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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)SexMalabsorption testsAutoantibodiesEndoscopyHistologyTreatmentOutcome
1Bledsoe I (2019) [32]11FemaleNRNRNRNRSteroids, IVIG, plasmapheresis, methotrexate, and tacrolimus. Botulinum toxin.NR
2Al Dallal R (2019) [31]12 (12)FemaleNRNRNRNRIVIG and systemic corticosteroid therapy. Rituximab, carbamazepine, imodium.NR
3Solera J (2017) [11]10 (12)FemaleCarbohydrate malabsorption/D-xylose.Anti-ACh receptor. Anti-GAD.NRNRCarbamazepine and gabapentin, IVIG, prednisone, methotrexate.Resolution of diarrhea with methotrexate and corticosteroids.
4Li J (2017) [12]6.5FemaleNRNRNRNRPrednisone, carbamazepine.Improvement of diarrhea.
5Aghoram R (2016) [13]28 (30)MaleNRAnti-ACh receptor. Reactivity to unknown neuronal antigens.Sigmoidoscopy revealed inflamed colon.Collagenous colitis.Steroids and mycophenolate mofetil, phenytoin, IVIG, plasmapheresis.Improvement of diarrhea.
6Rudnicka L (2014) [7]36 (37)FemaleNRANA (1/640), anti-La/SSB, anti-histone antibodies, trace of dsDNA.NRNRTriamcinolone and phenytoin.NR
74 (4)FemaleNRANA (1/160)NRNRPrednisone, cyclosporine.NR
81 (1)FemaleNRNRNRNRPrednisone, ciclosporin.NR
9Merino de Paz N (2014) [14]4 (4)FemaleNRAnti-gliadinNRNRCarbamazepine, otilonium bromide.Disappearance of diarrhea.
10Rosales RL (2013) [15]0 (0)FemaleNRNREsophagogastroduodenoscopy and colonoscopy. Flattened mucosal folds.NRIVIG, dantrolene, methylprednisolone.After IVIG he continued with loose stools. The digestive evolution after corticosteroids was not reported.
11Ishii K (2010) [16]13 (13)MaleNRAnti-ACh receptorColonoscopy documented no abnormality.Lymphocytic colitis.Steroid and dantrolene.Control of all his symptoms to achieve complete remission for 3 years.
12Castiglioni C (2009) [2]9 (10)FemaleD-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.
13Asherson RA (2008) [5]52 (53–54)FemaleNRAnti-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
14Matsuura E (2007) [6]8 (15)FemaleD-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.NRNR
15Heger S (2006) [17]12FemaleD-xylose positive.ANA (1/640)Endoscopy of the oesophagus, stomach, duodenum and ileo-colon with small duodenal ulcerations.NRCarbamazepine, IVIG, corticosteroids, methotrexate.Diarrhea improved after treatment with methotrexate.
16Nagahama T (2006) [4]17 (21)FemaleD-xylose positive. Fecal clearance of α1antitripsina positive. Oral glucose tolerance test with 75 g glucose revealed a flat curve.ANAUpper-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
17Ezgu FS (2005) [18]11 (11)FemaleNRNRNRNRPrednisoloneAfter three months, diarrhea disappeared.
18Ashalatha R (2004) [19]10 (10)FemaleNRNRUpper gastrointestinal endoscopy: normal.Biopsy normal.Phenytoin and prednisolone (previously clonazepam, tetrabenazine, baclofen, and diazepam).NR
19Cecchin CR (2003) [20]7FemaleD-xylose normal.NRUpper 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).
20Oyama M (1999) [21]4 (13)FemaleNRANA (1/640)They only stated that gastrointestinal tract examination was normal, with no inflammatory findings (endoscopy?).NRAntispasmodic 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.
21Ikeda K (1998) [8]30 (30)FemaleNRANA (×40) anti-microsomal and anti-thyroid antibodies.NRNRDantrolene and prednisolone.NR
22Merello M (1994) [22]36MaleNRNRNRNRDantrolene. Phenytoin and carbamazepine, botulinum toxin.NR
23Ikegawa S (1993) [23]4 (14)FemaleNRNRNRNRDantroleneNR
24Kuru S (1992) [24]12 (12)MaleD-xylose normal.NRNRNRSeveral muscle relaxants. Corticosteroids.NR
25Yamagata T (1991) [25]13FemaleSlight alteration in carbohydrate absorption.ANANRNRGlucocorticoidsFree of symptoms at 6 months, but no other reference.
26De-Xin W (1985) [26]13 (13.5)FemaleOral glucose tolerance test showed a relatively flat curve.NRNRNRCarbamazepine, phenobarbital, quinine sulfate and chlorpromazineNR
279FemaleOral glucose tolerance test showed a flat curve.NRFibrogastroscopy 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.NRNeostigmine and Chinese traditional medicine.NR
28Averianov IN (1984) [27]10 (11)FemaleD-xylose positive.NRColonoscopy: normal.NRThioridazine, haloperidol, phenytoin, acetazolamide.NR
29Satoh A (1983) [28]19 (21)FemaleD-xylosa positive. Oral glucose tolerance test showed a flat curve.Anti-ACh receptor antibody.NRNRDantroleneNR
30Itahara K (1976) [29]7 (8)FemaleOral glucose tolerance test showed a flat curve.NRGastroscopy: “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.NRNR
31Inoue K (1976) [30]NR (21)FemaleOral glucose tolerance test showed a flat curve.NRDisseminated white spots in the jejunum.Fat droplets in epithelial cells after administration of olive oil by tube.NRNR
32Satoyoshi E (1975) [1]9 (10)FemaleD-xylosa positive. High fecal excretion of I-triolein. Altered oral glucose test.NRNRAutopsy data available. Gastroenteritis cystica polyposa.NRDeath
339 (9)MaleD-xylosa positive.NRNRNRNRNR
3414?FemaleD-xylosa positive. High fecal excretion of I-triolein. Oral glucose tolerance test showed a flat curve.NRNRAutopsy data available. Gastroenteritis cystica polyposa.NRDeath
3512 (16)FemaleNRNRNRNRNRNR
377 (10)FemaleNRNRNRNRNRTwo years later, frequent defecation unchanged.
389 (9)MaleMalabsorption was suspected, but test were not performed.NRNRNRNRSuicide at age 19.
3910 (30)FemaleNRNRNRNRNRNR
  1. ANA Anti-nuclear antibodies; Anti-ACh receptor Anti acetyl-choline receptor; Anti-GAD Anti-Glutamate acid decarboxylase; IVIG Intravenous immunoglobulins; NR Not reported