Endomysial antibodies predict celiac disease irrespective of the titers or clinical presentation

Table 1 Demographic data on the study participants and primary reason for celiac disease suspicion n (%)

Female	265 (67)
Age below 18 yr	92 (23)
Age (yr), median (range)	40 (1-79)
Main reason for disease suspicion
Gastrointestinal symptoms1	166 (43)
Anemia or malabsorption	38 (10)
Extraintestinal symptoms2	50 (13)
Screening in at-risk groups3	97 (24)
Screening in the population4	39 (10)
Unknown	5 (1)

¹Diarrhea, abdominal pain, flatulence, constipation, dyspepsia, and heartburn;

²Osteoporosis, infertility, aphthous stomatitis, short stature, delayed puberty, arthralgia, ataxia, epilepsy, fatigue, and alopecia;

³Family history of celiac disease, type 1 diabetes, thyroid disorders, Sjögren´s syndrome, and immunoglobulin A nephropathy;

⁴Population-based research studies that included serological screening.

Table 2 Serum endomysial and transglutaminase 2 antibody values, divided according to the clinical presentation

	EmA, titer		TG2-ab, U/L
	Low 1:5-200	High 1:≥500	Low 5.0-29.9	High≥30
	n = 224, %	n = 154, %	n = 116, %	n = 166, %
Abdominal symptoms	45	40	45	45
Anemia or malabsorption	8	12	5	13
Extraintestinal symptoms	9	16	5	13
Screen-detected subjects	38	31	45	28
	P = 0.061		P = 0.002

EmA: Endomysial; TG2-ab: Transglutaminase 2 antibody.

Table 3 Association between high and low serum endomysial and transglutaminase 2 antibody values and small-bowel mucosal morphology

	EmA (L)		TG2-ab(U/L)
	Low 1:5-200	High 1:≥500	Low 5.0-29.9	High≥30
	n = 227, %	n = 156, %	n = 146, %	n = 169, %
Marsh 0	5	1	4	1
MarshI-II	16	5	16	5
Marsh IIIa	22	13	24	12
Marsh IIIb	28	29	31	28
Marsh IIIc	29	52	25	53
	P < 0.001		P < 0.001

EmA: Endomysial; TG2-ab: Transglutaminase 2 antibody.

Table 4 Association between endomysial antibody titers and small-bowel mucosal damage

EmA titer	Subjects	Marsh 0-II, n (%)	Marsh III, n (%)
1: ≥ 5	372	57 (15)	315 (85)
1: ≥ 50	323	39 (12)	284 (88)
1: ≥ 100	282	26 (9)	256 (91)
1: ≥ 200	243	22 (9)	221 (91)
1: ≥ 500	155	9 (6)	146 (94)
1: ≥ 1000	96	3 (3)	93 (97)
1: ≥ 2000	48	2 (4)	46 (96)
1:4000	20	0 (0)	20 (100)

EmA: Endomysial.

Table 5 Baseline and follow-up data on subjects with positive endomysial antibodies but normal small-bowel mucosal villous structure

	Low1 EmA and TG2-ab, n = 40	High EmA or TG2-ab, n = 17
Baseline
Age, median (range), yr	39 (5-68)	39 (6-70)
Females, n (%)	30 (75)	11 (65)
Age below 18 yr	7 (18)	7 (41)
Gastrointestinal symptoms	28 (70)	12 (71)
Extraintestinal symptoms	2 (5)	4 (23)
Screen-detected subjects	10 (25)	1 (6)
EmA, median (range), titer	1:50 (1:5-1:200)	1:500 (1:5-1:2000)
TG2-ab, median (range), U/L	6.3 (0-24.8)	45.5 (13.9-> 100)
HLA DQ2 or DQ8, n (%)	33/33 (100)	16/16 (100)
Marsh 0	9 (23)	3 (18)
MarshI-II	31 (77)	14 (82)
Follow-up
Celiac disease diagnosis	29 (73)	16 (94)
Villous atrophy later2	12 (30)	8 (47)
Positive response to GFD	17 (43)	8 (47)
Gluten, no villous atrophy	2 (5)	0
Lost to follow-up	9 (22)	1 (6)

¹EmA titer 1: < 500, TG2-ab value < 30.0 U/L;

²Up to 10 yr of follow-up. EmA: Endomysial; TG2-ab: Transglutaminase 2 antibody; HLA: Human leukocyte antigen; GFD: Gluten-free diet.