Table E-1 Patient Characteristics and Summary of Ancillary Testing

Table e-1 Patient characteristics and summary of ancillary testing.

Disease Auto- Age MRC sum- ESR Patient Neuropathy type Gender duration Treatment immune Nerve biopsy (years) score (0-130) (mm/h) (months) parameters Vasculitic syndromes Systemic vasculitis,

1 non-small cell lung M 59 3 100 None 36 -

carcinoma anti-SSA-52, Systemic vasculitis, 2 F 75 11 121 None anti-SSA-60, 71 - M. Sjögren anti-SS-B Systemic vasculitis, 3 F 67 24 126 None anti-SSA/B 82 - M. Sjögren Systemic vasculitis, Prednisone, 4 F 69 58 115 53 - M. Sjögren methotrexate Systemic vasculitis, Focal signs of 5 M 53 1 129 None 129 PAN/MDS vasculitis 6 Systemic vasculitis, GPA F 48 3 130 Prednisone, ANCA-PR3 53 -

Azathioprine,

1 Cyclosfosfamide 7 Systemic vasculitis, GPA F 73 4 122 Prednisone ANCA-MPO 105 - Prednisone,

8 Systemic vasculitis, GPA M 46 3 130 Cyclosfosfamide, ANCA-PR3 46 -

Rituximab Focal signs of 9 Systemic vasculitis, GPA M 65 1 126 None ANCA-PR3 53 vasculitis Prednisone, Systemic vasculitis, 10 F 72 43 102 Azathioprine, ANCA-MPO 123 - microscopic polyangiitis Cyclosfosfamide Systemic vasculitis, Focal signs of 11 M 62 3 118 None ANCA-MPO 98 microscopic polyangiitis vasculitis 12 Non-systemic vasculitis F 33 18 113 IVIG and prednisone None 17 - 13 Non-systemic vasculitis M 65 4 125 None None 7 - Asymmetric

and patchy 14 Non-systemic vasculitis F 43 14 124 None None 12 axonal

degeneration Focal signs of 15 Non-systemic vasculitis M 69 132 130 None None 6 vasculitis

2 Focal signs of 16 Non-systemic vasculitis F 68 3 109 None None 11 vasculitis Disease Auto- Age MRC sum- ESR Patient Neuropathy type Gender duration Treatment immune Nerve biopsy (years) score (0-130) (mm/h) (months) parameters Non-inflammatory axonal neuropathy controls 1 Cryptogenic F 76 118 116 2 Cryptogenic M 47 118 130 3 Cryptogenic F 53 75 129 4 Cryptogenic M 50 60 130 5 Cryptogenic F 61 15 130 6 Cryptogenic F 58 122 130 7 Cryptogenic F 68 20 126 8 Cryptogenic F 57 24 130 9 Cryptogenic F 82 46 130 10 Cryptogenic M 57 7 130 Axonal, vitamin B6 11 F 45 30 130 intoxication Axonal, vitamin 12 12 M 56 48 128 deficiency Axonal, vitamin 12 13 F 59 72 130 deficiency 14 Axonal, diabetic M 40 14 120 15 CMT2 M 56 24 126 16 CMT2 M 66 72 124

3 Table e-2 Nerve size of arm nerves in patients with vasculitic neuropathy and the non-inflammatory axonal

polyneuropathy disease controls.

Patient MN MN MN MN MN MN UN UN Distal UN UN UN UN UN UN

Carpal Carpal Forearm Forearm Upper Upper Distal sulcus Sulcus Sulcus Proxima Proximal Upper Upper

4 tunnel tunnel Right Left arm arm sulcus Left Right Left l sulcus sulcus arm arm

Right Left Right Left Right Right Left Right Left

Vasculitic syndromes Cross-sectional area (CSA) values in mm2 1 12.9 10.9 12.6 10.5 17.6 17.6 8.7 11.4 10.2 13.6 12.5 13.4 14.8 9 2 12.4 15.6 6.4 7.8 8.3 11.1 6.9 8.5 12.8 18 10.5 8.4 7.8 8.4 3 8.5 10.8 6.7 8 10.8 10.8 6.7 5.6 6.9 9.6 9.4 7.1 8.9 6 4 15 15.4 7.9 11 11.4 10.1 9.6 10.5 11.1 10.2 10 9.6 7.7 7 5 11.3 12.7 6.6 8.5 x x 10.5 9.1 9.5 10.2 12.1 18 x x 6 9.3 9.5 4.2 5.2 8.5 7.2 9.2 6.5 7.9 5.6 7.4 7.9 9.6 5.7 7 12.7 9.9 8.7 7.1 8.4 8.9 8.7 9.2 13.4 8.8 8.5 7.5 11.1 8.8 8 6.6 7.2 8.4 8.7 8.5 8.2 5.9 7.9 10 10.5 8.9 8.3 6.8 5.4 9 11.6 12.7 9.6 9.8 10.1 11.2 7.7 7.4 10.9 11.5 8.5 9.9 9.6 6.4 10 8 6.8 7.5 6.3 x x 9.6 5.4 16.5 3.3 5.1 6.7 x x 11 11.1 8.7 7.1 7.7 x x 8.3 11.1 7.9 18.6 7.1 11.8 x x 12 6.4 7.4 6.1 9.8 8.8 11.4 4 4.7 5.1 4.4 5.1 6 6.8 5.8 13 16.8 16.5 12.9 12.8 14.5 13.9 11.7 10.8 15.7 11.9 20.2 11.4 11.2 10.6 14 16.7 15.3 10.1 10 13.9 10.1 6.7 7.9 11.9 11.5 9 8.5 6.3 7.1 15 12.7 16 10.7 9.5 10.5 10.1 15.2 9.8 13.2 10.9 13.5 11.4 7.7 8.3 16 13.8 15.7 9.7 11.2 11.2 12.4 7.2 8.0 11.7 10.1 6.2 6.6 5.7 5.9 Non-inflammatory axonal neuropathy controls 1 6.2 6.9 4.4 4.8 6.5 7.2 5.6 5.9 8.8 16.5 6 8.2 6.3 6 2 11.8 6.9 4.4 4.8 6.5 7.2 5.6 5.9 8.8 16.5 6 8.2 6.3 6 3 10.5 13 6.3 7.3 6.7 9 7 7.2 6.2 7.1 8.1 8.3 7.6 7.5 4 7.9 9.1 6.2 5.4 7.7 6.4 7.1 7.4 6.3 6.2 5.2 5.5 5 5.6 5 8.8 10.5 8.4 8.4 9.1 8.8 9.1 8.7 13.1 8.7 5.7 6.8 7.3 6.9 6 10.8 8 5.2 6.1 6.7 8.9 4.7 5.8 8.9 7.4 7.4 8.2 5.2 7.3 7 13.7 11.6 8.8 9 8.9 9 7.4 8.3 17.5 9.9 11.9 7.8 7.5 8.1

5 8 11.7 12.6 6.3 7.9 6.6 9.2 7.6 9 6.3 8.5 7.7 9.1 5.9 7.2 9 10.9 12.6 6.4 8.7 9.2 8.3 6.4 7.9 11.3 9.8 7.1 8.9 5 7.9 10 14.6 12.3 5.3 8.1 8.5 8.6 6.3 6.9 9.1 11.8 5.9 6.8 6.5 5.5 11 8.8 12.9 8.3 8.6 8.8 6 7.9 7 10.7 10.3 8.6 8 8.4 6.7 12 12.8 9.5 8.4 8.4 8.9 8.1 8.7 8.8 10.6 10.4 8.8 9 5.9 8.6 13 16.4 12.6 6.3 6.3 8.7 9.2 9.4 8.5 7.2 7.3 8.4 8.2 6.5 6.3 14 12 16.9 8.2 8.6 8.9 8.6 6.5 8.6 10.5 12.5 8.9 9.5 6 7 15 11.9 11.8 8.4 7.5 9 8.8 8.8 8.8 10.2 9.5 9 8.9 6 6.9 16 12.1 12.3 5.3 5.2 8.5 6.7 4.6 8.4 8.4 8.2 8.4 7.4 7.3 5.7

Enlarged nerves are indicated by bold type. MN = median nerve, UN = ulnar nerve

Table e-3 Summary of nerve conduction studies and nerves with hypervascularisation in vasculitic syndromes

Nerves with decreased Patient Nerves with decreased SNAP Nerves with hypervascularisation distal CMAP 1 Right ulnar, sural on both sides Right ulnar None Left median, right fibular and 2 Right sural None tibial 3 Left median, ulnar, sural on both sides Left median, ulnar, right None

fibular and tibial on both

6 sides Right fibular, left ulnar, tibial 4 Right median, left sural None and fibular on both sides Right tibial and fibular on Left median nerve at carpal tunnel, left ulnar 5 Sural both sides both sides nerve at sulcus, right fibular nerve at fibular head 6 Right fibular None None Right ulnar, tibial and fibular 7 Right ulnar and sural None on both sides 8 Sural both sides None None Left tibial and fibular on both 9 Left sural, fibular on both sides None sides Right fibular and tibial on 10 Sural both sides Right sural nerve both sides Right median, left tibial, 11 Right fibular, left sural Left ulnar nerve fibular on both sides Median and ulnar on both 12 Median, ulnar and sural on both sides None sides 13 Left sural Tibial, fibular on both sides None 14 Left sural Fibular on both sides None Right tibial, fibular on both 15 Left sural None sides 16 Right median, left sural, fibular on both Right ulnar, fibular and tibial None

7 sides

Any CMAP and SNAP value, below lower limit of normal of the reference values in our clinical neurophysiology lab was classified as decreased.