Vasculitis

Background

• Large vessel vasculitides - affect the Aorta and its major branches:
  • Takayasu's Arteritis: most commonly affects <50 years old and young Asian Females
    • Branches of the aorta and aortic arch (subclavian, innominate, and carotid arteries)
  • Giant Cell: most commonly affects adults >50 years old
    • Almost never seen under the age of 50; and affects F>M slightly
    • Cranial arteries like the temporal artery, although can involve aorta and its branches
• Medium vessel vasculitides - affect the muscular arteries that supply visceral organs:
  • PAN:  Necrotizing vasculitis, involves muscular arteries including the renal arteries, mesenteric artery, and arteries that supply neurons and the skin
    • PAN spares the lung (consider other process if pulmonary complaints)
    • Affects middle age to older adults (peaks at 5th decade of life)
    • Can be associated with HBV infection and HBsAg
  • Kawasaki’s: most commonly affects children (not addressed here)
• Small Vessel Vasculitides - affect arterioles, capillaries, and venules
  • Cryoglobulinemic vasculitis and Henoch Schonlein Purpura - not discussed here
  • GPA: Necrotizing and granulomatous; involves the nasopharynx, lungs, and kidneys
  • MPA: Necrotizing; affects multiple organs, predilection for lungs and kidneys
    • Nasopharyngeal involvement and granulomas are absent (contrast to GPA)
  • EGPA: Granulomatous necrotizing inflammation with eosinophilia
    • Uncommon over the age of 65; predilection for heart and lungs

Presentation

• Unequal pulses, arterial bruits, or different BP in both arms (known as “pulseless disease”)
• Constitutional symptoms: fevers, arthralgias, myalgias, rash, weight loss
• Can also cause carotidynia (tenderness of carotid artery), lightheadedness, vertigo, headaches, syncope, and strokes due to involvement of carotid arteries

Evaluation

• Inflammatory markers: ESR & CRP (usually elevated) 
• Arteriography: MRA or CTA of head/neck, chest, and abdomen

Management

• If signs of aortitis/carotidynia or arterial stenosis: 1mg/kg prednisone daily (max 60-80mg/kg) for 2-4 weeks followed by steroid taper
• Organ threatening disease or ischemia: 500-1000 mg IV methylprednisolone (1-3 days) then 1mg/kg prednisone daily (2-4 weeks) followed by steroid taper
• MTX, immunomodulators

Presentation

• In a pt >65 with headache and vision changes (diplopia or amaurosis fugax) this needs to be ruled out!
• Classically presents as headache, jaw claudication, tenderness to palpation of scalp in addition to constitutional symptoms
• Polymyalgia rheumatica will often accompany GCA
  • B/l shoulder and hip pain and stiffness (elevated ESR but normal CK)
     

Evaluation

• Order ESR, CRP, CK level, TSH
• Detailed vascular exam for (4 limb blood pressures with attention to symmetry, abdominal/neck/chest auscultation for bruit)
• Ophthalmology eval if there is ocular involvement.
• Temporal artery biopsy – evaluating for evidence of vasculitis or giant cells
  • (Vascular surgery can assist with timely biopsy)
     

Management

• If ESR is elevated in a moderate to high probability setting, give steroids without delay (biopsy results will be unaffected if done within ~2 weeks)
• Without visual loss: prednisone 1mg/kg (max of 60 mg) daily
• With visual loss or diplopia: 100-1000mg of IV methylprednisolone daily for 3 days, followed by prednisone 1mg/kg daily (max of 60mg)  
• Duration: daily high-dose steroids as above for 2-4 weeks, then begin to taper steroids

Presentation

• Constitutional symptoms: fevers, arthralgias, myalgias, rash, weight loss
• Asymmetric polyneuropathy (most common symptom) & both motor and sensory deficits
• Foot drop, radial and ulnar neuropathies
• Hypertension if renal artery involvement
• Abdominal pain and melena if mesenteric artery inflammation
• Skin manifestations: livedo reticularis, palpable purpura, or tender erythematous nodules

Evaluation

• ESR, CRP, CK level, TSH
• Arteriography: MRI or CT or catheter based angiogram - “string of pearls” appearance 
• Histology: fibrinoid necrosis and vasculitis in target tissue
   

Management

• Hepatitis screening if not previously done to evaluate for HBsAg
• Mild disease or isolated cutaneous disease: Glucocorticoids
• Moderate disease: Up to 1mg/kg prednisone daily (up to 60-80mg max) for 2-4 weeks followed by steroid taper +/- DMARD
• Severe disease or life threatening disease (evidence of renal insufficiency, significant proteinuria, gastrointestinal, cardiac, or neurologic involvement): 500-1000mg IV methylprednisolone daily for up to 3 doses followed by prednisone as above for 4 weeks with taper plus cyclophosphamide

Presentation

• Sinus and respiratory symptoms, sinusitis/ulcers, hemoptysis
• Renal involvement – elevated Cr, hematuria, or proteinuria from RPGN
• Less commonly affects eyes (scleritis: red, painful, light sensitive), skin (palpable purpura), peripheral nerves (mononeuritis multiplex)

Evaluation

• ANCA + (Usually PR3-cANCA > MPO-pANCA)
• U/A with urine microscopy: need to spin the urine to evaluate for active renal disease
• Biopsy of a site of suspected disease activity (necrotizing granulomatous vasculitis)