|
| Study/patient ID | Age/sex | IBD phenotype (CD/UC/IC) | Age of IBD diagnosis/temporal relation to diagnosis of AA | IBD flare during AA | Symptoms | Location of AA | Other IBD EIM | Antibiotic treatment | Corticosteroids | Additional immunotherapy | Surgical procedures | Maintenance therapy after diagnosis of AA | Number of relapses |
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| André et al. 2007 [5]/1–21 | Mean 24.4 (range 10–54)/12 F & 9 M | 17CD, 3UC, 1IC | Average age of IBD onset = 23.7 (range 12–38)/before (), concomitant (), after () | Yes (), no () | Fever (), abdominal pain (), weight loss (), diarrhea () | Spleen alone () or spleen and lymph nodes (); pharynx and cervical muscles (); liver (); liver and lymph nodes (); kidney (), and lymph nodes alone () | Arthritis (x4), myalgia (x5), ND (x2), aphthous ulcer (x7) | Yes () | Yes () | Total (), cyclophosphamide (), azathioprine (), methotrexate (), infliximab () | Splenectomy () | Information not provided | Mean = 1.38 (), range 0–5 |
|
| Lamport et al. [6]/22 | 38/F | CD | 34/before | No | Leg weakness | Bilateral psoas muscle and epidural | — | Yes | Yes | — | — | — | 1 |
|
| Actis et al. [7]/23 | 30/M | CD | 30/after | Yes | Fever, diarrhea, abdominal pain | Spleen, abdominal lymph nodes | Panniculitis, polyneuropathy | Yes | Yes | Azathioprine | Splenectomy and lymph node excision | Prednisone (stopped after 4th relapse), azathioprine (after last relapse) | 4 |
|
| Tirpitz et al. [8]/24 | 80/F | UC | 72/before | Yes | Abdominal pain, abdominal swelling, bloody diarrhea | Bi-temporal upper eyelids | Pyoderma gangrenosum | Yes | Yes | — | — | Prednisone, sulfasalazine | 0 |
|
| Murata et al. [9]/25 | 18/F | UC | 15/before | No | Fever | Sternum | Arthritis | Yes | Yes | — | I&D | — | 4 |
|
| Hara et al. [10]/26 | 39/M | UC | 34/before | No | Painful facial lesions, fever, nonbloody diarrhea | SC scalp, face, right inner canthus, submaxilla, and chest | Arthritis | Yes | Yes | — | I&D | Sulfasalazine (after relapse) | 1 |
|
| Coat et al. [11]/27 | 31/F | CD | 28/before | Yes | Fever, back pain | Spleen | — | Yes | Yes | Azathioprine | Splenectomy | — | 1 |
|
| Kinjo et al. [12]/28 | 34/F | UC | 31/before | Yes | Fever, bloody stool | SC sternum | Arthritis | No | Yes | — | I&D | — | 1 |
|
| Holstein et al. [13]/29 | 26/M | CD | 24/before | No | Fever, diarrhea, weight loss, weakness, loss of appetite | Liver, spleen | HLA B27 sacroiliitis, Arthritis | Yes | Yes | Azathioprine | Splenectomy | Azathioprine (after 3rd relapse) | 3 |
|
| Li et al. [14]/30 | 39/F | UC | 26/before | Yes | Fever, bloody diarrhea, abdominal pain | SC left forearm | Arthritis | Yes | Yes | — | I&D | — | 0 |
|
| Coyne [15]/31 | 34/M | CD | 33/before | No | Abdominal pain | Spleen | — | No | No | — | Splenectomy | — | 0 |
|
| Renna et al. [16]/32 | 20/F | CD | 20/concomitant | Yes | Fever, abdominal pain, nonbloody diarrhea, weight loss | Spleen | — | Yes | Yes | — | Splenectomy | Prednisone (before, during, and after relapse) | 1 |
|
| Zakout et al. [17]/33 | 29/F | CD | 29/concomitant | Yes | Fever, abdominal pain, pain in right shoulder radiating from abdomen, right pleuritic chest pain, nonbloody diarrhea | Liver | — | Yes | No | Azathioprine | — | Azathioprine, sulfasalazine | 0 |
|
| Yilmaz et al. [18]/34 | 34/F | UC | 22/before | Yes | Fever, nasal ache, difficulty with breathing | Nasal septum | — | Yes | Yes | — | I&D | — | 0 |
|
| Brooks and Ghaffari [19]/35 | 19/F | CD | 19/concomitant | Yes | Abdominal pain | Spleen | Pustular skin lesions | Yes | Yes | Azathioprine | — | Azathioprine | 0 |
|
| Sakharpe et al. [20]/36 | 48/F | CD | Unknown/before | No | Fever, weakness, loss of appetite, coughing, chest pain | Liver | — | No | Yes | — | — | — | 0 |
|
| Boschetti et al. [21]/37 | 40/F | CD | Unknown/after | No | Fever, abdominal pain | Spleen, pancreas | Sweet’s syndrome | Yes | Yes | Adalimumab | Laparoscopic biopsy of mesenteric lymph nodes | Adalimumab | 0 |
|
| Bollegala et al./case | 33/M | UC | 18/before | Yes | Abdominal pain, fever, weight loss, sweat | Spleen | Pyoderma gangrenosum | Yes | Yes | Infliximab | — | Prednisone & 5-ASA (before relapse),
infliximab (after relapse) | 1 |
|
| Bollegala et al./case | 27/F | UC | 26/before | No | Abdominal pain, chest pain radiating to left shoulder, fever, weight loss | Spleen | Sweet’s syndrome, oral ulcers, arthritis | No | No | Infliximab | Splenectomy | Infliximab | 0 |
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