7 Rheumatology Vasculitis

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 cANCA 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 high-dose steroids aggressive. Start with 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 reve al a leukocytoclastic vasculitis . For treatment, steroids are the main option.

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