Table 1 Clinical characteristics, treatment and outcome in 39 patients with SS and digestive manifestations
From: Gastrointestinal manifestations in Satoyoshi syndrome: a systematic review
Author (year) [ref] | Age of onset (age of onset digestive manifestations) | Sex | Malabsorption tests | Autoantibodies | Endoscopy | Histology | Treatment | Outcome | |
|---|---|---|---|---|---|---|---|---|---|
1 | Bledsoe I (2019) [32] | 11 | Female | NR | NR | NR | NR | Steroids, IVIG, plasmapheresis, methotrexate, and tacrolimus. Botulinum toxin. | NR |
2 | Al Dallal R (2019) [31] | 12 (12) | Female | NR | NR | NR | NR | IVIG and systemic corticosteroid therapy. Rituximab, carbamazepine, imodium. | NR |
3 | Solera J (2017) [11] | 10 (12) | Female | Carbohydrate malabsorption/D-xylose. | Anti-ACh receptor. Anti-GAD. | NR | NR | Carbamazepine and gabapentin, IVIG, prednisone, methotrexate. | Resolution of diarrhea with methotrexate and corticosteroids. |
4 | Li J (2017) [12] | 6.5 | Female | NR | NR | NR | NR | Prednisone, carbamazepine. | Improvement of diarrhea. |
5 | Aghoram R (2016) [13] | 28 (30) | Male | NR | Anti-ACh receptor. Reactivity to unknown neuronal antigens. | Sigmoidoscopy revealed inflamed colon. | Collagenous colitis. | Steroids and mycophenolate mofetil, phenytoin, IVIG, plasmapheresis. | Improvement of diarrhea. |
6 | Rudnicka L (2014) [7] | 36 (37) | Female | NR | ANA (1/640), anti-La/SSB, anti-histone antibodies, trace of dsDNA. | NR | NR | Triamcinolone and phenytoin. | NR |
7 | 4 (4) | Female | NR | ANA (1/160) | NR | NR | Prednisone, cyclosporine. | NR | |
8 | 1 (1) | Female | NR | NR | NR | NR | Prednisone, ciclosporin. | NR | |
9 | Merino de Paz N (2014) [14] | 4 (4) | Female | NR | Anti-gliadin | NR | NR | Carbamazepine, otilonium bromide. | Disappearance of diarrhea. |
10 | Rosales RL (2013) [15] | 0 (0) | Female | NR | NR | Esophagogastroduodenoscopy and colonoscopy. Flattened mucosal folds. | NR | IVIG, dantrolene, methylprednisolone. | After IVIG he continued with loose stools. The digestive evolution after corticosteroids was not reported. |
11 | Ishii K (2010) [16] | 13 (13) | Male | NR | Anti-ACh receptor | Colonoscopy documented no abnormality. | Lymphocytic colitis. | Steroid and dantrolene. | Control of all his symptoms to achieve complete remission for 3 years. |
12 | Castiglioni C (2009) [2] | 9 (10) | Female | D-xylose positive. | Anti-endomysial antibody negative. | Upper digestive endoscopy: leucoplakias were observed in the duodenum and a pronounced vasculature. | Moderate increase of the lymphoplasmacytic infiltrates of the lamina propria with granular neutrophils and eosinophils. | Prednisone. Restriction of simple carbohydrates from the diet. | Improvement of diarrhea with corticosteroids and disappearance after restriction of simple carbohydrates. |
13 | Asherson RA (2008) [5] | 52 (53–54) | Female | NR | Anti-endomysial and anti-gluten antibodies both negative. ANA (1/1280). Anti-microsomal and anti-thyroglobulin antibodies. | The patient underwent foregut and hindgut endoscopy, but no significant abnormalities were detected. Later, upper endoscopic study with infiltration of the duodenum. | Biopsy of the infiltrated area compatible with eosinophilic enteritis. | Steroids, IVIG, diazepam, cyclophosphamide, azathioprine. | NR |
14 | Matsuura E (2007) [6] | 8 (15) | Female | D-xylose positive. | Band of 90-kDa position on western blot for brain, spinal cord, stomach and duodenum tissue lysates. | Upper gastrointestinal tract: almost the entire mucosa of the stomach was atrophic, and multiple ulcer scars were observed, mainly in the body. Small white granules -speculated to be a type of secretion- were observed from the first to the second portion of the duodenum. | Histopathology of the first portion of the duodenum showed mucosal infiltration with inflammatory cells. Helicobacter pylori was not present. | NR | NR |
15 | Heger S (2006) [17] | 12 | Female | D-xylose positive. | ANA (1/640) | Endoscopy of the oesophagus, stomach, duodenum and ileo-colon with small duodenal ulcerations. | NR | Carbamazepine, IVIG, corticosteroids, methotrexate. | Diarrhea improved after treatment with methotrexate. |
16 | Nagahama T (2006) [4] | 17 (21) | Female | D-xylose positive. Fecal clearance of α1antitripsina positive. Oral glucose tolerance test with 75 g glucose revealed a flat curve. | ANA | Upper-GI endoscopy revealed a normal esophagus and numerous nodular protrusions, akin to submucosal tumors, involving the cardia and the body of the stomach. Kerckring’s folds in the duodenum were lost, and the mucosa showed a fine granular appearance with white spots; a portion of the mucosa also showed small polypoid protrusions. Total colonoscopy revealed only an occasional ulcer scar in the cecum, the descending colon, and the sigmoid colon. | Histopatology of biopsy specimens from the stomach, the duodenum, and the large intestine did not provide any definitive diagnosis, because the findings were not characteristic. Autopsy data available. | Total intravenous hyperalimentation. | Death |
17 | Ezgu FS (2005) [18] | 11 (11) | Female | NR | NR | NR | NR | Prednisolone | After three months, diarrhea disappeared. |
18 | Ashalatha R (2004) [19] | 10 (10) | Female | NR | NR | Upper gastrointestinal endoscopy: normal. | Biopsy normal. | Phenytoin and prednisolone (previously clonazepam, tetrabenazine, baclofen, and diazepam). | NR |
19 | Cecchin CR (2003) [20] | 7 | Female | D-xylose normal. | NR | Upper gastrointestinal endoscopy showed a 2-cm duodenal erosion. | Gastric and duodenal biopsies showed chronic gastritis and duodenal ulcer, respectively. | Prednisone and amitriptyline. | Asymptomatic (diarrhea was not named). |
20 | Oyama M (1999) [21] | 4 (13) | Female | NR | ANA (1/640) | They only stated that gastrointestinal tract examination was normal, with no inflammatory findings (endoscopy?). | NR | Antispasmodic and antipyretic drugs at first. Prednisolone. | Antispasmodic and antipyretic drugs caused only a decrease in the muscle spasms and diarrhea. Not reported after starting steroids. |
21 | Ikeda K (1998) [8] | 30 (30) | Female | NR | ANA (×40) anti-microsomal and anti-thyroid antibodies. | NR | NR | Dantrolene and prednisolone. | NR |
22 | Merello M (1994) [22] | 36 | Male | NR | NR | NR | NR | Dantrolene. Phenytoin and carbamazepine, botulinum toxin. | NR |
23 | Ikegawa S (1993) [23] | 4 (14) | Female | NR | NR | NR | NR | Dantrolene | NR |
24 | Kuru S (1992) [24] | 12 (12) | Male | D-xylose normal. | NR | NR | NR | Several muscle relaxants. Corticosteroids. | NR |
25 | Yamagata T (1991) [25] | 13 | Female | Slight alteration in carbohydrate absorption. | ANA | NR | NR | Glucocorticoids | Free of symptoms at 6 months, but no other reference. |
26 | De-Xin W (1985) [26] | 13 (13.5) | Female | Oral glucose tolerance test showed a relatively flat curve. | NR | NR | NR | Carbamazepine, phenobarbital, quinine sulfate and chlorpromazine | NR |
27 | 9 | Female | Oral glucose tolerance test showed a flat curve. | NR | Fibrogastroscopy with mucosal atrophy of the stomach and duodenum. A fungoid lesion of 0.3 mm diameter was seen on the mucosal surface of the stomach. | NR | Neostigmine and Chinese traditional medicine. | NR | |
28 | Averianov IN (1984) [27] | 10 (11) | Female | D-xylose positive. | NR | Colonoscopy: normal. | NR | Thioridazine, haloperidol, phenytoin, acetazolamide. | NR |
29 | Satoh A (1983) [28] | 19 (21) | Female | D-xylosa positive. Oral glucose tolerance test showed a flat curve. | Anti-ACh receptor antibody. | NR | NR | Dantrolene | NR |
30 | Itahara K (1976) [29] | 7 (8) | Female | Oral glucose tolerance test showed a flat curve. | NR | Gastroscopy: “Mosaic pave with stone” and partially “small polyp-like” edematous mucosa at corpus ventriculi, pars pylorica and duodenum. | Normal and/or atrophic chief cells in mucosa with edematous submucosa tissue or lymphangiectasia. | NR | NR |
31 | Inoue K (1976) [30] | NR (21) | Female | Oral glucose tolerance test showed a flat curve. | NR | Disseminated white spots in the jejunum. | Fat droplets in epithelial cells after administration of olive oil by tube. | NR | NR |
32 | Satoyoshi E (1975) [1] | 9 (10) | Female | D-xylosa positive. High fecal excretion of I-triolein. Altered oral glucose test. | NR | NR | Autopsy data available. Gastroenteritis cystica polyposa. | NR | Death |
33 | 9 (9) | Male | D-xylosa positive. | NR | NR | NR | NR | NR | |
34 | 14? | Female | D-xylosa positive. High fecal excretion of I-triolein. Oral glucose tolerance test showed a flat curve. | NR | NR | Autopsy data available. Gastroenteritis cystica polyposa. | NR | Death | |
35 | 12 (16) | Female | NR | NR | NR | NR | NR | NR | |
36 | 7 (16) | Male | NR | NR | NR | NR | NR | NR | |
37 | 7 (10) | Female | NR | NR | NR | NR | NR | Two years later, frequent defecation unchanged. | |
38 | 9 (9) | Male | Malabsorption was suspected, but test were not performed. | NR | NR | NR | NR | Suicide at age 19. | |
39 | 10 (30) | Female | NR | NR | NR | NR | NR | NR |