Table 2 Characteristics of reported AIE in literature
Clinical features | All cases* | Results |
|---|---|---|
Age (years) at Dx | 129 | 49.0 (IQR: 36.5~60.5) |
Gender | 145 | Female/Male = 82/63 = 1.3/1 |
Comorbidities | 178 | CVID/hypogammaglobulinemia/agammaglobulinemia in 48 cases (27%) |
Diarrhea duration (months) | 78 | 6.0 (IQR: 2.9 ~ 24.0) |
Weight loss (kg) | 48 | 16.0 (IQR: 10.0 ~ 25.8) |
Stool frequency (/day) | 40 | ≥ 10+/day accounts for 83% (33/40) |
Stool volume (/day) | 27 | 1000 ~ 10,000 ml |
Character of stool | 77 | Mostly watery, without blood or mucus, sometimes steatorrhea or protein-losing. |
D-xylose test | 20 | (+) in 19 cases (95%) |
Fasting test | 23 | (–) in 19 cases (83%) |
Gluten-free diet | 95 | Ineffectiveness in 82 cases (86%) |
HLA-DQ2/8 | 90 | (+) in 45 cases (50%) |
AE antibody | 96 | (+) in 50 cases (52%) |
AG antibody | 68 | (+) in 9 cases (13%) |
Celiac antibody profiles | 136 | (–) in 111 cases (82%), tTG + in 11 cases (8%), AGA + in 9 cases (7%), tTG and AGA + in 4 cases (3%), DGP + in 1 case (1%) |
Villous atrophy | 170 | Reported in 150 cases (88%) |
IEL | 149 | IEL in 76 cases (51%), minimal IEL in 73 cases (49%) |
Increased apoptotic bodies | 122 | Reported in 60 cases (49%) |
Decreased or absent goblet cells | 107 | Reported in 55 cases (51%) |
Decreased or absent Paneth cells | 107 | Reported in 35 cases (33%) |
Neutrophil infiltration | 107 | Reported in 60 cases (47%) |
Corticosteroids therapy | 167 | Used in 148 cases (89%) |
Immunosuppressant therapy | 155 | Used in 63 cases (41%) |
Biological agent therapy | 160 | Used in 51 cases (32%) |
Follow-up outcomes | 164 | Death in 23 cases (14%) |