Table 1 Differential diagnosis of inflammatory and lymphoproliferative disorders.
| Age | AD | ED | CD | VG | GSE | RS | EITCL | IPSID | CD4 TLPD | FA/CMSE | AE |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Variable | Children/adults | Variable | Variable | Children/adults | Adults | Adults | Young adults | Adults | Children | Children/adults | |
| HLA DQ2/DQ8 | − | − | − | Unrelated | + | + | + | − | − | − | +/− |
| Autoantibodies | − | − | − | − | − | +/− | − | − | − | Enterocytes or goblet cells | |
| EMA, tTGA | − | − | − | Anti‐gliadin ab | + | +/− | − | − | Absent | +/− | |
| GFD | − | − | − | Independent | Responsive | Unresponsive | Unresponsive | Unresponsive | Unresponsive | Unresponsive | Unresponsive |
| Distribution of the lesions | Duodenal Bulb | Variable | Focal | Diffuse | Diffuse/patchy | Diffuse | Diffuse | Diffuse | Diffuse | Patchy, most prominent on the duodenal bulb, LNH | Diffuse |
| Architecture distortion | Minimal | Variable | Normal | Variable | Variable | Variable | Variable | Variable | Minimal | Normal/minimal | Variable |
| Villi | GDM/blunted/atrophic | Variable | Normal/blunted | VA | Normal/VA | VA | VA | Present | Slightly blunted | Absent | Blunted/VA, Goblet cell depletion |
| Crypts | Normal | Normal | Normal | Increased mitosis | CH | Variable CH | Normal | Normal | Normal/ | ||
| IELs | Normal | Normal | Normal | Increased CD3+/ CD4+ or CD3+/ CD8+ in a variable admixture | Increased CD3+, CD4−, CD8−, and TCRγδ+ | Increased, morphologically normal cCD3ε+ TCR−, CD3−, CD4−, or CD8− | CD3+, CD4− CD8− or CD3+, CD4−, CD8+, CD56+, +/−CD30+ | Centrocyte‐like lymphocytes with lymphoepithelial lesions | Normal count | CD3+, γδ+ and αβ+ IELs increased less than in GSE | Normal hyperplastic |
| Neutrophils | Diffuse | Variable | Focal acute inflammation flanked by almost normal appearing mucosa | No | Acute phase | No | No | No | No | No | No |
| Lamina propria | Neutrophils, increased non‐specific mononuclear cells | Eosinophils in clusters, neutrophils, plasma cells, lymphocytes | Neutrophils, non‐specific mononuclear infiltrate | Non‐specific mononuclear | Non‐specific mononuclear infiltrate | Diffuse and dense mononuclear infiltrate of mainly plasma cells | Neoplastic lymphocytes, non‐specific mononuclear infiltrate | Dysmorphic plasma cells CD20− and centrocyte‐like lymphocytes CD20+, cytoplasmic Ig αheavy chain+ | Dense and diffuse, abnormal pleomorphic lymphocytes and variably in the submucosa, CD2+, CD3+, CD5+, CD7+ and CD4 clonal TCRβ rearrangement present | Non‐specific mononuclear infiltrate | CD4+, CD8+ lymphocytes |
| Eosinophils | +/− | In clusters, infiltrating crypts and the surface epithelium, eosinophilic microabscess | +/− | Normal | Present | Normal | Normal | Normal | Present | Present | Normal |
| Granulomas | − | − | +/− | − | − | − | − | − | +/− | − | − |
| Epithelioid Microaggregates | − | − | +/− | − | − | − | − | − | − | − | − |
| DD | CD GSE | FA/CMSE, GSE | GSE | GSE | FA/CMSE, AE, CD, EITCL | GSE, EITCL, AE | GSE, RS, AE | GSE, EITCL | GSE | GSE | GSE |
AD, acute duodenitis; AE, autoimmune enteropathy; cCD3ε, intracitoplasmic CD3; CD, Crohn's disease; CD4 TLPD, CD4 T cell lymphoproliferative disorders; CH, crypt hyperplasia; DD, differential diagnosis; ED, eosinophilic duodenitis; EITCL, enteropathy‐type intestinal T cell lymphoma; EMA, endomysial antibodies; FA/CMSE, food allergy/cow's milk protein‐sensitive enteropathy; GDM, gastric duodenal metaplasia; GFD, gluten‐free diet; GSE, gluten‐sensitive enteropathy; IELs, intraepithelial lymphocytes; IPSID, immunoproliferative small intestinal disease; LNH, lymphonodular hyperplasia; RS, refractory sprue; tTGA, Tissue T ransglutaminase antibody; VA, villous atrophy; VG, viral gastroenteritis.
+, present; −, absent; +/−, variably present.