Vasculitis¶

Hannah Angle

Background¶

Large vessel vasculitis (aorta and its major branches)
- Takayasu arteritis:
  - Most commonly <30 years old, 80-90% cases in females, greatest prevalence in Asia
  - Branches of aorta/aortic arch (subclavian, innominate, carotid arteries)
- Giant cell Arteritis:
  - Most commonly >50 years old, slight F > M predominance
  - Cranial arteries (temporal artery), aorta and its branches
Medium vessel vasculitis (muscular arteries that supply visceral organs)
- Polyarteritis nodosa (PAN):
  - Most often middle age/older adults (peaks in 5^th decade of life)
  - Necrotizing vasculitis of muscular arteries (renal, mesenteric, arteries supplying neurons and skin)
- Kawasaki’s: not addressed here- most often children
Small Vessel Vasculitis (arterioles, capillaries, and venules)
- ANCA-associated vasculitis:
  - GPA: granulomatous; classically involves nasopharynx, lungs, kidneys
  - MPA: affects multiple organs but predilection for lungs and kidneys; in contrast to GPA, no nasopharynx involvement or granulomas
  - EGPA: granulomatous; predilection for heart and lungs; associated with eosinophilia, chronic rhinosinusitis, asthma; uncommon > 65 years old
Immune-complex vasculitis: not addressed here
- Cryoglobulinemic vasculitis (see lab section)
- IgA vasculitis
- Anti-GBM disease

Subacute constitutional symptoms (fevers, arthralgias, myalgias, rash, weight loss), limb claudication, neuro symptoms from carotid/vertebral arteritis (vertigo, headaches, syncope, strokes), GI symptoms from mesenteric ischemia, angina from coronary arteritis
HTN (renal artery involvement), discrepant BP between arms (arterial stenosis), diminished or absent pulses (“pulseless disease”), arterial bruits, carotidynia (tenderness of carotid artery)

New arterial stenosis or aorta/carotid artery involvement: 1mg/kg prednisone daily (max 60-80mg) for 2-4 weeks followed by steroid taper
Organ threatening disease (coronary artery involvement, critical stenosis of carotid/vertebral arteries): 500-1000mg IV methylprednisolone daily for 1-3 days, then 1mg/kg prednisone daily for 2-4 weeks followed by steroid taper

Always consider in pts >50 years old with new headache, abrupt vision changes (diplopia, amaurosis fugax), jaw claudication, temporal artery tenderness
Polymyalgia rheumatica will often accompany GCA; classically with bilateral shoulder and hip pain/stiffness (with elevated ESR, but normal CK)

ESR/CRP (almost always elevated), CK, TSH
Evaluate for any temporal artery abnormalities (tenderness to palpation, presence of nodules)
Ophthalmology evaluation if any concern for ocular involvement
Temporal artery biopsy by vascular surgery

Start glucocorticoids as soon as GCA is suspected, do not delay while awaiting biopsy
- No vision loss: prednisone 1mg/kg daily (max 60 mg) for 2-4 > weeks followed by steroid taper
- Vision loss/diplopia: 500-1000mg IV methylprednisolone daily for > 3 days, followed by prednisone 1mg/kg daily (max 60mg) for 2-4 > weeks followed by steroid taper

Constitutional symptoms: fatigue, weakness, fevers, arthralgias, myalgias, rash, weight loss
Evidence of multisystem involvement:
- Asymmetric polyneuropathy with motor and sensory deficits (foot > drop, radial/ulnar neuropathy)
- Skin manifestations: livedo reticularis, palpable purpura, > ulcers, tender erythematous nodules, bullae, vesicles
- HTN (renal arteritis)
- Abdominal pain and melena (mesenteric arteritis)
- Ischemic cardiomyopathy (coronary arteritis)
- Testicular pain (orchitis)
Classically spares the lungs (consider other dx if pulmonary complaints)

ESR/CRP, CK, TSH
Hepatitis panel (HBV/HCV can cause secondary PAN)
Arteriography: MRI, CT, or angiogram with classic “string of pearls” appearance
Biopsy: segmental transmural inflammation of muscular arteries, fibrinoid necrosis of arterial wall (no granulomas, presence suggests another process)

Mild disease (ex: isolated cutaneous disease): prednisone 1mg/kg daily (max 60-80mg) for 4 weeks followed by steroid taper
Moderate disease: cyclophosphamide + prednisone 1mg/kg daily (max 60-80mg) for 4 weeks followed by steroid taper
Severe/life-threatening disease (renal failure, significant proteinuria, GI/cardiac/neurologic involvement): cyclophosphamide + 500-1000mg IV methylprednisolone daily for 3 days, followed by prednisone 1mg/kg daily (max 60mg) for 4 weeks followed by steroid taper

Constitutional symptoms (fevers, fatigue, weight loss) + evidence of glomerulonephritis (hematuria, proteinuria), upper respiratory tract involvement (nasal crusting, rhinorrhea, epistaxis, sinusitis), and pulmonary involvement (dyspnea, cough, hemoptysis)
Less commonly affects eyes (scleritis), skin (palpable purpura), and peripheral nerves (mononeuritis multiplex)

ANCA + (typically PR3-cANCA)
ESR/CRP, ANA, anti-GBM, C3/C4, cryoglobulins, HBV/HCV, HIV
UA with microscopy (hematuria, proteinuria, RBC casts, dysmorphic RBCs)
CT chest if pulmonary symptoms
Biopsy: necrotizing granulomatous vasculitis, pauci-immune glomerulonephritis
Clinical Pearl: If you suspect renal disease based (elevated Cr, hematuria) ask renal fellow or rheum fellow to help spin the urine to evaluate for RBC casting or dysmorphic cells. Quick way to confirm active GN, renal biopsy takes time to arrange

Mild-moderate disease: MTX + prednisone 0.5 mg/kg/day followed by steroid taper
Severe disease: rituximab or cyclophosphamide + prednisone 1mg/kg/day (60-80mg max)
- For pts with RPGN, pulmonary hemorrhage, mononeuritis multiplex, > or optic neuritis: IV methylprednisolone 7-15mg/kg/day (1000mg > max) x 3 days for induction therapy
DVT ppx (high risk for DVT/PE)

Similar to GPA, but without upper respiratory tract involvement; classically only involves lungs and kidneys

Similar to GPA/MPA + atopic symptoms such as asthma (>90%), rhinosinusitis, and peripheral eosinophilia
Cardiac involvement (accounts for 50% deaths from EGPA): coronary arteritis, myocarditis, heart failure, arrhythmias
Skin involvement (>50%): tender subcutaneous nodules

ANCA + (typically MPO-pANCA), peripheral eosinophilia
IgE, ANA, RF, C3/C4
Biopsy: necrotizing granulomatous vasculitis, eosinophilic infiltrates with fibrinoid necrosis, pauci-immune glomerulonephritis

Mild-moderate disease: prednisone 0.5-1 mg/kg/day for 6-12 weeks followed by steroid taper
Severe disease: cyclophosphamide + prednisone 0.5-1 mg/kg/day for 6-12 weeks followed by steroid taper
- For pts with life-threatening multiorgan involvement (cardiac, > pulmonary, renal, neurologic): IV methylprednisolone 1000mg > daily x 3 days for induction therapy
DVT ppx (high risk for DVT/PE)

Last update: 2022-06-27 23:26:14