What are the classification criteria for medium-vessel vasculitides?

Updated: Dec 10, 2018
  • Author: Nadia Jennifer Chiara Luca, MD; Chief Editor: Lawrence K Jung, MD  more...
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Answer

The most common childhood medium-vessel vasculitis is Kawasaki disease. It is a necrotizing vasculitis that has a predilection for the coronary arteries. Classic features include prolonged fever, mucocutaneous changes and lymphadenopathy. Other manifestations may include irritability, arthritis, and abdominal pain.

Classification criteria for Kawasaki disease include a fever persisting for at least five days (mandatory criterion) plus 4 of the following 5 features:

  • Changes in peripheral extremities or perineal area

  • Polymorphous exanthema

  • Bilateral conjunctival injection

  • Changes of lips and oral cavity (injection of oral and pharyngeal mucosa)

  • Cervical lymphadenopathy

Childhood polyarteritis nodosa is a necrotizing vasculitis of medium-sized arteries and is recognized in distinct systemic and cutaneous forms.

Systemic polyarteritis nodosa involves all organ systems and the presentation widely varies. [4] Features include vasculitic skin lesions, hypertension, neuropathy, and myalgia. Note that renal involvement does not manifest as glomerulonephritis, as occurs with small-vessel disease. Unlike in adults, angiographic or biopsy evidence of vasculitis is required to make this diagnosis.

Classification criteria for childhood polyarteritis nodosa include a systemic illness characterized by the presence of either a biopsy finding that reveals small and mid-size artery necrotizing vasculitis or angiographic abnormalities (aneurysms or occlusions), plus at least 2 of the following:

  • Skin involvement (eg, livedo reticularis, tender subcutaneous nodules, other vasculitic lesions)

  • Myalgia or muscle tenderness

  • Systemic hypertension, relative to childhood normative data

  • Mononeuropathy or polyneuropathy

  • Abnormal urine analysis and/or impaired renal function (glomerular filtration rate of < 50% normal for age)

  • Testicular pain or tenderness

  • Signs or symptoms suggesting vasculitis of any other major organ system (GI, cardiac, pulmonary, CNS)

Cutaneous polyarteritis nodosa is characterized by the presence of subcutaneous nodular, painful, nonpurpuric lesions with or without livedo reticularis and absence of systemic involvement. However, more than half of patients also have myalgia, arthralgia, and nonerosive arthritis. Cutaneous polyarteritis nodosa has been associated with serological or microbiological evidence of streptococcal infection in 40% of patients. [4]

Childhood PACNS is defined by clinical evidence of a newly-acquired focal or diffuse neurologic deficit plus angiographic or histologic evidence of CNS vasculitis, in the absence of a systemic condition associated with these findings. Two clinically and radiologically distinct types of childhood PACNS are noted: large-medium vessel (angiography-positive) and small vessel (angiography-negative). These have different clinical presentations (see Primary CNS Vasculitis of Childhood).


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