An Introduction to Vasculitis

• Vasculitis is a health condition first brought into the media spotlight by the death of Ghostbusters star Harold Ramis in 2014. In recent weeks, the well-publicized revelation that Ashton Kutcher battled vasculitis, an autoimmune disorder that affected his sight, hearing, and balance has increased public awareness. 
• Vasculitis is an autoimmune disease that causes inflammation of blood vessels. Most patients develop a localized form of vasculitis, as the more serious systemic response is less common. 
• For clinicians, this presents a wide diagnosis differential. Medications or infections can present as vasculitis, leading to an added complexity in the diagnosis. 

Celebrities who discuss their chronic illness in the public arena bring awareness to rare and lesser-known medical conditions. Vasculitis is a health condition first brought into the media spotlight by the death of Ghostbusters star Harold Ramis in 2014. 

In recent weeks, the well-publicized revelation that Ashton Kutcher battled vasculitis, an autoimmune disorder that affected his sight, hearing, and balance has increased public awareness.  

With vasculitis gaining attention, we can help our patients understand the complex and problematic autoimmune disease. 

The Immune System

Humans thrive and fight off disease through an intricate network of organs, cells and proteins that sense danger from viruses and microbes and kick into gear an apparatus designed to manage the host of internal and external threats to our human physiology.  

Our body’s response to a physiological threat, called an immune response, sometimes entangles healthy cells and tissues, resulting in weakened host defense, chronic inflammation, and autoimmune diseases.

How Vasculitis Comes Into Play

Secondary to a physiological insult, the human body mounts a cell-mediated immune response which causes a reaction in the walls of the veins and arteries that fosters inflammation, restricting the flow of blood. The results of the inflammation can affect small, medium, or large vessels. 

A Case Study

A 24-year-old woman presents to the emergency room with fever, fatigue, weight loss and arthritic aches and pains. Her boyfriend reports she has been confused and complaining of a severe headache. The patient reports a history of arm numbness and double vision. The triage examination showed a notable asymmetric blood pressure discrepancy between the right and left arms. Lab work showed a positive ANA and elevated CRP.  

CT angiography disclosed a focal narrowing of the abdominal and thoracic aorta, along with an occlusion of the subclavian arteries. The nurse checked the blood pressure in her right arm, which was difficult to palpate. The blood pressure in the left arm was 88/52 mm Hg in the right arm. Radial and brachial pulses were impalpable. Her heart rate of 119 beats per minute, with an oral temperature of 38.1°C.  

Her physician comes to a diagnosis of Takayasu’s arteritis an inflammatory large vessel vasculitis more prevalent in young Asian women.  

There are two phases within the disease, a systemic phase, and an occlusive phase, where the affected blood vessels narrow to a point where normal arterial pulsations in the extremities are difficult to palpate. Secondary to the difficulty obtaining peripheral pulses, specialists refer to Takayasu’s arteritis as a pulseless disease. 

A Diagnostic Challenge

Patients with vasculitis, as either a primary or secondary disorder, can present with a breadth of clinical manifestations. Either benign or more serious, forms of vasculitis can threaten vital organs and human life. These symptoms can range from localized cutaneous vasculitis to a more complex multisystem involvement.  

In addition, several medical conditions can mimic vasculitis.  

For the clinicians, this presents a wide diagnosis differential. Medications or infections can present as vasculitis, leading to an added complexity in the diagnosis.

Vasculitis is Inflammation

Vasculitis can affect any blood vessel or organ. As the inflammatory cells infiltrate and the thin cell walls, vascular permeability increases, and the cell walls can rupture. As the vessels narrow, partial or complete occlusion can occur, leading to intimal and intraluminal thrombus. Thrombus or clot formation can lead to organ ischemia or infarction. Vasculitis can lead to an aneurysm, and dangerous internal bleeding. 

Patients with peripheral disease may present with necrotic skin ulcers. With cutaneous involvement, raised non-blanching hemorrhage occurs within the skin. Constitutional symptoms can include fever, night sweats, malaise, weight loss, arthralgia and myalgias.  

Abnormal lab findings can indicate anemia, leukocytosis, thrombocytosis, and elevated erythrocyte sedimentation rate (ESR) and elevated C reactive protein (CRP), both significant markers of inflammation.  

Most patients develop a localized form of vasculitis, as the more serious systemic response is less common. Lung manifestations can include hemorrhage of the pulmonary capillaries within the alveoli of the lungs and patients can present breathlessness with significant hemoptysis. 

Three Primary Types

Vasculitis can occur in the small vessels, capillaries, and venules. Clinical features can include palpable purpura of the extremities and palmar surfaces of the hands and fingers. Necrotic lesions and ulcers, abdominal pain, and gastrointestinal bleeding are present with medium vessel vasculitis. When the aorta and its branches are involved, large vessel vasculitis subsists.

With a diagnosis of large vessel vasculitis, symptoms can include temporal headaches (temporal artery), blindness (ophthalmic artery), and jaw claudication secondary to occlusion of the vessels supplying the muscles of mastication. Similar symptoms of large vessel vasculitis left Ashton Kutcher unable to see, hear, or walk. 

The Bottom Line

There are important questions surrounding the diagnosis of vasculitis.  

Is the patient presenting with a condition that could mimic vasculitis? Is there another underlying cause? How extensive is the disease? What is the form of vasculitis?  

It is important to remember that forms of vasculitis improve without treatment and others need medical intervention. 

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