Cryptogenic multifocal ulcerous stenosing enteritis: Radiologic features and clinical behavior

Table 1 Clinical characteristics, diagnostic tools, and treatment outcomes of patients with cryptogenic multifocal ulcerous stenosing enteritis

Patient No./sex/age (yr)	Duration until hospital visit (yr)1	Presenting symptom at admission	Hb (g/dL)	SBS	APCT/CTE/MRE	DE/CE	Treatment	Treatment response (No. of relapse)	Relapse-free period (yr)	No of surgery	Follow up period (yr)2
1/M/23	9.6	Abdominal pain	13.3	1	3/-/-	1/-	Surgery	Relapse (1)	1.1	1	2
2/F/56	22.9	Abdominal pain	11.4	ND	1/-/-	-/-	Steroid, 5-ASA, surgery	Remission		1	3
3/M/49	10.4	Abdominal pain	13.7	ND	1/1/-	-/1	Surgery	Remission		1	1.5
4/F/61	25	Anemia	10.5	1	1/-/-	-/-	Surgery	Remission		1	2.9
5/F/29	1	Anemia	7.4	2	6/1/-	1/1	Steroid, 5-ASA, surgery	Relapse (1)	1	1	9.9
6/F/37	8.6	Anemia	8	2	1/-/-	-/-	Steroid, 5-ASA, surgery, others (albumin)	Relapse (2)	2.7, 6.4	1	13.4
7/F/45	7.5	Anemia, GI bleeding	7.6	3	-/3/-	2/-	Steroid, surgery	Relapse (2)	0.5, 3.7	1	9.4
8/F/49	5.1	Abdominal pain	12.9	ND	-/2/1	2/-	Steroid, surgery	Relapse (3)	1.4, 6, 1.7	3	9.8
9/M/54	3.5	Abdominal pain, anemia	10.4	1	-/1/-	-/-	Surgery	Remission		1	2.5
10/M/30	4.8	Abdominal pain	8.5	5	1/4/-	-/1	5-ASA, surgery	Relapse (2)	7.9, 0.7	2	10
11/F/16	14.6	Abdominal pain	7.7	ND	-/1/-	-/-	Surgery	Remission		1	0.5
12/M/24	0.9	Abdominal pain, anemia, GI bleeding	11.3	1	-/2/-	-/-	Surgery, others	Relapse (1)	2.5	1	3.6
							(conservative treatment)
13/M/41	12	Abdominal pain	9	1	-/1/-	1 /-	Steroid	Remission		0	0
14/F/64	23	Abdominal pain	12.3	ND	-/-/1	-/-	Surgery	Remission		1	0
15/F/39	10.3	Abdominal pain	9	1	-/3/-	-/-	5-ASA, surgery	Relapse (1)	2	1	6.2
16/M/60	10	Anemia, GI bleeding	9.1	ND	-/1/-	-/1	Steroid, 5-ASA	Remission		0	1.1
17/F/15	4	Abdominal pain, anemia	7.7	4	1/-/-	-/-	Steroid, 5-ASA, surgery	Relapse (3)	3, 4, 4, 3	4	13.5
18/F/34	0.1	Anemia	7.6	1	1/1/-	1/-	Steroid, tuberculosis drug	Remission		0	10.6
19/M/33	1.2	Anemia	6.1	1	1/-/-	1/1	Steroid	Remission		0	4.5
20/F/48	0.2	GI bleeding	13.4	1	1/-/-	-/-	Surgery	Remission		1	4.5

¹Time interval between the symptom onset and hospital visit (yr);

²Time interval between prior remission and recurrence (yr). SBS: Small bowel series; APCT: Abdominopelvic CT; CTE: CT enterography; MRE: MR enterography; DE: Double-balloon endoscopy; CE: Capsule endoscopy; ND: Not done; 5-ASA: 5-aminosalicylic acid; GI: Gastrointestinal.

Table 2 Radiologic features on computed tomography/magnetic resonance Imaging of patients with cryptogenic multifocal ulcerous stenosing enteritis (n = 20)

Stricture	n = 52
Total No. (mean No. per patient, range)	52 (2.6, 1-6)
Location (Jejunum/Ileum)	5/47
Stricture length (mm), mean ± SD	10.44 ± 3.95
Stricture thickness (mm), mean ± SD	5.56 ± 1.58
Enhancement pattern (layered/transmural)	48/4
Degree of enhancement (mild/moderate/strong)	8/39/5
Interval between strictures (cm), median (range)	4 (1.5-20)
Segmental bowel wall thickening	n = 4
Location (Jejunum/Ileum/both)	0/3/1
Wall thickening length (cm), mean ± SD	36.25 ± 13.77
Wall thickening thickness (mm), mean ± SD	7.75 ± 1.26
Wall thickening pattern (circumferential/eccentric)	2/2
Enhancement pattern (layered/transmural)	3/1
Degree of enhancement (mild/moderate/strong)	1/2/1
Small bowel obstruction
Absent/low grade/high grade/complete	13/5/2/0
Mesenteric manifestation
Mesenteric lymphadenopathy	6
Mesenteric hypervascularity or infiltration	4

Table 3 Radiologic features on small bowel series of patients with cryptogenic multifocal ulcerous stenosing enteritis (n = 14)

Stricture	n = 36
Total No. (mean No. per patient, range)	36 (1.8, 1-5)
Location (Jejunum/Ileum)	1/35
Stricture length (mm), mean ± SD	9.81 ± 4.65
Interval between strictures (cm), median (range)	4.5 (1.5-20.4)
Ulcer	n = 10
Total No. (mean No. per patient, range)	10 (0.5, 1-2)
Location (Jejunum/Ileum)	1/9
Ulcer length (mm), mean ± SD	11.3 ± 5.58
Ulcer pattern (eccentric/circumferential/linear/ aphthous)	9/1/0
Small bowel obstruction
Absent/low grade/high grade/complete	7/5/2/0