Rheumatology [VASCULITIS]
Vasculitides are a bit peculiar. They’re often a diagnosis of
exclusion as they’re considered only after other diseases have
been ruled out. Let’s focus on the few test associations for classic
presentations.
LARGE VESSEL VASCULITIS
Giant Cell Arteritis
Also called temporal arteritis, this is the most common primary
vasculitis. It effects the arteries of the external carotid (the
temporal artery and ophthalmologic artery). The classic
presentation is age > 50 person with unilateral temporal
headache and a tender swollen temporal artery. Another
pattern could be visual changes or jaw claudication. This is an
intensely inflammatory disorder, so systemic symptoms may be
present (elevated ESR, weight loss, low-grade fever, and
malaise). GIVE STEROIDS FIRST. Even though the diagnosis
is definitely made with a biopsy, the risk for blindness is too
great; treatment should precede the diagnostic step.
Polymyalgia Rheumatica
While this is actually not a vasculitis it’s so commonly associated
with Giant Cell Arteritis that it’s discussed here. PMR presents
with symmetric pain and stiffness in the shoulder, neck, and
hips. It’ll present as a proximal muscle weakness similar in
pattern to the idiopathic inflammatory myopathies but with a
normal CK (the ESR and systemic signs of inflammation are
present). This disease requires no diagnostic step, though an
angiogram could rule out other diseases. It responds well to
steroids.
Takayasu Arteritis
This disease is super rare and has the same pathology as GCA,
but instead of the distal arteries it impacts the vessels of the aorta
and its major branches (subclavian, renal, femoral). There are
systemic signs (↑ESR, weight loss, fever), but what you’re keying
in on is the absent pulses in large vessels (brachial, femora,
carotid). Look for a young person (< 40 years old) who has
systemic inflammation and large vessel impairment. An
angiogram will make the diagnosis. Treat with high dose
prednisone
SMALL VESSEL – ANCA RELATED
Granulomatosis with Polyangiitis (Wegner’s)
Most common ANCA associated vasculitis. GPA is usually c-
ANCA positive. Look for renal + lung + nasal. This is usually
seen on the test as hematuria and hemoptysis but also with
nasal involvement. A biopsy of affected tissue is required for
treatment; it will show vasculitis and necrotizing granulomas.
Treatment is aggressive: steroids and cyclophosphamide. Other
DMARDs can be used as maintenance therapy.
MEDIUM VESSEL VASCULITIS
Polyarteritis Nodosa
PAN is associated with Hepatitis B but can exist on its own. It’s
tough to diagnose because it affects so many organs. When
vasculitis in multiple organs at the same time without a
common vascular distribution is seen, think PAN. In the
kidneys it causes a non-glomuerlonephritis vasculitis (renal
failure). In the gut is causes mesenteriC vasculitis (mesenteric
ischemia). In the nervous system it causes an asymmetric painful
motor and sensory neuropathy (mononeuritis multiplex). In the
skin it causes purpura and painful nodules. What will probably
happen is that you will go hunting for ONE problem and do some
vascular imaging like an angiogram. That angiogram will reveal
aneurysms and stenosis in medium-sized vessels. Treatment is
aggressive. Start with high-dose steroids and
cyclophosphamide. Treat Hep B.
Kawasaki Disease
This is as Asian Childhood Disease (look for infants and
children, most commonly Asian boys). The buzz phrase is
strawberry-like tongue. Also look for the trunCal rash with a
paLmar and plantar erythema with desquamation. This can
cause vasculitis of the coronary arteries leading to myocardial
infarction in a child. Treatment is with IVIG and Aspirin (even
though you’re not supposed to give kids aspirin).
SMALL VESSEL – COMPLEX MEDIATED
Cryoglobulinemia
Think of cryoglobulinemia when there’s palpable purpura and
Hepatitis C. Assess for cryoglobulins in the serum. Other clues
may be a positive RF, elevated ESR, and decreased compliment.
Treatment can be with steroids, cyclophosphamide, rituximab,
and plasmapheresis (in increasing order of severity).
Henoch–Schönlein purpura
HSP is suspected when there’s palpable purpura and
abdominal pain or abdominal bleeding. The kidneys can
be involved. The diagnosis is made by biopsying the
effected tissue (usually the skin) to reveal a leukocytoclastic
vasculitis. For treatment, steroids are the main option.
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