Mesenteric Vasculitis

What is Mesenteric Vasculitis?

Vasculitis is a condition where the walls of the blood vessels in different parts of the body become inflamed. This inflammation can happen in large, medium, and small arteries, the tiny blood vessels known as capillaries, and in veins. When this inflammation takes place in the blood vessels of the gastrointestinal tract (digestive system), it’s called mesenteric vasculitis. Generally, this condition tends to show up alongside vasculitis in other parts of the body, but sometimes, it can occur on its own. We’ll explore the various disorders that can result in mesenteric vasculitis, what symptoms they cause, how they’re diagnosed, and how they’re treated.

What Causes Mesenteric Vasculitis?

Vasculitis, which is an inflammation of blood vessels, is usually categorized by the size of the affected vessels. However, it’s important to note that there’s often a crossover in terms of which vessels become inflamed. This means that several types of vasculitis can affect the mesentery, the tissue that keeps our intestines in place. It’s a bit like a support or bridge in our stomachs, linking everything together.

Risk Factors and Frequency for Mesenteric Vasculitis

There isn’t much information available specifically about the spread of mesenteric vasculitis. However, there are facts about different types of vasculitis and diseases that can affect the mesentery, which is the part of the body associated with this condition.

• Polyarteritis nodosa (PAN) mostly affects adults between 35 to 60 years. It’s a little more common in men. There are two types of PAN, one with a known cause (hepatitis B virus), and one where the cause is unknown. Cases of PAN caused by hepatitis B are going down due to better prevention and treatment strategies against hepatitis B. A specific type of PAN caused by a gene mutation, called DADA2 PAN, has also been identified.
• Another type of vasculitis, called Anti-neutrophilic cytoplasmic antibody (ANCA) associated vasculitis, is more common in men and usually starts when people are between 65 and 74 years old.
• Immunoglobulin-A (IgA) vasculitis is the most common type in children, especially between the ages of 4 and 6. It’s slightly more common in boys and in the Asian population.
• Takayasu arteritis is seen worldwide but may be more common in Asia. It’s mostly found in women (over 80% of cases) and usually starts when people are between 10 and 40 years old.
• Behcet disease is more common in the Mediterranean and eastern Asian populations, and usually affects people between the ages of 20 to 40. There is no gender preference.
• Kawasaki disease mainly occurs in young children, particularly those under two years, of east Asian origin.
• Systemic lupus erythematosus is more common in females, with a ratio of up to 12 females for every male, especially in middle-aged adults. More than half of patients are diagnosed between the ages of 16 to 55.
• Rheumatoid arthritis is also more common in women and usually diagnosed in older adults, often after age 50.

Signs and Symptoms of Mesenteric Vasculitis

Vasculitis is a condition that can lead to a range of abdominal symptoms. It’s important to think about vasculitis if someone’s medical history, physical examination, or test results point towards it. Many of the symptoms are caused by inadequate blood supply to the intestines, which can result in tissue death. One type of this condition, called mesenteric vasculitis, can cause similar symptoms as a similar disease caused by hardening of the arteries. Common signs of this condition are stomach ache, sensitivity to touch in the abdomen, and spotting blood in stools.

The condition can also cause symptoms like nausea, vomiting, and diarrhea. If the sudden blockage of a blood vessel causes mesenteric ischemia, the symptoms might include sudden severe stomach pain that does not match the physical examination findings and lower gastrointestinal bleeding. In some cases where the blood supply to the intestines has slowly decreased, it can result in chronic stomach pain after eating and weight loss.

For some people, severe complications like dying intestinal tissue or a hole in the intestine can happen. This can cause serious changes in body functions and mental state.

Testing for Mesenteric Vasculitis

If your doctor suspects you may have an issue with your blood vessels causing your digestive system to not get enough blood, such as mesenteric ischemia from vasculitis, they might request a range of tests. This range could include a basic metabolic panel to make sure your body chemistry is balanced, a complete blood count to check the different components in your blood, a liver function test to make sure your liver is working well, and a urinalysis to check for signs of disease in your urine.

Your doctor may also order tests for inflammatory markers like erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) which can tell how much inflammation is in your body. Though these markers aren’t specific to mesenteric ischemia, they can still provide useful information. For some patients, more specific tests like viral hepatitis serology (which checks for signs of hepatitis), serum cryoglobulins (that can tell if certain abnormal proteins are in your blood), ANCA, antinuclear antibody (ANA), and rheumatoid factor (all tests for certain types of autoimmune diseases) may be required.

If your doctor thinks you may be dealing with bowel ischemia (a lack of blood supply to the intestines that can lead to tissue damage), they’ll check your lactic acid levels. If your doctor suspects you have a genetic condition called DADA2, which can cause inflammation in the body including the blood vessels, they might recommend genetic testing and a test to check the functioning of an enzyme called ADA2.

Your doctor might also suggest imaging tests like a Computerized Tomography (CT) scan with intravenous contrast, which can help visualize the blood flow in your organs. However, if you’re unable to have a CT scan with contrast, another imaging method called magnetic resonance angiography can be used instead.

Endoscopy, which uses a flexible tube with a light and camera attached to it to visually inspect your digestive tract, should be done carefully in patients with gastrointestinal vasculitis. This is because the lack of blood flow can cause the wall of the digestive tract to tear during the procedure. The findings from an endoscopy can be non-specific, meaning they might suggest multiple possible conditions, but they can help rule out some other diagnoses. For example, the tissue lining the digestive tract could appear pale, swollen, and damaged. In severe cases, there could be bleeding and ulcers. In long-standing cases, there could be a shrinking of the tissue lining the digestive tract and strictures, or narrowed areas. The microscopic analysis of a small tissue sample, or histopathology, usually shows damage to the tissue lining the digestive tract and different levels of tissue hardening or scarring known as fibrosis.

Treatment Options for Mesenteric Vasculitis

Vasculitis is a condition where the body’s immune system attacks the blood vessels causing inflammation. Sometimes, this inflammation can affect the gastrointestinal, or digestive, tract. The treatment process is dual-faceted: it manages the inflammation in the blood vessels and addresses any specific issues in the digestive system.

Usually, mainstream medical treatment for vasculitis comprises using medications like glucocorticoids and immunosuppressive drugs such as cyclophosphamide, azathioprine, and mycophenolate mofetil. Biological agents like rituximab and TNF alpha inhibitors can also be beneficial depending on the specific type of vasculitis.

When vasculitis causes a lack of blood flow, or ischemia, in the intestines, immediate medical attention is needed if there are complications like tissue death or rupture. A surgical operation to remove the affected section of the intestine can save lives. In cases of chronic bowel ischemia, early intervention can lessen the risk of complications. A CT angiography scan can aid in identifying any blockage or narrowing in the mesenteric circulation that supplies the intestines. If there is a blockage, it can be reopened using angioplasty, possibly with a stent, to restore blood flow.

Below are some different types of vasculitis, each with unique treatments.

Polyarteritis Nodosa (PAN) is an inflammation type that primarily affects medium and small-sized arteries. PAN symptoms are versatile and based on the number of arteries involved. Common signs include abdominal pain and other gastrointestinal symptoms. When patients with PAN show up with worsened gastrointestinal issues, critical imaging evidence like bowel wall thickening or organ rupture can increase suspicion. Tests are necessary to screen for viral infections like Hepatitis B, C, and HIV which are usually linked with this condition. Treatment usually involves immune-suppressant drugs and sometimes antiviral therapy along with short-term corticosteroids, especially for patients with Hepatitis B.

ANCA Vasculitis is a group of three types of inflammation of blood vessels usually affecting small arteries, capillaries, and venules. These types can affect the eye, ear, nervous system, and, rarely, gastrointestinal system. Normally, more than 80% of patients with two types of this vasculitis have ANCA. Treatment typically involves cyclophosphamide and glucocorticoids, followed by some kind of immune-suppressant therapy.

IgA Vasculitis, also known as Henoch-Schonlein purpura (HSP), usually affects children. Key features consist of a rash, blood in urine, and joint pain. Often, there is a preceding respiratory infection. Prednisone has been shown to alleviate stomach pain in these patients, but it’s unclear whether it can alter the disease course.

In cases of vasculitis restricted to the gastrointestinal tract, treatment depends on the organ involved, with gallbladder cases typically having good results from laparoscopic removal. For example, Takayasu Arteritis, a form of vasculitis of the aorta and its branches, is ideally treated with glucocorticoids and immune-suppressant drugs.

Behcet Disease, which often involves recurrent painful ulcers, has been treated successfully with glucocorticoids, azathioprine, and infliximab. Patients with Systemic Lupus Erythematosus (SLE) presenting with gastrointestinal symptoms, due to vasculitis like Lupus mesenteric vasculitis (LMV), requires immediate treatment with high doses of intravenous cortisone.

Vasculitis can happen in patients with longstanding and severe rheumatoid arthritis. This can cause ulcers or bowel ischemia, an inadequate supply of blood to the bowel. Medical treatment involves therapy for the underlying disease with glucocorticoids, other immune-suppressing agents, and biological agents.

Vasculitis affecting the intestines also has been seen infrequently in other systemic vasculitis like giant cell arteritis, relapsing polychondritis, Kawasaki disease, and cryoglobulinemic vasculitis.

What else can Mesenteric Vasculitis be?

Diagnosing similar health conditions requires a thorough medical history and physical examination. Sometimes, different diseases can show the same symptoms, making the diagnosis a bit tricky. Common conditions with similar symptoms to what is being tested for include:

• Hardening of the arteries in the gut (atherosclerotic mesenteric ischemia)
• Infections
• Cancer (malignancies)
• Side effects of medication
• Other conditions that block blood vessels (occlusive processes)

For patients already known to have vasculitis (inflammation of blood vessels), the first step is to rule out other conditions. After that, the treatment for vasculitis can be focused on. However, diagnosing vasculitis in patients who don’t already have a known diagnosis can be more challenging. If a patient shows symptoms that indicate involvement of other organs or symptoms of bowel blood supply problems (ischemia) without having risks associated with hardened arteries (atherosclerosis), doctors should start looking into the possibility of vasculitis in the gut (mesenteric vasculitis).

What to expect with Mesenteric Vasculitis

The chances of recovering from mesenteric vasculitis, a condition affecting the blood vessels in the abdomen, largely depend on how severe the disease is and how much it has spread at the time of diagnosis. The outlook also depends on the nature of any underlying medical conditions and whether other organs are affected.

Unfortunately, in most patients, the disease affects the mesentery (tissues that connect and support your intestines) in the later stages. If not treated, these patients tend to have a very poor outlook.

For this reason, spotting the condition early and starting treatment as soon as possible is crucial. This can greatly improve the patient’s quality of life and chances of survival.

Possible Complications When Diagnosed with Mesenteric Vasculitis

Having blood vessel inflammation in the abdominal region can cause varying levels of decreased blood flow to the intestines, which can end up damaging them. This typically happens because of the formation of a clot in the main artery supplying the intestines. Other less frequent but serious complications may include significant bleeding in the digestive tract, bowel blockage resulting from narrowed areas, and a condition where a section of the intestine folds into itself due to swelling in the inner lining of the small intestine. If a clot blocks blood flow in the abdominal circulation, it can result in sudden reduced blood supply to the intestines.

There are less frequent but potentially life-threatening complications, such as damage to a part of the intestine, a hole in the bowel wall, inflammation of the inner abdominal wall, and a severe infection from bacteria moving out of the gut. In some cases, reduced blood supply to the liver due to inflammation of its blood vessels may not cause any symptoms, and will usually appear as abnormal results on liver function tests.

Main complications:

• Decreased blood flow to intestines
• Blockage of the main artery to the intestines
• Significant bleeding in the digestive tract
• Bowel blockage due to narrow areas
• Intestine folding into itself
• Sudden reduced blood supply to intestines
• Potential fatal outcomes:
• Damage to intestine
• Hole in the bowel wall
• Inflammation of the inner abdominal wall
• Severe infection from bacteria
• Abnormal liver function test results due to reduced blood supply to the liver

Preventing Mesenteric Vasculitis

Since mesenteric vasculitis – a condition where the blood vessels in the tissue that surrounds your intestines get inflamed – and vasculitis in general are relatively rare, complex, and present in different ways, doctors have a responsibility to explain the disease well to those affected, and their families. This can be done through one-on-one meetings, informative pamphlets, or even group discussions. Besides, it’s essential to fully understand your medication and any potential side effects. Doctors should thoroughly discuss this with the patients to make sure they are taking it correctly and safely.