Table 1
Clinical and histological features of our present case and cases in the literature
| Sex/age (y) | Histological features | Treatment | Evolution | True lymphocytic intestinal leiomyositis | |
|---|---|---|---|---|---|
| M, male; F, female; NR, not reported; PN, parenteral nutrition; PE, pancreatic enzymes. | |||||
| Present case | F 16 | T lymphocytic infiltrate in muscularis propria | Steroids and later budesonide. Azathioprine | Mild symptoms, oral nutrition 2 y later | Yes |
| Nezelof5 | M 6 mo | Mononuclear infiltrate in muscularis propria | Steroids | Death 4 y later | Yes |
| Ruuska6 | M 2 | Predominant T lymphocytic infiltrate | Steroids, azathioprine, ciclosporin | Total PN | Yes |
| Mann7 | M 47 | Chronic inflammatory infiltrate + fibrosis of longitudinal muscle | NR | Death 2 y later | Probably yes |
| Rigby3 | F 27 | Predominant fibrosis of the circular layer | Immunosuppression | Oral diet plus gastrostomy feeds. Alive at 21 months | Probably no |
| Giniès4 | F 6 mo | Very polymorphic infiltrate: lymphocytes, plasmocytes, histiocytes, and eosinophils | Steroids | Oral nutrition. Normal weight and height | No (probably B lymphocytes) |
| McDonald1 cases 1/2/3/4 | F 51/F 21/ F 29/F 18 | Mucosa predominantly affected | Cyclophosphamide and steroids/steroids/ antibiotics/cisapride | Mild symptoms at 9 y/PN one year later/NR/NR | Probably no (B lymphocytes) |
| Arista-Nasr2 cases 1/2/3 | F 23/F 29/ F 23 | Mucosa predominantly affected | Cyclophosphamide/ tetracycline, tinidazol, PE/tetracycline, steroids, chemotherapy. | Death from inanition/death from inanition/alive, severe inanition | Probably no (B lymphocytes) |
