sJIA-and IBD-related characteristics of the sJIA-IBD cohort (n = 16).
| sJIA Features | n (%) | IBD Features | n (%) |
|---|---|---|---|
| Clinical manifestations at sJIA diagnosis | Clinical manifestations at IBD diagnosis | ||
| Arthritis | 16 (100) | Diarrhea | 12 (75) |
| Fever attributed to sJIA | 15 (94)a | Abdominal pain | 11 (69) |
| Rash attributed to sJIA | 11 (69) | Weight loss | 10 (63) |
| Lymphadenopathy | 4 (25) | Arthritis | 8 (50) |
| Hepato/splenomegaly | 3 (19) | Hematochezia | 7 (44) |
| Weight loss | 3 (19) | Fever | 6 (38) |
| Serositis | 2 (13) | Oral ulcers | 2 (13) |
| Myositisb | 1 (6) | Perianal disease | 2 (13) |
| Presence of MASc | Failure to thrive | 1 (6) | |
| At sJIA diagnosis | 2 (13) | Vomitingb | 1 (6) |
| During sJIA treatment | 2 (13) | Rash attributed to IBD | 0 (0) |
| Course of sJIA disease | Specific IBD diagnosis | ||
| Monocyclic | 3 (19) | Crohn disease | 13 (81) |
| Polycyclic | 1 (6) | Ulcerative colitis | 0 (0) |
| Persistent | 12 (75)d | Indeterminate colitis | 3 (19) |
| Laboratory variables | Laboratory variables | ||
| Leukocytosis | 9/13 (69) | Leukocytosis | 5/15(33) |
| Anemia | 10/14 (71) | Anemia | 9/16 (56) |
| Thrombocytosis | 10/13 (77) | Thrombocytosis | 9/15 (60) |
| Elevated AST and/or ALT | 3/12 (25) | Elevated AST and/or ALT | 1/14 (7) |
| Hypoalbuminemia | 4/8 (50) | Hypoalbuminemia | 8/14 (57) |
| Elevated ESR | 12/12 (100) | Elevated ESR | 10/14 (71) |
| Elevated CRP | 12/12 (100) | Elevated CRP | 11/15(73) |
| Elevated ferritin | 8/10 (80) | Elevated stool calprotectin | 4/6 (67) |
Data on laboratory values were missing for several patients; the denominator for each variable indicates the number of patients for whom a response was provided. Features shown were queried in the survey, except for 2, as noted.
↵aOne patient had a recent history of fever, which was likely masked by medication at sJIA diagnosis, per the case reporter.
↵bFeatures that were specified as additional features by respondents.
↵cNo patients developed MAS following IBD diagnosis.
↵dOf the subjects with persistent sJIA, 4 were described as systemic-predominant and 5 as polyarthritis-predominant courses, per case reporters who responded to this follow-up question. ALT: alanine aminotransferase; AST: aspartate aminotransferase; ESR: erythrocyte sedimentation rate; CRP: C-reactive protein; IBD: inflammatory bowel disease; MAS: macrophage activation syndrome; sJIA: systemic juvenile idiopathic arthritis.