Baseline characteristics of the patients
| Characteristic . | Patient A . | Patient B . | Patient C . | Patient D . | Patient E . | Patient F . | Patient G . |
|---|---|---|---|---|---|---|---|
| Age, y/sex | 62/M | 70/M | 65/F | 63/M | 64/F | 59/F | 51/M |
| Age at CD diagnosis, y | 56 | 62 | 61 | 48 | 44 | 47 | 50 |
| Age at RCD II diagnosis, y | 59 | 64 | 63 | 63 | 56 | 58 | 51 |
| Age at ASCT, y | 60 | 68 | 64 | 63 | 64 | 59 | 51 |
| Date of ASCT | Mar 2004 | Aug 2004 | May 2005 | Aug 2005 | Nov 2005 | Dec 2005 | Mar 2006 |
| HLA-DQ2 | Homozygous | Homozygous | Heterozygous | Homozygous | Heterozygous | Heterozygous | Homozygous |
| Marsh at RCD diagnosis | III-A | III-B | III-A | III-A | III-C | III-C | III-A |
| BMI, kg/m2 | 19.4 | 18.9 | 17.1 | 24.1 | 20.1 | 21.3 | 20.5 |
| Performance | 1 | 1 | 1 | 1 | 1 | 1 | 2 |
| Symptoms/associations | Diarrhea, pain, weight loss | Pain, diarrhea | Diarrhea, weight loss, hypocalcemia | Diarrhea, weight loss, dermatitis, herpetiformis, neurologic symptoms (ataxia) | Weight loss, skin rash, Hashimoto thyroiditis | Weight loss, diarrhea | Diarrhea, hypocalcemia, weight loss, extensive small bowel resection |
| Serology at CD diagnosis | EMA+, anti-tTG+ | EMA+, anti-tTG+ | EMA+, anti-tTG+ | EMA+, anti-tTG+ | EMA+, anti-tTG+ | EMA+, anti-tTG+ | EMA+, anti-tTG+ |
| Serology at RCD diagnosis | EMA−, anti-TTG− | EMA−, anti-tTG− | EMA−, anti-tTG− | EMA−, anti-tTG− | EMA−, anti-tTG− | EMA−, anti-tTG− | EMA−, anti-tTG− |
| Endoscopy* | Nodular mucosa | Mosaic mucosa, erosions, and ulcerations | Mosaic mucosa, visible vessels, no ulcerations | Nodular mucosa, disappearance of folds, erosions | Ulcerative jejunitis | Ulcerative jejunitis | Ulcerative jejunitis with multiple stenoses |
| CT scan | Splenic atrophy, thickened SI wall | Thickened SI loops | Splenic atrophy, dilated SI loops | Splenic atrophy | No abnormality | No abnormality | SI ileus |
| PET scan | Increased uptake in SI | Increased uptake in SI | Increased uptake in SI | No abnormality | No abnormality | No abnormality | No abnormality |
| Characteristic . | Patient A . | Patient B . | Patient C . | Patient D . | Patient E . | Patient F . | Patient G . |
|---|---|---|---|---|---|---|---|
| Age, y/sex | 62/M | 70/M | 65/F | 63/M | 64/F | 59/F | 51/M |
| Age at CD diagnosis, y | 56 | 62 | 61 | 48 | 44 | 47 | 50 |
| Age at RCD II diagnosis, y | 59 | 64 | 63 | 63 | 56 | 58 | 51 |
| Age at ASCT, y | 60 | 68 | 64 | 63 | 64 | 59 | 51 |
| Date of ASCT | Mar 2004 | Aug 2004 | May 2005 | Aug 2005 | Nov 2005 | Dec 2005 | Mar 2006 |
| HLA-DQ2 | Homozygous | Homozygous | Heterozygous | Homozygous | Heterozygous | Heterozygous | Homozygous |
| Marsh at RCD diagnosis | III-A | III-B | III-A | III-A | III-C | III-C | III-A |
| BMI, kg/m2 | 19.4 | 18.9 | 17.1 | 24.1 | 20.1 | 21.3 | 20.5 |
| Performance | 1 | 1 | 1 | 1 | 1 | 1 | 2 |
| Symptoms/associations | Diarrhea, pain, weight loss | Pain, diarrhea | Diarrhea, weight loss, hypocalcemia | Diarrhea, weight loss, dermatitis, herpetiformis, neurologic symptoms (ataxia) | Weight loss, skin rash, Hashimoto thyroiditis | Weight loss, diarrhea | Diarrhea, hypocalcemia, weight loss, extensive small bowel resection |
| Serology at CD diagnosis | EMA+, anti-tTG+ | EMA+, anti-tTG+ | EMA+, anti-tTG+ | EMA+, anti-tTG+ | EMA+, anti-tTG+ | EMA+, anti-tTG+ | EMA+, anti-tTG+ |
| Serology at RCD diagnosis | EMA−, anti-TTG− | EMA−, anti-tTG− | EMA−, anti-tTG− | EMA−, anti-tTG− | EMA−, anti-tTG− | EMA−, anti-tTG− | EMA−, anti-tTG− |
| Endoscopy* | Nodular mucosa | Mosaic mucosa, erosions, and ulcerations | Mosaic mucosa, visible vessels, no ulcerations | Nodular mucosa, disappearance of folds, erosions | Ulcerative jejunitis | Ulcerative jejunitis | Ulcerative jejunitis with multiple stenoses |
| CT scan | Splenic atrophy, thickened SI wall | Thickened SI loops | Splenic atrophy, dilated SI loops | Splenic atrophy | No abnormality | No abnormality | SI ileus |
| PET scan | Increased uptake in SI | Increased uptake in SI | Increased uptake in SI | No abnormality | No abnormality | No abnormality | No abnormality |
All patients were treated with prednisone and 2-CDA (cladribine).
SI indicates small intestine; tTG, tissue transglutaminase; and EMA, endomysial antibody.
Gastroduodenoscopy (GDS), video capsule enteroscopy (VCE), or double balloon enteroscopy (DBE).