Vasculitis

What is Vasculitis?

Vasculitis refers to a variety of conditions marked by inflammation in your blood vessels. These conditions share similarities in how they appear, how they are diagnosed in the lab, and how they affect your body. However, they can look and act differently depending on where in your body they occur and what type of blood vessels are impacted. Over 30 different kinds of vasculitis have been discovered through research.

Vasculitis can either occur on its own, which is termed as primary systemic vasculitis, or it can happen as a result of another underlying disease. When it comes to classifying these conditions, medical professionals often use guidelines set by the American College of Rheumatology from 1990. Additionally, the naming system most commonly used for vasculitis comes from the International Chapel Hill Consensus Conference.

What Causes Vasculitis?

Vasculitis, which is the inflammation of blood vessels, can be caused by many factors that aren’t completely understood. However, we do know that the likelihood of having vasculitis increases based on certain factors like where you live, how old you are, your ethnicity, sex, DNA, and your environment.

More specifically, Behcet’s disease, a type of vasculitis, is more commonly found in people living near the ancient Silk Route while Takayasu’s disease is more commonly seen in South Asia. Kawasaki disease, another type of vasculitis, is usually seen in children under the age of five. Meanwhile, Giant Cell Arteritis, yet another type, is more commonly seen in older adults, with recent information showing that the chances of having it have increased 2 to 5 times over the last 50 years.

When it comes to gender, Takayasu’s disease affects more women than men (at a 9 to 1 ratio), but Behcet’s disease tends to be more severe in men. Additionally, Giant Cell Arteritis and Granulomatosis with Polyangiitis, a type of vasculitis that causes inflammation in the blood vessels of your nose, sinuses, throat, lungs, and kidneys, are more common in White populations.

Researchers have found that certain genetic components like human leukocyte antigen (HLA)-B51 can increase the likelihood and severity of Behcet’s disease, and some other genetic traits like HLA-DRB1*0401 and HLA-DRB1*0101 may have a role in Giant Cell Arteritis.

There are also certain drugs and infections that are known to be linked to vasculitis. For example, hepatitis B can increase the odds of getting Polyarteritis Nodosa, a type of vasculitis that affects small and medium-sized arteries. Hepatitis C, on the other hand, is linked to Mixed Cryoglobulinemia, a condition that causes inflammation and damage to blood vessels throughout your body. Contact with silica dust can also heighten the chances of having Pauci-Immune Vasculitis, a type of vasculitis that typically affects small blood vessels.

Risk Factors and Frequency for Vasculitis

The overall occurrence of primary systemic vasculitis, a type of autoimmune disease where the body attacks its own blood vessels, is between 20 and 40 cases per million people each year, based on research done in both Europe and the U.S. The most common form of this ailment is Giant Cell Arteritis (GCA) which affects about 240 individuals per million each year, specifically in the population aged 50 or above.

There are other types of vasculitides too, such as Granulomatosis with Polyangiitis (GPA), Microscopic Polyangiitis (MPA), and Churg-Strauss syndrome. These are reported at a rate varying from 1 to 10 cases per million individuals annually.

• Every year, between 20 and 40 people out of a million get diagnosed with primary systemic vasculitis.
• Giant Cell Arteritis (GCA), a type of vasculitis, is the most common and affects about 240 out of a million people who are at least 50 years old each year.
• Other types of vasculitides like Granulomatosis with Polyangiitis (GPA), Microscopic Polyangiitis (MPA), and Churg-Strauss syndrome are relatively rare with annual incidence ranging between 1 and 10 per million.

Signs and Symptoms of Vasculitis

Vasculitis is a group of conditions characterized by inflammation of the blood vessels. It can show various symptoms and damage different organs in the body. There isn’t a standard procedure to follow to diagnose these conditions, but medical history, physical exam, and lab tests can be crucial to identify this relatively rare but potentially severe group of diseases.

Physicians should consider vasculitis if patients come in with general systemic symptoms along with signs of malfunction in one or more organs. Things to look out for in a patient’s history include unexplained fever, weight loss, signs of upper airway disease, inexplicable bleeding, and a history of hepatitis. Physical exams might reveal nerve disorders, peculiar rashes, absence or unusualness of pulses, unexplained sounds over arteries and blood pressure irregularities.

Certain signs may suggest a vasculitis diagnosis. If a patient has a mix of the following, it could be a vasculitis indicator:

• Particular types of rash
• Abnormal results in lung tests and blood in urine
• Long-term sinus inflammation
• Nerve issues
• Unexpected blocked arteries
• Kidney inflammation associated with signs of disease in other systems

This summary will discuss two types of vasculitis: GCA and ANCA-associated vasculitis (AAV).

GCA or Giant Cell Arteritis patients often have fever, weight loss, general malaise, headaches, and visual symptoms like transient blindness, vision loss, double vision, jaw pain upon chewing, dizziness, and scalp tenderness. Some may experience unusual symptoms like throat pain, issues with large arteries, nerve disorders, mini-strokes, heart attacks, or delirium.

AAV can affect any part of the body, and symptoms usually depend on the specific type of vasculitis and the organ system involved. Patients with a subtype called GPA, for example, may experience inflammation of the upper airway, nose bleeds, sinus inflammation, and damage to the trachea or nasal cartilage, which might lead to a specific type of nasal deformation. Eye and orbital inflammation are frequent in another subtype, MPA, as well. Pulmonary issues common in GCA, MPA, and CSS can show as unexplained coughing up of blood, nodules, inflammation of the pleura, and lung bleeding.

Patients with AAV might also present with peripheral nerve disorders, hearing loss, and central nervous system vasculitis. Kidney inflammation can show early in the disease in approximately half of the patients with AAV.

Testing for Vasculitis

When a doctor suspects that a patient has vasculitis, a medical condition characterized by inflammation in the blood vessels, they will order several tests. These include a complete blood count (CBC) which looks at the types and numbers of cells in your blood, and kidney and liver function tests, which assess how well these organs are working. Other tests might include the erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP), tests that measure inflammation in your body, and tests for viral hepatitis, a type of liver infection.

Additional tests could be done for antibodies – proteins that your immune system produces to fight off ‘invaders’ in your body – known as anti-neutrophil cytoplasmic antibodies (ANCA) and anti-nuclear antibodies (ANA). Other checks might include complement levels, which are proteins that help your immune system work correctly. Doctor may also order immunoelectrophoresis, a test that looks at the proteins in your blood, specifically for any abnormal ones. Lastly, the doctor will want to do testings to rule out conditions that have similar symptoms to vasculitis but are actually caused by different issues, including certain infections.

If a patient has symptoms related to their lungs, a radiographic evaluation, such as a chest x-ray or a type of CT scan called high-resolution computed tomography (HR-CT), might be required. By looking at tissue from the affected body part under the microscope, a biopsy can help diagnose many types of vasculitis. A biopsy can also be very useful in cases of Giant Cell Arteritis (GCA), a type of vasculitis that affects the arteries in your head. Skin or kidney biopsy might also be done as they can provide useful diagnostic information.

If the vasculitis is suspected to be in the sinuses, CT imaging might be necessary. To detect lesions in the large arteries, the doctors might use vascular imaging, including MRI, MR angiograms, CT angiograms, ultrasound, and positron emission tomography (PET).

Treatment Options for Vasculitis

Vasculitis, an inflammation of your blood vessels, is treated based on the specific type you have. If a reason for the inflammation, such as an infection like hepatitis, is identified, it will be treated alongside vasculitis. The treatment depends on the exact diagnosis and how severe or widespread the disease is. Treatment typically consists of three parts: achieving a temporary pause in the disease (remission induction), keeping the disease in control (remission maintenance), and regular check-ups (monitoring).

Glucocorticoids, a type of steroid medication, are the initial treatment for vasculitis patients. These can either be used alone or in combination with other drugs that suppress your immune system. The nature of your vasculitis will determine the type of immunosuppressive medication chosen. Several medications including Methotrexate, Azathioprine, Mycophenolate, and Cyclophosphamide have been used to treat different forms of vasculitis. Once the disease is well-controlled or in remission, doctors gradually lower the dose of the steroids to maintain disease control and limit the risk of side effects from the drug. Both doctors and patients should be aware of the possible temporary and long-term side effects from the medication being used for treatment.

Let’s now discuss the treatment for specific types of vasculitis like GCA (Giant Cell arteritis), GPA (Granulomatosis with Polyangiitis), and MPA (Microscopic Polyangiitis).

For GCA, initial treatment includes steroids typically starting at doses of 40 to 60 mg per day. If you have GCA, you should start the steroids promptly. Steroids delivered directly through a vein (intravenous) can be used in patients who have recently lost vision. After achieving control over the disease, the steroid dose should be gradually decreased. Other drugs like Methotrexate and Azathioprine can be used to reduce the number of steroids needed. A newer drug, Tocilizumab, has been recently approved for treating GCA.

Patients with AAV (ANCA-Associated Vasculitis) have a lesser survival rate if not treated, with a high mortality rate within a year from diagnosis. The current treatment methods aim to get the condition into remission and then to maintain that status. Treatment is adjusted based on the disease’s severity and the organs it is affecting. Treatments include taking Methotrexate or Mycophenolate Mofetil along with glucocorticoids for less severe AAV or administration of Cyclophosphamide or Rituximab with glucocorticoids for more severe cases. The exchange of the plasma (the liquid part of your blood) can be performed in patients with kidney failure or lung bleeding due to vasculitis. Finally, Azathioprine, Mycophenolate Mofetil, or Rituximab along with a gradually reducing course of steroids have been effective in maintaining remission.

What else can Vasculitis be?

When dealing with a potential case of vasculitis, or inflammation of the blood vessels, doctors need to keep in mind that certain other conditions can produce similar symptoms. This means they have to be very thorough in their evaluation of the patient.

This is especially important because treatments for vasculitis usually involve medicines that suppress the immune system. As such, before starting treatment, doctors must ensure the patient does not have an ongoing infection that could worsen.

The following conditions are some that might appear to be vasculitis at first but are in fact different ailments:

• infective endocarditis (an infection of the heart’s inner lining)
• histoplasmosis (an infection caused by a type of fungus)
• gonococcal infection (a sexually transmitted infection)
• antiphospholipid syndrome & thrombotic thrombocytopenic purpura (blood clotting disorders)
• atrial myoma and lymphoma (types of tumors)
• drug toxicity, such as with cocaine or levamisole.

So while the symptoms may initially suggest vasculitis, doctors must be careful to consider these other possibilities through a process of comprehensive testing and evaluation.

What to expect with Vasculitis

The long-term survival rate for patients with vasculitis, a condition causing inflammation in blood vessels, greatly depends on factors such as the timeliness of diagnosis, how well patients respond to treatment, and side effects of medications, which can include infections. A study conducted to determine long-term survival rates in cases of ANCA-associated vasculitis showed that survival rates one, two, and five years after diagnosis were 88%, 85%, and 78% respectively.

In relation to the general population, the mortality ratio for patients with this condition was found to be 2.6 times higher. This data takes into account deaths resulting from the active vasculitis disease itself, and from complications arising from treatment.

Possible Complications When Diagnosed with Vasculitis

The problems that can arise from vasculitis, inflammation of the blood vessels, depend on the size of the blood vessel affected. Large blood vessel vasculitis, as seen in conditions like GCA, Takayasu’s arteritis, or Kawasaki disease, can result in serious conditions like heart attacks and strokes. This includes lack of blood flow to brain arteries like the ophthalmic artery, mesenteric ischemia (lack of blood flow to the intestines), aortic dissection (a tear in the aorta), intramural hematoma (bleeding into the wall of a blood vessel), or severely reduced blood flow to the limbs.

On the other hand, small blood vessel vasculitis can lead to different life-threatening conditions, such as alveolar hemorrhage (bleeding into the lungs), kidney failure, and intestinal ischemia.

Furthermore, the formation of aneurysms, or abnormal bulges in blood vessel walls, is a known complication of GCA, polyarteritis nodosa, and Behcet’s disease. Blood clot formation in the deep veins (deep venous thrombosis) and lungs (pulmonary embolism) occur more often in AAV and Behcet’s than in other types of vasculitis.

Common complications of vasculitis:

• Heart attacks and strokes
• Ischemia of cranial arteries like the ophthalmic artery
• Mesenteric ischemia
• Aortic syndromes (including dissection or intramural hematoma)
• Critical extremity ischemia (severely reduced blood flow to the limbs)
• Alveolar hemorrhage (bleeding into the lungs)
• Kidney failure
• Intestinal ischemia
• Aneurysm formation
• Deep venous thrombosis (blood clot formation in the deep veins)
• Pulmonary embolism (blood clot in the lungs)

Preventing Vasculitis

If you start to notice any signs that could point to a condition called vasculitis, it’s important to talk to your doctor. Vasculitis is inflammation of the blood vessels that can cause them to swell and narrow, cutting off blood supply to your bodies tissues and organs. If you are diagnosed with this condition, you will need to be cared for by a team of healthcare professionals. This team will likely be led by your regular doctor, or primary care physician, and may also include a specialist in diseases of the joints, muscles, and bones, known as a rheumatologist.