Table 1.

Clinical and Laboratory Manifestations in Patients with Deficiency of Adenosine Deaminase 2.

Clinical Manifestation	Patients
	no./total no.
Fever	9/9
Ischemic stroke	8/9
Hemorrhagic stroke	3/9
Ophthalmologic involvement*	5/9
Livedo racemosa	8/9
Hepatosplenomegaly	6/7
Documented vasculitis†	4/9
Polyarteritis nodosa	2/9
Antinuclear antibody	3/9
Antineutrophil cytoplasmic antibody	0/9
Low serum IgM	5/5

^*The ophthalmologic manifestations included central retinal artery occlusion in one patient, optic nerve atrophy in one, diplopia with irregular enhancement of the medial rectus muscle (as observed on magnetic resonance imaging) in one, third cranial nerve palsy in one, and strabismus in two. Patients could have more than one ophthalmologic disorder.

^†The diagnosis of vasculitis included polyarteritis nodosa.