Table 1.

Clinical characteristics of the adults with AAHS^*

	Total (n = 116)†	Underlying SLE (n = 61)	Underlying AOSD (n = 31)	P‡
Age, mean ± SD (range) years	41.6 ± 18.7 (16–86)	34.4 ± 14.2	43.4 ± 17.4	0.014
Male:female ratio	1:3	1:4.1	1:4.2
Physical findings
Fever, no./total no. (%)§	99/114 (86.8)	56/60 (93.3)	30/31 (96.8)	NS
Lymphadenopathy, no./total no. (%)	32/78 (41)	16/40 (40)	13/24 (54.2)	NS
Hepatomegaly, no./total no. (%)	28/67 (41.8)	12/29 (41.3)	10/20 (50)	NS
Splenomegaly, no./total no. (%)	40/88 (45.5)	15/43 (34.9)	16/25 (64)	NS
Laboratory findings
WBC count, mean ± SD (range) ×109/liter	4.1 ± 6.08 (0.1–41.1)	2.2 ± 1.2	6.8 ± 6.6	0.002
Hgb, mean ± SD (range) gm/dl	8.9 ± 2 (4.8–14.1)	8.7 ± 1.8	9.6 ± 1.9	NS
Platelet count, mean ± SD (range) ×109/liter	98.1 ± 96.8 (2–560)	81.9 ± 52.2	141.9 ± 142	NS
Coagulopathy, no./total no. (%)¶	40/79 (50.6)	24/35 (68.6)	12/27 (44.4)	NS
AST, mean ± SD (range) IU/liter	264.2 ± 548 (9–3,420)	211.3 ± 341	423.4 ± 757.9	NS
ALT, mean ± SD (range) IU/liter	183.6 ± 351 (6–2,126)	96.1 ± 117	315.9 ± 402	0.029
LDH, mean ± SD (range) IU/liter	1,251 ± 1,592 (115–10,586)	1,012 ± 974	1,923.1 ± 1,721	NS
CRP, mean ± SD (range) mg/dl	7.7 ± 10.2 (0.2–39.3)	2.8 ± 4.0	14.9 ± 12.6	0.00002
Ferritin, mean ± SD (range) μg/liter	15,334.1 ± 35,181 (16–210,000)	5,849.5 ± 10,472	40,136.4 ± 64,069.7	0.003

^*NS = not significant; WBC = white blood cell; Hgb = hemoglobin; AST = aspartate aminotransferase; ALT = alanine aminotransferase; LDH = lactate dehydrogenase; CRP = C-reactive protein.

^†Includes the 61 patients with underlying systemic lupus erythematosus (SLE), the 31 with underlying adult-onset Still's disease (AOSD), and the 24 with other autoimmune diseases underlying autoimmune-associated hemophagocytic syndrome (AAHS).

^‡Underlying SLE versus underlying AOSD.

^§Body temperature ≥37.5°C.

^¶Hypofibrinogenemia, increased fibrinogen degradation products, or prolonged prothrombin time or activated partial thromboplastin time.