Table 4

irVasculitis

Subtypes:
(Median 8, range 6–9)
Possible symptoms:
(Median 8, range 7–9)
Supportive examination findings:
(Median 8, range 7–9)
Diagnostic criteria: All levels of diagnostic certainty for irVasculitis require a supportive history, examination, and timing relative to ICI therapy plus:
(Accuracy median 7.5, range 6–9; usability median 8, range 5–9)
  • Primary angiitis of the CNS (specify small, medium or large vessel involvement, if possible)

  • Systemic vasculitis with CNS involvement (specify systemic subtype and small, medium or large vessel involvement, if possible)

  • Headache

  • Stroke with focal neurologic symptoms

  • Seizure

  • Symptoms of encephalitis, myelitis, or meningitis

  • Systemic symptoms of vasculitis including rash

  • Focal neurologic deficits

  • Signs of irEncephalitis and irMyelitis

  • Systemic signs of vasculitis including rash

Definite
Required:
  1. Symptoms and signs consistent with CNS vasculitis AND

  2. Biopsy proven CNS vasculitis


Supportive:
  1. Vascular abnormality consistent with vasculitis on CTA, MRA, conventional angiogram

  2. Parenchymal abnormality on MRI brain or spinal cord

  3. Inflammation on CSF studies (lymphocytic pleocytosis, elevated protein)

  4. Presence of CSF-specific oligoclonal bands and/or elevated IgG index

  5. Evidence of systemic vasculitis including biopsy, serum markers and/or imaging findings

Evaluation may include:
(Median 8, range 7–9)
Common:
  1. MRI brain with contrast (including post-contrast vessel wall studies if available)

  2. MRA head and neck or CTA head and neck

  3. Lumbar puncture with CSF studies including those for irEncephalitis (especially VZV testing) and syphilis testing (if serum testing positive)

  4. Carotid Doppler, EKG, heart rhythm monitoring, echocardiogram

  5. CRP, ESR

  6. Serum syphilis testing

  7. ANCA, ANA and other serologic markers associated with systemic vasculitis depending on context (can consider rheumatology evaluation for assistance)

Probable
Required:
  1. Symptoms and signs consistent with CNS vasculitis AND At least one of the following

  2. Biopsy demonstrating inflammation surrounding blood vessels (but no leukocytes within blood vessel wall) OR

  3. CNS vascular imaging (MRA, CTA or conventional angiogram) demonstrating vasculopathy consistent with vasculitis (eg, vessel narrowing or beading) OR

  4. MRI with concentric contrast enhancement of blood vessel wall


Supportive:
  1. Other parenchymal abnormality on MRI of brain or spinal cord

  2. Inflammation on CSF studies (lymphocytic pleocytosis, elevated protein)

  3. Presence of CSF-specific oligoclonal bands and/or elevated IgG index

  4. Evidence of systemic vasculitis including biopsy, serum markers and/or imaging findings

Possible:
  1. Formal rheumatology and/or dermatology evaluation

  2. Brain biopsy

  3. Extracranial biopsy (eg, temporal artery biopsy, skin biopsy)

  4. Retinal fluorescein angiography

  5. Conventional (digital subtraction) angiogram

  6. CTA, MRA or conventional angiogram of other vascular beds (eg, splanchnic, renal)

  7. Antiphospholipid antibody panel, serum hypercoagulability evaluation (eg, factor V Leiden, etc)

Possible
Required:
  1. Symptoms and signs consistent with CNS vasculitis AND

  2. Cerebral infarct, especially multiple CNS infarcts crossing vascular territories and no evidence for alternative cause such as thromboembolic disease)


Supportive:
  1. Other parenchymal abnormality on MRI of brain or spinal cord

  2. Inflammation on CSF studies (lymphocytic pleocytosis, elevated protein)

  3. Presence of CSF-specific oligoclonal bands and/or elevated IgG index

  4. Evidence of systemic vasculitis including biopsy, serum markers and/or imaging findings

Uncommon:
1.Body PET imaging
  • ANA, anti-nuclear antibody; ANCA, anti-neutrophil cytoplasmic antibody; CNS, central nervous system; CRP, c reactive protein; CSF, cerebrospinal fluid; CTA, CT angiogram; EKG, electrocardiogram; ESR, Erythrocyte sedimentation rate; MRA, MR angiogram; PET, positron emission tomography.