Patients with vasculitis learn that making the diagnosis is sometimes quite difficult.

Many endure numerous doctors visits, tests, and hospitalizations before the pieces of the puzzle are
assembled. The diagnosis of vasculitis usually requires a biopsy of an involved organ to secure a tissue diagnosis. The two reasons for this are:

# ONE: Vasculitis has many MIMICKERS (other diseases that have similar features but require different treatments).

# TWO: The treatments for vasculitis itself involve substantial risk. No physician should prescribe such treatment without making every effort to secure a firm diagnosis.

Blood tests, Xrays, and other studies may suggest the diagnosis of vasculitis, but often the only way to clinch the diagnosis is to biopsy an involved tissue, examine the tissue under the
microscope in consultation with a pathologist (ideally one experienced at examining biopsies in vasculitis), and find the pathologic hallmarks of the disease. The trick to diagnosing vasculitis is
to figure out the least invasive method of accomplishing this task. If a patients symptoms, physical examination, and diagnostic testing suggest involvement of a particular organ, one of the
procedures below may be used to confirm (or exclude) the diagnosis of vasculitis:

> Skin BiopsyOne of the least invasive ways of making the diagnosis. A minor procedure performed under local anesthesia. The wound is closed with 12 stitches that are removed 710 days
later. Pictured belowis an abnormal skin biopsy showing leukocytoclastic vasculitis. The white oval shapes are subcutaneous fat cells beneath the dermis.

Pictured below is an example of an inadequate skin biopsy.

The correct diagnosis of PAN (polyarteritis nodosa) was not confirmed by this biopsy because the biopsy was not deep enough. The biopsy specimen contains only the epidermis and
superficial dermis. PAN classically affects mediumsized arteries located in the deep dermis.

In contrast to the biopsy above, the skin biopsy below was deep enough to include the deep dermis as well as some subcutaneous fat.
The white, ovalshaped areas are fat lobules. Just superficial to the subcutaneous fat, within the deep dermis, an inflamed mediumsized vessel is evident. A closer view of the vessel is
provided in the next figure which provides a high power view of the vasculitic artery lying at the junction of the dermis and subcutaneous fat.

> Kidney BiopsyPerformed if there is evidence of kidney involvement by vasculitis (red blood cells or protein in the urine, for example). Done under local anesthesia while the kidney is
visualized by ultrasound. Because of the small but significant risk of bleeding after this procedure, patients are usually monitored in the hospital for 24 hours after the biopsy. The biopsy below
shows a crescent in a glomerulus.
> Sural Nerve BiopsyThe sural nerve is a sensory nerve over the lateral aspect of the foot. Under local anesthesia in an operating room, a surgeon removes a small piece of the nerve, usually
along with a piece of the adjacent muscle (the gastrocnemius). Because the sural nerve does not innervate muscles (remember: it is a sensory nerve, not a motor nerve), the patient does not
lose any strength on the side of the foot and lower leg. There maybe, however, some residual numbness on the side of the foot. Patients generally tolerate this numbness well (if the vasculitis
has involved the nerve severely enough, some patients already have numbness in that region). Below is the surgical site of a sural nerve and gastrocnemius muscle biopsy one week after the
procedure: a few sutures and a thin, wellhealing scar.

> Temporal Artery BiopsyPerformed to diagnose Giant Cell Arteritis, also known as Temporal Arteritis, because the temporal artery is often involved. The temporal artery courses up the
temples, just in front of the ears. The biopsy, done under local anesthesia, is performed by making a small incision just above the hairline (sometimes shaving a small area of hair is required).
The procedure is extremely welltolerated by patients. Within several weeks, there is usually little or no sign that a biopsy was done. Complications of temporal artery biopsies are extremely
rare. Sometimes, to increase the diagnostic yield, both temporal arteries (i.e., the ones on each side of the head) are biopsied.

> Lung Biopsy Often the best way to make a diagnosis of vasculitis that involves the lungs, such as Wegeners Granulomatosis. May be performed in one of two ways: 1) open lung biopsy, a
sizeable surgical procedure; or 2) thoracoscopic lung biopsy, a less invasive but still significant procedure. Even a thoracoscopic biopsy usually requires at least 48 hours in the hospital and the
temporary placement of a chest tube to permit the lung to reexpand.

> Brain BiopsyOften necessary to confirm the diagnosis of Central Nervous System (CNS) Vasculitis. Usually performed on the nondominant side of the patients brain (that is, if the patient is
righthanded and therefore leftbrained the biopsy is performed on the right side of the brain). Biopsy of the brains covering, the meninges, is usually performed at the same time.

> Abdominal AngiogramHelpful in the diagnosis of Polyarteritis Nodosa (PAN). Similar to a heart catheterization. After inserting a catheter into a large artery in the leg and advancing the
catheter into the aorta, radiographic dye is injected into blood vessels supplying the gastrointestinal tract. In the proper clinical setting, the detection of aneurysms (small outpouchings of blood
vessel walls) is diagnostic of PAN.
 > Central nervous system angiogram Frequently part of the workup of CNS vasculitis. The procedure is identical to an abdominal angiogram, except the catheter is advanced all the way
up to the large vessels supplying the head and neck (for example, the carotid arteries). On angiography, CNS vasculitis is characterized by beading (dilated areas alternating with narrowing of
the blood vessels). A strikingly abnormal angiogram may eliminate the need for a brain biopsy. The angiogram pictured below shows prominent dilations of arteries visible at several sites in the
intracerebral region.

> Other Useful TestsThere are many other tests that are helpful in the diagnosis of vasculitis, or in evaluating the activity of the disease:

Erythrocyte sedimentation rate (ESR)

Creactive protein (CRP)
Urinalysis
CT Scan
ANCA tests
Erythrocyte sedimentation rate (ESR) Also known as the sed rate, for short. This is an old but useful test first employed by the ancient Greeks as a test for pregnancy (the ESR is elevated
not only in inflammatory conditions, but also in pregnancy).

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Creactive protein (CRP) A modern day version of the ESR, more expensive but not necessarily superior to the ESR in the diagnosis and management of vasculitis. CRP is a protein
produced by the liver in response to inflammation within the body.

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Urinalysis Many forms of vasculitis affect the kidneys. A simple way of determining whether or not the kidneys are involved is to perform a urinalysis. By performing checks for several
indicators of inflammation in a patients urine, the physician may determine if inflammation is present within the kidneys. These indicators include:

Protein (proteinuria)
Red blood cells (hematuria)
Clumps of red blood cells (casts)
Pictured below is a urine specimen from a patient with Wegeners granulomatosis and glomerulonephritis (inflammation in the kidneys). This is a view of the specimen examined under the
microscope, showing cylindrical casts comprised of red blood cells. This finding strongly indicates vasculitis in the kidney.
From another Wegeners granulomatosis patients urinalysis, blebs (identified by white arrows) protrude from the surface of the red blood cells that have been damaged in transit through the
kidney. Because inflamed kidneys leak blood, red blood cells dismorphic as these are appear in the urine. (pictured below)

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CT Scan (a CAT scan, or computed tomography) A type of radiology test that permits a non-invasive, crosssectional view of a patients anatomy. On the illustration below (a chest CT scan
from a patient with Wegeners granulomatosis), the view is up (looking toward the patients head, from his or her feet). The heart is the white, rounded object in the upper center of the picture.
The black regions are the patients lungs. The large spot in the left lung (corresponding to the patients right lung) is a nodule caused by Wegeners. Other smaller nodules are also evident.

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ANCA tests ANCA is an abbreviation (acronym) for antineutrophil cytoplasmic antibodies. These antibodies are found in the blood of patients with several different types of vasculitis,
including Wegeners Granulomatosis, Microscopic Polyangiitis, and the ChurgStrauss Syndrome. ANCAs and their association with vasculitis were recognized in the mid1980s, and their use
has become increasingly widespread since the 1990s. ANCAs are detected by a simple blood test. These antibodies are directed against the cytoplasm (the nonnucleus part) of white blood
cells. Their precise role in the disease process remains uncertain but is a topic of considerable research interest. ANCAs come in two primary forms: 1) the CANCA [C stands for cytoplasmic]
and, 2) the PANCA [P stands for perinuclear]. CANCAs have a particularly strong connection to Wegeners Granulomatosis (up to 80% of patients and possibly more of those with active
disease have these antibodies). When CANCAs are present in the blood of a patient with symptoms or signs suggesting Wegeners, the likelihood of the diagnosis increases considerably.
Because of the long list of other conditions that are sometimes associated with ANCAs, however, in most cases it is still VERY IMPORTANT to biopsy an organ involved by vasculitis to verify
the diagnosis.

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