Discover the causes, symptoms, and treatment options for vasculitis. Get expert insights and guidance on managing this inflammatory condition with Meri Sehat.

Vasculitis

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Syed Intisar Hussain

Overview

Vasculitis is defined as an inflammation of the blood vessels. 
&nbsp;

Also called angiitis and arteritis, vasculitis refers to a group of rare disorders characterized by inflammation of the blood vessels. These vessels include arteries, veins, and capillaries. There are several forms of vasculitis, each with its own set of symptoms, severity, and longevity. Most kinds of vasculitis are rare with unknown causes. Vasculitis affects people of all ages and genders. However, a few types are only found in certain populations. For example, Kawasaki disease mostly affects Asian children, while IgA vasculitis (Henoch-Sch&ouml;nlein) occurs in children and adolescents. Moreover, giant cell arteritis only affects people over the age of 50.

The incidence and prevalence of the different forms of vasculitis also vary. Takayasu&rsquo;s arteritis has a global incidence of 1-2 million<a href="#accordion">[1]</a>. As per a 2019 study, giant cell arteritis has an incidence of 10 per 100,000 people above the age of 50, with Scandinavian countries reporting the most cases<a href="#accordion">[10]</a>. On the other hand, <a href="https://merisehat.pk/disease/kawasaki-disease">Kawasaki disease</a> predominantly affects children in Japan. The current incidence rate in the country is 359 per 100,000 children under the age of five<a href="#accordion">[10]</a>.

In the West, Henoch- Sch&ouml;nlein purpura is the most common type of childhood vasculitis with an incidence of 20/100,000 aged less than 17 years<a href="#accordion">[1]</a>. Inflammation of the blood vessels causes them to weaken and stretch. Thus, increasing the risk of aneurysms. Vasculitis can also cause a narrowing of the vessels, formation of blood clots, and/or blockage of blood flow to the organ. Moreover, vasculitis has been linked to hematologic cancers.

Thus, the severity of vasculitis can range from mild to life-threatening. 
&nbsp;

The term vasculitis includes approximately 20 different disorders. Thus, this group of disorders is often referred to as vasculitides (plural for vasculitis).

Doctors typically classify the disease based on the size of the blood vessels affected. 
&nbsp;

<h5>Large Vessel Vasculitis</h5>

Giant Cell Arteritis (Temporal Arteritis) - This is the most prevalent kind of vasculitis, with the highest prevalence among Scandinavian communities, where the yearly incidence ranges from 15 to 35/100,000 people over the age of 501. The disease is characterized by an inflammation of the arteries of the head and neck. It can cause headaches, double vision, pain around the temples, and jaw pain while eating or talking.

Polymyalgia Rheumatica - It affects adults over the age of 50 and most commonly occurs alongside giant cell arteritis. Symptoms include pain and stiffness in the neck, shoulders, and hips, fever, fatigue, and loss of appetite.

Takayasu&rsquo;s Arteritis - Takayasu&#39;s arteritis has a worldwide incidence of one to two cases per million1. It affects the aorta as well as its branches. Symptoms include fever, weight loss, fatigue, and muscle and joint pain. Young women are most commonly affected. 
&nbsp;

<h5>Medium Vessel Vasculitis</h5>

Polyarteritis Nodosa - This rare multisystem disorder is characterized by extensive inflammation, thinning, and atrophy of small and medium-sized arteries. Blood vessels in any organ or organ system, including those supporting the kidneys, heart, gut, neurological system, and/or skeletal muscles, may be impacted. Although the actual etiology of polyarteritis nodosa is unknown, many experts believe the condition is caused by immune system malfunctions. Polyarteritis nodosa mostly develops in childhood or middle age. Signs and symptoms include abdominal pain, muscle &amp; joint pain, ulcers in the gut, and pins and needles or numbness.

Kawasaki Disease - Kawasaki disease is most prevalent in Asian children, mostly those under the age of 5. Coronary arteries are most commonly affected. Common signs and symptoms include fever, skin rash, enlarged lymph nodes, red eyes, dry, cracked lips, and red fingers or toes.&nbsp;&nbsp; 
&nbsp;

<h5>Small Vessel Vasculitis</h5>

Vasculitis affecting the small-sized blood vessels is further divided into anti-neutrophil cytoplasm antibodies (ANCA)-associated vasculitis and non-ANCA vasculitis. ANCA-associated vasculitis includes&nbsp; eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, and granulomatosis with polyangiitis. Non-ANCA vasculitis includes Henoch-Sch&ouml;nlein purpura.

Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) - Churg-Strauss syndrome is a rare disorder characterized by the development and accumulation of an unusually large number of eosinophils, inflammation of blood vessels, and inflammatory nodular lesions in several organ systems (granulomatosis). It mainly affects adults aged between 30 to 45 years. Symptoms include fever, allergic rhinitis, tiredness, muscle &amp; joint pain, and loss of appetite.

Henoch-Schonlein Purpura (IgA vasculitis) - Henoch-Sch&ouml;nlein purpura is the most frequent form of childhood vasculitis in the Western world. It is a rare, self-limiting inflammatory illness of the capillaries. The kidneys, skin, or bowel can be affected. Infections may trigger vasculitis, causing joint pain, abdominal pain, reddish-purple spots, and blood in urine or stool.&nbsp;

Wegener&rsquo;s Granulomatosis (Granulomatosis With Polyangiitis) - Wegener&#39;s granulomatosis tends to be more prevalent in northern Europe1.&nbsp; It is a rare collagen vascular condition that mainly affects the vessels in the ears, nose, kidneys, and lungs. Symptoms include fever, night sweats, nosebleeds, shortness of breath, and renal issues. If left untreated, it can lead to organ failure. The specific cause of Wegener&#39;s granulomatosis remains unknown.

Microscopic Polyangiitis - This is a rare and long-term form of vasculitis that mostly affects middle-aged adults. The kidneys, lungs, and nerves are most commonly affected. Symptoms include a rash, shortness of breath, red eyes, kidney problems, and pins &amp; needles or numbness. 
&nbsp;

<h5>Other Types Of Vasculitis</h5>

The following types of vasculitis are less common:

Behcet&rsquo;s Disease - Beh&ccedil;et&#39;s disease is found throughout the Silk Road and the Mediterranean littoral, with a prevalence of 380/100,000 in Turkey 1. It generally causes mouth ulcers and genital ulcers.

Bueger&rsquo;s Disease - Buerger&#39;s disease causes blood vessels in the extremities to constrict, resulting in decreased blood flow to the hands and feet. It is significantly associated with smoking.

Cogan&rsquo;s Syndrome - This is characterized by an inflammation of the blood vessels in the eyes and inner ears.

Rheumatoid Vasculitis - As the name suggests, vasculitis is associated with rheumatoid arthritis.

Cryoglobulin-associated vasculitis - This usually follows a hepatitis c infection due to the presence of proteins called cryoglobulins in the blood. Symptoms include a rash, abdominal pain, joint pain, kidney problems, and nerve damage.

Hypersensitivity Vasculitis - Medicines, such as NSAIDs and antibiotics can result in hypersensitivity vasculitis.

Primary Angiitis Of The Central Nervous System - The blood vessels in the brain become inflamed.

Vasculitis can also be classified based on its etiology, into primary and secondary.

<ul>
	<li>Primary Vasculitis - It has no known cause.</li>
	<li>Secondary Vasculitis - It is mostly triggered by an infection, or medication, or occurs as part of an inflammatory disorder or cancer. 
	&nbsp;</li>
</ul>

Signs and Symptoms

There are several forms of vasculitis, each of which can limit blood flow to virtually any tissue, organ, or structure in the body. Depending on where the inflammation is present or blood flow is impaired, this results in a comprehensive list of possible symptoms.

Symptoms might be moderate or severe. However, in some cases, no symptoms appear until the illness has progressed significantly. General signs of most types of vasculitis include:

<ul>
	<li>Fever</li>
	<li><a href="https://merisehat.pk/disease/cluster-headaches">Headache</a></li>
	<li>Fatigue</li>
	<li>Weight loss</li>
	<li>General aches and pains 
	&nbsp;</li>
</ul>

Symptoms can be divided based on the part of the body that is affected.

<ol>
	<li>Digestive System: Individuals may experience abdominal pain, especially after eating. <a href="https://merisehat.pk/disease/peptic-ulcer">Ulcers </a>and perforations can occur and may result in blood in the stool.</li>
	<li>Ears: People may have dizziness, ringing in the ears, and sudden hearing loss.</li>
	<li>Eyes: Vasculitis can cause the eyes to redden, itch, or burn. Giant cell arteritis can result in double vision and, in severe cases, blindness in one or both eyes. This is occasionally the first symptom of the illness.</li>
	<li>Hands And Feet: Numbness or weakness in the extremities can be caused by several kinds of vasculitis. The palms of the hands and the soles of the feet may enlarge or stiffen.</li>
	<li>Lungs: If vasculitis affects the lungs, it may cause shortness of breath or hemoptysis.</li>
	<li>Skin: Bleeding beneath the skin might manifest as red patches. Vasculitis can also create skin lumps, open wounds, or even rashes. 
	&nbsp;</li>
</ol>

Causes

Researchers are still trying to figure out what triggers most types of vasculitis. The condition appears to be slightly influenced by genetic factors. Vasculitis is regarded to be an autoimmune condition, which implies that the body&#39;s immune system attacks itself.

Some instances of vasculitis are caused by allergies or drug responses. Additionally, some chronic infections, such as those caused by <a href="https://merisehat.pk/disease/hepatitis-c-114">hepatitis C</a> or <a href="https://merisehat.pk/disease/hepatitis-b-113">hepatitis B</a> virus, as well as environmental factors, can result in vasculitis. 
&nbsp;

Diagnosis

A person&#39;s medical history, present symptoms, a thorough physical examination, and the findings of specific laboratory testing are used to make a diagnosis of vasculitis. Doctors may evaluate the patient for anemia, levels of white blood cells, elevated platelet counts, and symptoms of liver or kidney disease.

The following tests and procedures are conducted: 
&nbsp;

<ol>
	<li>Blood Work - These tests identify indicators of inflammation, such as elevated C-reactive protein levels. A total blood cell count can determine if one has anemia. Moreover, the anti-neutrophil cytoplasmic antibody (ANCA) test can help diagnose vasculitis. &nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;</li>
	<li>Urine Tests - These are used to assess the kidney&rsquo;s functioning.</li>
	<li>Imaging Tests - Noninvasive imaging methods can aid in determining which blood arteries and organs are involved. They can also assist the doctor in determining how well patients are responding to treatment. X-rays, ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography (PET) are among the imaging procedures used to diagnose vasculitis.</li>
	<li>Echocardiogram - This helps determine how well the heart is functioning. It is useful when vasculitis affects coronary arteries or the aorta and its branches.</li>
	<li>X-rays Of Your Blood Vessels (Angiography) - A flexible catheter, similar to a thin straw, is placed into a major artery or vein during this procedure. The catheter is then injected with a particular dye, and X-rays are obtained while the dye fills the vessel. On the resultant X-rays, the outlines of the blood vessels are evident.</li>
	<li>Biopsy - A sample of tissue from the afflicted area of the body is removed and examined for signs of inflammation. 
	&nbsp;</li>
</ol>

Treatment

Treatment of vasculitis depends upon the affected organ and it primarily focuses on reducing inflammation and addressing any underlying causes. Vasculitis caused by an allergic response may resolve on its own. However, if vital organs such as the lungs, brain, or kidneys are damaged, immediate treatment is required. The plan of treatment includes: 
&nbsp;

<h5>Medications</h5>

The most frequent type of medicine used to reduce the inflammation associated with vasculitis is steroids, such as prednisone, prednisolone, or others. The dose and duration of treatment are determined by the severity and duration of the disease. Although effective at reducing inflammation, these medications can cause long-term side effects, such as:

<ul>
	<li>Weight gain</li>
	<li><a href="https://merisehat.pk/disease/diabetes-type-1-and-2">Diabetes</a></li>
	<li><a href="https://merisehat.pk/disease/osteoporosis-80">Osteoporosis</a> 
	&nbsp;</li>
</ul>

Other drugs may be used in conjunction with corticosteroids to manage inflammation. Moreover, they allow the corticosteroid dosage to be weaned more quickly. The drug prescribed is determined by the kind of vasculitis present. A few of them are enlisted below:

<ul>
	<li>Methotrexate</li>
	<li>Azathioprine</li>
	<li>Mycophenolate</li>
	<li>Cyclophosphamide</li>
	<li>Tocilizumab</li>
	<li>Rituximab</li>
</ul>

Remission is usually induced with the help of immunosuppressants and high-dose steroids. In severe vasculitis, cyclophosphamide or rituximab may be used. For less severe vasculitis, doctors may use a less potent immunosuppressant (methotrexate azathioprine) or rituximab to induce remission. A continuation of immunosuppressive therapy with or without low-dose steroids can then help maintain remission.&nbsp;

Patients undergoing treatment with immunosuppressants need to be monitored for opportunistic and other infections. Moreover, tuberculosis and hepatitis B can become reactivated in these patients.

Recently, the FDA approved avacopan as an adjunctive treatment for adults with severe and active ANCA-associated vasculitis. 
&nbsp;

<h5>Surgery</h5>

An aneurysm is a bulge or ballooning in the wall of a blood artery caused by vasculitis. This bulge may necessitate surgery to limit the danger of rupture. Blocked arteries may also demand surgical intervention to restore blood flow to the afflicted location. 
&nbsp;

The complications of vasculitis vary depending on the severity of the disease. Some common complications include:

<ul>
	<li>Blood Clots And Aneurysms - Vasculitis can lead to the formation of blood clots which can then obstruct blood flow to the organs. Moreover, inflammation of the vessels can cause them to weaken and bulge, resulting in an aneurysm. This may potentially break open, resulting in bleeding. Although rare, it can be fatal.</li>
	<li>Organ Damage - When a blood vessel becomes inflamed and constricted, blood flow to that area of the body may be partially or completely blocked. Thus, resulting in organ damage.</li>
	<li>Visual Disturbances - Untreated giant cell arteritis can result in vision loss or blindness</li>
	<li>Infections - Medications used to treat vasculitis can weaken one&rsquo;s immune system, making people prone to infections.</li>
	<li>Alveolar hemorrhage</li>
	<li>Mesenteric ischemia</li>
	<li>Rapidly progressive glomerulonephritis 
	&nbsp;</li>
</ul>

The following factors can increase the risk of certain types of vasculitis: 
&nbsp;

<ul>
	<li>Age: Giant cell arteritis is infrequent before the age of 50, while Kawasaki illness is most prevalent in children under the age of five.</li>
	<li>Gender: Giant cell arteritis is more frequent in women, whereas Buerger&rsquo;s syndrome is more common in men.</li>
	<li>Hereditary Factor: Behcet&#39;s disease, granulomatosis with polyangiitis and Kawasaki disease sometimes run in families.</li>
	<li>Medication: Drugs such as hydralazine, allopurinol, minocycline, and propylthiouracil might induce vasculitis.</li>
	<li>Infections: Hepatitis B or C may increase the risk of vasculitis.</li>
	<li>Immune Disorder: Vasculitis risk may be increased in those with immunological diseases, such as scleroderma, lupus, and rheumatoid arthritis.</li>
	<li>Lifestyle Factors:&nbsp;Cocaine use is known to increase the risk of vasculitis. Moreover, smoking and alcohol can increase the risk of Buerger&rsquo;s disease. 
	&nbsp;</li>
</ul>

The following measures can help prevent vasculitis: 
&nbsp;

<ul>
	<li>Quit using cocaine as it may increase the risk of developing vasculitis.</li>
	<li>Quit smoking and alcohol.</li>
	<li>Adopt a heart-healthy lifestyle. 
	&nbsp;</li>
</ul>

Key Points

<ul>
	<li>Also called angiitis and arteritis, vasculitis refers to a group of rare disorders characterized by inflammation of the blood vessels. These vessels include arteries, veins, and capillaries.</li>
	<li>Vasculitis affects people of all ages and genders. However, a few types are only found in certain populations. For example, Kawasaki disease mostly affects Asian children, while IgA vasculitis (Henoch-Sch&ouml;nlein) occurs in children and adolescents. Moreover, giant cell arteritis only affects people over the age of 50.</li>
	<li>Inflammation of the blood vessels causes them to weaken and stretch. Thus, increasing the risk of aneurysms. Vasculitis can also cause a narrowing of the vessels, formation of blood clots, and/or blockage of blood flow to the organ.</li>
	<li>There are several forms of vasculitis, each of which can limit blood flow to virtually any tissue, organ, or structure in the body. Depending on where the inflammation is present or blood flow is impaired, this results in a comprehensive list of possible symptoms.</li>
	<li>The most frequent type of medicine used to reduce the inflammation associated with vasculitis is steroids. Although effective at reducing inflammation, these medications can cause long-term side effects, such as weight gain, osteoporosis, and diabetes.</li>
	<li>Remission is usually induced with the help of immunosuppressants and high-dose steroids. In severe vasculitis, cyclophosphamide or rituximab may be used. For less severe vasculitis, doctors may use a less potent immunosuppressant (methotrexate azathioprine) or rituximab to induce remission. 
	&nbsp;</li>
</ul>

[1] Watts RA, Scott DG. Epidemiology of the vasculitides. Semin Respir Crit Care Med.

[2] NHS choices.  Accessed December 6, 2022.

[3] Vasculitis. Mayo Clinic.

[4] American College of Rheumatology. Accessed December 6, 2022.

[6] Vasculitis: Symptoms, diagnosis & treatment. Cleveland Clinic.

[7] Vasculitis. NORD (National Organization for Rare Disorders).

[8] Types of vasculitis. Johns Hopkins Vasculitis Center.

[9] Villa-Forte A. Overview of vasculitis - musculoskeletal and connective tissue disorders. MSD Manual Professional Edition.

[10] Watts RA, Hatemi G, Burns JC, Mohammad AJ. Global epidemiology of vasculitis. Nat Rev Rheumatol. 2022;18(1):22-34. doi:10.1038/s41584-021-00718-8.