|
| Study | Age of onset/gender | Presentation | Treatment | Follow-up time | Outcome |
| Disease extent |
|
| Leiby et al. [3] | 2 y/M
Gastroduodenocolitis | Watery diarrhea (2 mo) | IV steroids for 2 weeks, followed by PPI, mesalamine, PO steroids for 8 wks (response, but failed wean), bismuth subsalicylate (response) | 14 wk | 2 wk: Clinical resolution, biopsies with less inflammation but increased collagen band persisted. Relapsed 2 weeks after discontinuing systemic steroids. Then bismuth subsalicylate for 4 weeks with resolution of symptoms. |
| Emesis (1 mo) |
| Low-grade fevers (1 mo) |
| Hypoalbuminemia (3.1 g/dL) |
| Elevated ESR |
|
| Billiémaz et al. [2] | 9 mo/M
Gastro-duodeno-ileocolitis | Watery diarrhea
Intermittent emesis and diarrhea since age 2 weeks
Poor weight gain
Hypoalbuminemia (1.4 g/dL)
Hypogammaglobulinemia
Lymphocytosis | 13 yrs of prednisone 0.5–1 mg/kg/d, alternating with budesonide 3-4 mg/d, overnight enteral nutrition, gluten-free diet (persistent symptoms), TPN (full clinical improvement) | 14 y | 3 y: Gastric pseudopolyps with inflammatory areas in the gastric body, areas of mucosal erythema in the colon, histopathology not available |
| 10 y: Thick small tubular stomach, diffuse atrophic (extending into duodenum), erythema and multiple small submucosal nodules. Colon pale, thickened and nodular with disappearance of normal vascular pattern. Histology with atrophic mucosa and increase in the subepithelial collagen layer to 12–100 µm. |
| 14 y: Asymptomatic on TPN and off steroids within 2 mo. |
|
| Almadhoun et al. [4] | 15 mo/F
Gastro-duodeno-ileocolitis | Diarrhea (4 wk) | Budesonide 3 mg for 5 days (no response)
TPN, gut rest
IV methylprednisolone 2 mg/kg (response)
PO prednisone 1 mg/kg/dose with 2 mo steroid taper and oral budesonide 3 mg (remained asymptomatic)
Symptom recurrence after weaning of budesonide, requiring reinduction with oral steroid, maintained on oral prednisone and budesonide 3 mg | 1 y | 5 mo (on budesonide): Persistent collagenous colitis without gastric or duodenal involvement. |
| Peripheral edema (2 wk) | 1 yr (on budesonide): Normal biopsies |
| Emesis (1 wk) |
| Hypoalbuminemia (2.4 g/dL) |
| Stool alpha-1 antitrypsin >1.33 mg/g [<0.62] |
|
| Current case | 2.5 y/F
Gastro-duodeno-ileocolitis | Diarrhea (10 wk) | IV methylprednisolone for 2 weeks, followed by PO steroids (response)
Symptom recurrence after tapering of PO steroids, unable to maintain on budesonide.
Reinduction with IV steroids and maintenance on methotrexate (response) | 4 y | 2 mo: Asymptomatic and normal pathology (on steroids) |
| Emesis (1 wk) |
| Low-grade fevers |
| Hypoalbuminemia (1.5 g/dL) |
| Hypogammaglobulinemia |
| 9 mo: Asymptomatic and normal pathology (after 6 mo of MTX) |
| 17 mo: Recurrence of collagenous colitis, good response and symptom resolution on oral budesonide |
| 4 y: Asymptomatic off treatment |
|
