Vasculitis

Vasculitides – Trever Stevens and Greg Jackson

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

 

 

Takayasu's Arteritis

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

 

 

Giant Cell Arteritis

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

 

 

Polyarteritis Nodosa (PAN)

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

 

 

Granulomatosis with Polyangiitis (GPA)

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)

 

Management

  • If treating inpatient, has 20x increase risk of DVT/PE – ensure DVT ppx
  • Mild to moderate disease: MTX + glucocorticoids
  • Severe disease: rituximab or cyclophosphamide, then maintenance w/ Rituxan or DMARD

 

Microscopic Polyangiitis (MPA)

Presentation

  • Similar to GPA but will only involve kidneys and lungs (less sinus involvement)
  • Hemoptysis with bilateral nodular lung involvement and hematuria/proteinuria common

 

Evaluation

  • ANCA +, typically MPO-pANCA
  • U/A with urine microscopy: need to spin the urine to evaluate for active renal disease
  • Biopsy of a site of suspected diseased activity
     

Management

  • DVT ppx is important as patients are at higher risk for DVTs/PE
  • Mild to moderate disease: MTX (or similar DMARD) + glucocorticoids
  • Severe disease: rituximab or cyclophosphamide, then maintenance w/Rituxan or DMARD

 

Eosinophilic Granulomatosis with Polyangiitis (EGPA or Churg-Strauss Syndrome)

Presentation

  • Similar to MPA and GPA; unique symptoms include atopic symptoms (asthma, etc.)
    • Asthma is the most common symptom with involvement in over 90% of patients
  • Can have Peripheral eosinophilia
  • More likely small vessel vasculitis to cause cardiac tissue involvement: Coronary arteritis, myocarditis, heart failure and arrhythmias
  • Skin lesions: tender subcutaneous nodules
  • Renal involvement: hematuria and proteinuria
     

Evaluation

  • ANCA + (typically MPO-pANCA) and peripheral eosinophilia
  • Histology: Eosinophilic infiltrates with fibrinoid necrosis
     

Management

  • DVT prophylaxis as patients are high risk for DVTs/PE
  • Mild to moderate disease: MTX (or similar DMARD) + glucocorticoids
  • Severe disease (cardiac or neurologic involvement): cyclophosphamide induction with maintenance DMARD, Rituximab