called allergic granulomatous angiitis (formerly Churgh-Strauss syndrome). It preferentially affects the respiratory tract, usually triggering asthma attacks with acute respiratory distress. White blood cells are detectable in the blood, which are also typical of allergy.

Microscopic panarteritis (MPA): Symptoms.

This form of vasculitis usually affects the small renal vessels: inflammation of the renal corpuscles (glomerulonephritis) develops, resulting in elevated blood pressure (hypertension) and headache.

If small skin vessels are affected by vasculitis, small nodules and palpable hemorrhages form under the skin (palpable purpura), especially on the legs.

Vasculitis in essential cryoglobulinemia: symptoms.

Hemorrhages on the hands and feet are typical for this vasculitis variant. In addition, tissue defects (ulcers) and joint pain may occur. In severe cases, kidney and nerve damage often develops.

Cutaneous leukocytoclastic angiitis (KLA): symptoms

Behçet’s disease: symptoms

If Behçet’s disease affects the skin and mucous membranes, painful ulcers develop in the mouth (oral aphthae) and intimate areas (genital aphthae). Sometimes pressure-sensitive nodules also form (erythema nodosum).

Often the eyes are also affected. Then very often the middle eye skin becomes inflamed (uveitis).

In addition, it is not uncommon for the joints to become inflamed (arthritis).

In up to 30 percent of those affected, vessels in the central nervous system (CNS) become inflamed.

As a general rule, the more active the inflammation, the higher the risk of dangerous blood clots (thromboembolisms).

Cerebral vasculitis: symptoms

Cerebral vasculitis may lead to insufficient blood supply to regions of the brain, which may result in a stroke (ischemic stroke). Occasionally, a stroke can also be caused by vascular bleeding (hemorrhagic stroke).

Epileptic seizures are also among the possible symptoms of CNS vasculitis.

Thrombangiitis obliterans: symptoms

The skin may have a bluish discoloration due to reduced blood supply. As vasculitis progresses, the tissue dies, especially on the tips of the toes – blackish skin defects become visible. In addition, nail growth may be impaired.

Vasculitis: development and triggers

In this context, special proteins play a role, which are usually activated by immune complexes. More precisely, these are certain glycoproteins known as complement factors. They can destroy cells and cause inflammations such as vasculitis.

Possible triggers of primary vasculitis

Vasculitis: examinations and diagnosis

The responsible specialist for vascular diseases is always an internist. If the skin is affected by vasculitis, a dermatologist may be the right contact. In addition, vaculitis can be diagnosed and treated in a specialized clinic.

Medical history

If vasculitis is suspected, the physician will first talk with you in detail to obtain your medical history (anamnesis). Possible questions to ask are:

  • What are your symptoms?
  • Do you feel fatigued and tired?
  • Have you lost weight unintentionally lately?
  • Do you sweat a lot at night?
  • Is your temperature elevated?
  • Have you noticed any skin changes (e.g. pinpoint redness)?
  • Do you have or have you recently had a flu-like infection? Do you still have to cough, possibly also blood?
  • Do you suffer from underlying diseases, for example rheumatism?
  • Do you have any known infections (e.g. with hepatitis viruses)?
  • What medications are you taking?

Physical examination

The ear, nose and throat tract is also examined to rule out any inflammation. If a patient suffers from dizziness, fainting spells or skin sensations, the neurological status can be checked using various tests.

Laboratory tests

Vasculitis often alters blood and also urine values. Therefore, blood and urine samples are examined in the laboratory for certain parameters that are typical of vasculitis. For example, inflammation values (CRP, erythrocyte sedimentation rate, leukocytes) often increase in vasculitis. Of particular importance are characteristic proteins of the defense system, autoantibodies or immune complexes.

Tissue examination

  • Samples from skin, mucosa or kidney are taken under local anesthesia. The procedure usually takes 15 to 30 minutes.
  • Lung tissue is usually obtained during lung endoscopy (bronchoscopy).
  • If giant cell vasculitis of the temporal artery (arteritis temporalis) is suspected, the physician removes a piece of this vessel at least 20 millimeters long.

Imaging

Imaging examinations help to rule out other causes for the symptoms that occur, such as cancer, bleeding or vascular changes. For example, magnetic resonance imaging (MRI) or computed tomography (CT) are used – often in combination with contrast media to better visualize vessels (angiography). Another imaging method is positron emission tomography (PET).

Another imaging examination is ultrasound (sonography), called echocardiography in the heart. This can be used, for example, to check blood flow (color duplex sonography) and vascular constrictions or bulges. Ultrasound is also suitable for joint examinations.

Further examinations

However, a similar reaction may also be seen in leukocytoclastic vasculitis and granulomatosis with polyangiitis. In addition, a negative test result does not exclude Behçet’s vasculitis.

Criteria for vasculitis diagnostics

Some vasculitis can only be diagnosed if certain conditions are (partially) met. The American College of Rheumatology (ACR, formerly ARA) has developed these criteria. They exist for the following vasculitis disorders:

  • Polyangiitis with granulomatosis
  • Eosinophilic granulomatosis with polyangiitis
  • Panarteritis nodosa
  • Giant cell arteritis
  • Takayasu arteritis

Vasculitis: Treatment

In addition, in certain cases, surgical interventions on vessels may become necessary (for example, in Takayasu’s arteritis).

In the treatment of secondary vasculitis, the underlying disease is treated first and foremost. Some triggers can be eliminated and should subsequently be avoided (such as certain drugs or food additives).

Treatment of small vessel vasculitis

In the case of vasculitis anaphylactoides (Schönlein-Henoch purpura), doctors prescribe cortisone – especially in cases of severe skin involvement. Alternatively, immunosuppressants (such as cyclophosphamide) or immunoglobulins are given. If the kidneys are damaged, doctors also use ACE inhibitors (or angiotension II blockers). They have a blood pressure-lowering effect.

In the case of eosinophilic granulomatosis with polyangiitis (EGPA), immunosuppressive therapy is generally initiated, for example with cortisone alone or cortisone plus methotrexate. Sometimes the treating physician also prescribes biologics or immunomodulators. These include the artificially produced antibody mepolizumab. Among other things, it has an anti-inflammatory effect. In individual cases, the treatment can be supplemented by other drugs.

Panarteritis nodosa is primarily treated with methotrexate. If the disease progresses, doctors prescribe a combination of cyclophosphamide and cortisone preparations. In the case of an additional hepatitis B infection, low-dose steroid therapy is given in combination with viral drugs (such as lamivudine).

Treatment of giant vessel vasculitis

In giant cell arteritis, cortisone preparations are the drug of choice. They must be taken for a longer period of time: first in high doses, then with gradual dose reduction. With the help of the artificial antibody tocilizumab (TOC), which is injected weekly under the skin, the cortisone dosage can be reduced more quickly. Alternatively, methotrexate can be given for this purpose.

Vascular interventions may be necessary, particularly in the case of Takayasu’s arteritis, but also in other forms of vasculitis that cause accessible veins to narrow. For example, the surgeon may insert “vascular supports” (stents) to keep a vessel open and pervious. The use of a vessel wall prosthesis can also be useful in the case of a dangerous vessel bulge (aneurysm).

Treatment of other forms of vasculitis

In endangiitis obliterans, doctors also prescribe cortisone preparations. In addition, vasodilators such as prostaglandins are sometimes prescribed – despite their uncertain effect. The most important basic measure for this vasculitis, however, is to abstain from nicotine.

Severe sequelae of vasculitis such as stroke, myocardial infarction, renal failure, aneurysm rupture or other organ damage must also be treated appropriately.

Vasculitis: disease course and prognosis

In addition to drug treatment, experts recommend vasculitis patients,

  • exercise regularly,
  • eat a healthy diet (high-quality vegetable oils, little meat, high-protein food – adapted to kidney function), and
  • avoid nicotine.

Exchanging information with other sufferers (for example in self-help groups or online in a vaculitis forum) can also help to cope better with the consequences of vasculitis.

  • Vascular inflammation can recur at any time and cause discomfort. Often, this flare-up is heralded by general, flu-like symptoms.
  • Classic infections such as a cold can cause the disease to flare up again.

In both cases, the physician should be consulted as soon as possible in order to alleviate the symptoms at an early stage and to counteract an aggravation of the vasculitis.