Multimodality imaging of the gastrointestinal manifestations of scleroderma

Scleroderma is a complex multisystem connective tissue disorder. Early visceral disease, such as gastrointestinal (GI) involvement, associated with significant morbidity and poorer prognosis. Prompt diagnosis crucial to allow disease modifying therapies be initiated early in the course of disease. The primary underlying pathophysiology GI tract dysmotility, muscular atrophy, fibrosis, this reflected imaging features. In paper, we demonstrate appearances involvement describe use advanced magnetic resonance enterography (MRE). A multimodal approach required identify both characteristic features scleroderma potential complications. Traditional fluoroscopic contrast (barium) studies are still commonly performed for assessment oesophagus. More recent advances cross-sectional thorough three-dimensional entire tract. MRE particularly useful small bowel evaluation while also allowing “pseudodynamic” functional concomitant other abdominal viscera structures. autoimmune disorder prevalence 20 per 100,000.1Mayes M.D. epidemiology.Rheum Dis Clin North Am. 2003; 29: 239-254Abstract Full Text PDF PubMed Scopus (209) Google Scholar Its involves immune system activation chronic low-grade inflammatory process characterised by vessel vasculopathy, altered neural function, smooth muscle eventual organ fibrosis.2Allanore Y. Simms R. Distler O. et al.Systemic sclerosis.Nat Rev Primers. 2015; 1: 15002Crossref (346) Scholar,3Wollheim F.A. Classification systemic sclerosis: visions reality.Rheumatology (Oxford). 2005; 44: 1212-1216Crossref (118) It may affect any system, involving respiratory, integumentary, musculoskeletal, or systems. Radiology plays an important evolving role management sclerosis. Although there considerable heterogeneity natural history severity systems affected, development severe often first identified radiologically, significantly prognosis.4Steen V.D. Medsger T.A. Severe sclerosis diffuse scleroderma.Arthritis Rheum. 2000; 43: 2437-2444Crossref (723) recognition can facilitate timely disease-modifying implemented at earlier stage before established fibrosis develops. second most involved after integumentary system. As demonstrated EUSTAR study, many 71% patients have symptoms disease.5Jaeger V.K. Wirz E.G. Allanore al.Incidences risk factors manifestations longitudinal study.PLoS ONE. 2016; 110163894Crossref (97) disorders responsible up 10% scleroderma-related deaths.6Forbes A. Marie I. Gastrointestinal complications: frequent internal complications sclerosis.Rheumatology 2009; 48: 36-39Crossref (117) This highlights importance radiological appraisal Evaluation requires combination imaging. includes atrophy circular layer leading dysmotility dilation, collagen deposition causing dysfunction autonomic nervous system.7Rohrmann Jr., C.A. Ricci M.T. Krishnamurthy S. al.Radiologic histologic differentiation neuromuscular tract: myopathies, neuropathies, progressive sclerosis.AJR Am J Roentgenol. 1984; 143: 933-941Crossref (83) helps explain spectrum along summarised Table 1.Table 1Gastrointestinal features.ManifestationsImaging featuresOesophagusHypomotilityFluoroscopic (barium swallow):-Reduced/absent peristaltic waves-Delayed emptying-Dilated patulous morphology-Widened lower oesophageal sphincter-Gastro-oesophageal refluxCT/MRI-Dilated morphology (coronal diameter >1 cm)-May filled fluid/food debris (risk aspiration)Atrophy fibrosisLower sphincter dysfunctionStomachHypomotilityFluoroscopic meal):-Delayed emptying-Dilation-Gastroesophageal refluxCT/MRI-DilationGastric antral vascular ectasia (GAVE)Small bowelHypomotilityCT/MRI-Dilation (diameter >3 cm) – duodenum jejunum-Hidebound (tight packing/apparent thickening valvulae conniventes), known “stack coins” sign-No mural hyperenhancement diffusion restriction disease-Sacculation-Pneumatosis cystoides intestinalis (CT)-Small faeces sign (CT)Atrophy fibrosisBacterial overgrowthLarge bowelHypomotilityCT-Loss normal haustral pattern-Sacculation-Diverticular disease-Pneumatosis intestinalisAtrophy fibrosisAnorectumAtrophy dysregulation faecal incontinenceMRI-Atrophy anal sphincter-Anterior buckling rectal wall Open table new tab classifications, terminology, distinguished between localised (also morphea only affecting skin), limited cutaneous CREST syndrome), [PSS] skin more widespread involvement) sine (visceral nailfold abnormalities without involvement).8LeRoy C. 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Kawaguchi al.Diagnostic criteria, guidelines sclerosis.J Dermatol. 2018; 45: 633-691Crossref (16) Owing absence single diagnostic test all across wide scleroderma, multiple iterations been suspected cases. were proposed 1980 undergone serial revisions reflect advancing knowledge.12Subcommittee Criteria Association Diagnostic Therapeutic Committee Preliminary (scleroderma).Arthritis 1980; 23: 581-590Crossref (4868) current standard 2013, scoring based on presence specific clinical, biochemical, findings, which listed 2.10Van Different weighting applied finding threshold score definite These increasingly emerge, helping reliably classic end-stage develop, treatments their effectiveness.13Araujo F.C. Camargo C.Z. Kayser Validation ACR/EULAR scleroderma.Rheumatol Int. 2017; 37: 1825-1833Crossref (3) ScholarTable 2Criteria scleroderma.4Steen ScholarSkin fingersFingertip lesions (ulcers pitting scars)TelangiectasiaAbnormal capillariesPulmonary arterial hypertension (proven right heart catheterisation) and/or interstitial lung (pulmonary pronounced basilar portions lungs high-resolution computed tomography chest X-ray)Raynaud's phenomenonScleroderma-related antibodies (e.g., anti-centromere) clinical examination, blood tests, sclerosis.11Asano examples, assessed clinically, cardiac New York Heart (NYHA) class echocardiography, pulmonary function tests (CT). overall determine treatment, generally involve steroids immunosuppressant agents.11Asano Treatment medical gastro-oesophageal reflux, pro-kinetics, nutritional supplements. oesophagus frequently skin.14Sjogren R.W. motility 1994; 1265-1282Crossref (291) symptomatic present patients, has reported 90% cases focused testing.14Sjogren Smooth dominant pathology oesophagus, results abnormal (LOS).15Roberts C.G. 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Standard include distal allows atrophy. Indeed, field view potentially increased thoracic thereby “one-stop shop” evaluating alimentary canal. 40 min ingestion medium complete passage bolus stomach expected. Persistence within suggests gastroesophageal reflux. Additionally, further longer delay sometimes better persistence images infer dysmotility23Lohan Meehan al.MR enterography: optimization timing.Clin 2007; 62: 804-807Abstract (35) 5). cine pathologies scleroderma.22Taylor routinely during MRE, considered optional additional sequence patients. Involvement less common manifests traditionally best nuclear medicine emptying studies. Delayed planar following radio-labelled solids liquids demonstrates into bowel.12Subcommittee Gastric CT/MR 6). ectasia, watermelon stomach, occur bleeding. With similar evaluated morphological dynamic “pseudo-dynamic” assess impaired 7).Figure 7Gastric Coronal acquired 80 ingestion. large volume residual lumen consistent liquids.View (duodenum jejunum) (anorectum) common.14Sjogren Scholar,19Savarino feature sites, dilation. Stasis contents bacterial overgrowth bowel, found 30–62% patients.24Pittman N. Rawn S.M. Wang al.Treatment intestinal systematic review.Rheumatology 57: 1802-1811Crossref (26) Bacterial interferes digestion lead malabsorption diarrhoea.25Sakkas L.I. Simopoulou T. Daoussis al.Intestinal review.Dig Sci. 63: 834-844Crossref (21) Muscular stasis cause jejunum 8, 9). Intestinal overgrowth. Acute pseudo-obstruction, estimated 3.7% acute hospital admission patients.26Mecoli Purohit Sandorf al.Mortality, recurrence, sclerosis-related pseudo-obstruction.J 2014; 41: 2049-2054Crossref (15) Chronic sepsis form pneumatosis.25Sakkas 9Small image. Diffuse (blue arrow) (red demonstrated. “Hidebound” crowding conniventes again seen.View Cross-sectional MRI replaced older follow-through radiographic bowel. provides excellent soft-tissue investigation disorders. accurate evaluation, usually readily possible unrelated indications. Small transverse 9, 10). dilates contraction restricts lengthening decreased distance adjacent conniventes. seven 2.5 mucosal fold spacing (4–7 folds despite tightly packed conniventes, sclerosis, classically hidebound 9), term depict emaciated cattle attached bony skeleton.27Leshchinskiy D’Agostino Hidebound sign.Abdom Radiol (NY). 2513-2516Crossref (1) was initially well MRE. recently, coins sign” 10) additionally imaging.28Venkatasamy Minault Q. Romain B. stack scleroderma.Abdom 2878-2879Crossref Findings clear resolution, give thickened 11). Absence surrounding fat stranding differentiate from infectious causes thickening. addition improve crowded unable 12). Sacculation, wide-mouthed outpouchings, asymmetric transient intussusception. sign, persistent slow transit finding.29Fuchsjager M.H. small-bowel sign.Radiology. 2002; 225: 378-379Crossref (42) 12(a) positive loops arrows). Addition improves Fig 11.View contrast-enhanced intravenous gadolinium recommended MRE.22Taylor its sites active enteritis, MR previously described. Mild negligible enhancement 13). fibrotic/atrophic nature component perceived folds. Low-grade inflammation below detection MRI. Diffusion-weighted protocols.22Taylor Similarly, uncertain, add value Crohn's disease.30Abd-El Khalek Abd-ALRazek Fahmy D.M. diffusion-weighted apparent coefficient activity Crohn disease: 1.5 3 T.J Comput Assist Tomogr. 42: 688-696Crossref No 14), high-grade change. stages yet described.Figure 14Diffusion-weighted B400 B800 mildly high signal intensity wall. corresponding low ADC map (c), negative restriction.View Pneumatosis (PCI), clusters gas wall, complication bowel.31Balbir-Gurman Brook O.R. Chermesh al.Pneumatosis conditions.Intern 2012; 323-329Crossref (31) plain radiographs 15) evaluation.31Balbir-Gurman PCI benign but pneumoperitoneum difficulty distinguishing sinister intramural ischaemia. Classically, extensive no free fluid peritoneal cavity. Recognition avoid unjustified surgical intervention.31Balbir-Gurman under-recognised volvulus, diverticular disease.32Brandler J.B. 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