Table 2.

Comparison of clinical features among AGS, soJIA, and CINCA

Clinical features	Our Patient	Other AGS [2, 3]	soJIA [4–6]	CINCA [7, 8]
Onset within the first year of life	no	common	rare	common
Fever	frequently often	rare	common	common
Preserved or normal intelligence	yes	less common	yes	less common
Mental retardation	no	common	rare	common
Joint swelling	yes	rare	common	common
Arthralgia	yes	rare	common	common
Destructive arthritis	no	rare	common	common
Chilblains	yes	common	NR	rare
Urticarial rash	no	rare	less common	common
Salmon-pink rash	no	rare	common	less common
Conjunctivitis	no	none	less common	common
Visual damage	no	less common	rare	common
Sensor neural deafness	no	rare	NR	common
Progressive chronic meningitis	no	rare	NR	common
Auto-inflammatory manifestations	yes	less common	common	common
Auto-immune manifestations	no	common	rare	none
Severe intra-uterine growth retardation	no	common	NR	rare
Microcephaly	no	common	NR	rare
Psychomotor retarded	not obvious	common	rare	common
Feeding difficulties	no	common	NR	less common
Growth retardation	yes	common	less common	common
Hepatosplenomegaly	no	common	common	common
Cerebral atrophy	milder	common	NR	common
White matter abnormalities	yes	common	NR	less common
Intracranial calcification	yes	common	NR	none
Chronic kidney disease	yes	none	rare	common
Renal amyloidosis	not yet	none	rare	common
Leukocytosis	no	rare	common	common
C-reactive protein	significantly elevated	rare	significantly elevated	significantly elevated
Erythrocyte sedimentation	significantly elevated	less common	significantly elevated	significantly elevated
Ferritin	normal	normal	significantly elevated in MAS	significantly elevated in MAS
Triglycerides	normal	normal	elevated in MAS	elevated in MAS

AGS Aicardi-Goutières, SoJIA Systemic juvenile idiopathic arthritis, CINCA onset chronic infantile neurologic, cutaneous, and arthritis syndrome, NR not reported, MAS macrophage activation syndrome