(I) Normal
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XVI Congresso della Federazione Nazionale delle Malattie Digestive (FISMAD) Verona, 6-9 Marzo 2010 Joint Meeting GISMAD-AIGO-SIED-SIGE DISTURBI DELLA MOTILITA’ GI NELLE PATOLOGIE SISTEMICHE Patologie reumatiche Francesca Galeazzi UOC Gastroenterologia Azienda Ospedale-Università Padova G.I. motility and Rheumatic diseases Rheumatic disease GI motor abnormalities: • clinical impact • prognostic value • progression • Clinical manifestation +/• Altered motor function Suspected Rheumatic disease G.I. involvement in autoimmune diseases 100 % 0 SSc Wegener Sjogren Beçhet RA LES Poli/Derma Mixed Motility Others (mucosal, vascular, side effects) Adapted: Schneider A et al, Gastrointest Endoscopy Clin N Am 2006 Systemic sclerosis Mucosa Vascular Neural Vascular Muscular Neural Fibrotic Normal Submucosa Serosa Oedema Normal Endothelial oedema Oedema Smooth muscle Normal Collagen + inflammatory cells around vessels normal ENS Inflammatory cells in l propria normal Collagen Thickness of vascular wall Collagen Patchy fibrosis Fibrosis of glands Collagen atrophy mm normal Axonal degeneration Degeneration Extensive fibrosis of Serosal epithelium Fibrosis submucosa thickening Muscular Collagen Axonal degeneration Patchy fibrosis (mainly circular) intercellular gap junction Fibrotic Extensive axonal degeneration Fibrosis, atrophy of muscular wall thinning Adapted: Sallam H et al, Aliment Pharmacol Ther 2006 Systemic sclerosis • Direct neural damage (antiAch Abs) • Cells of Cajal Sakkas LI, Arthritis & Rheumatism 2004 Roberts GC et al, Gut 2006 G.I. motility in SSc Peristalsis LES pressure Dysphagia GERD Accomodation EGG abnormalities Delayed empying Vomiting Malnutrition Contractility Pseudoobstruction Bacterial overgrowth Malabsorption Colon Anus-Rectum Constipation Diarrhea G.I. motility in SSc 100 % 0 Esophagus Stomach Small bowel Colon Ano-rectum Forbes A, Rheumatology 2008 Systemic sclerosis Esophagus Uncoord perist LES Normal / Aperistalsis, LES pressure Reflux - Impaired clearance In symptomatic pts: Esophagitis: 56-60% Strictures: > 40% Asymptomatic pts suspected Barrett: 37% ? Disease subtype Duration Symptoms Zamost BJ et al, Gastroenterol 1987 Basilisco G et al, Gut 1993 Katzka DA et al, Am J Med 1987 Systemic sclerosis 133 pts SSc; duration 1-38 yrs (M 6 yrs) PPI standard dose Upper GI endoscopy, Manometry Heartburn Dysphagia Nausea/Vomiting 77.4% 14.3% 9.8% Esophagitis Barrett Candidiasis 32% 6.8% 7% (IV) Aperistalsis LES pressure (II-III) Uncoord perist LES Normal / (I) Normal 48% 28% 24% Marie I et al, Alimen Pharmacol Ther 2006 Systemic sclerosis 133 pts Esophagitis/Barrett: No relation with •subtype (diffuse, localized) •duration Symptoms! •age Marie I et al, Alimen Pharmacol Ther 2006 SSc: esophagus and lung 133 pts No association with • subtype • duration • age Association with lung disease! Severe esophageal motor abnormalities Marie I et al Alimen Pharmacol Ther 2006 SSc: esophagus and lung 40 consecutive SSc pts 15 dcSSc 25 lcSSc 45% pulmonary fibrosis • HRCT • pH-impedance More severe reflux (acid and non-acid) in pts with interstitial lung disease Savarino E et al, Am J Resp Crit Care 2008 SSc: esophagus and lung 5 cm above LES Proximal reflux in pts with ILD ILD pts no relation •subtype •duration •age •GERD symptoms Savarino E et al, Am J Resp Crit Care 2008 15 cm above LES Juvenile Localized Scleroderma 14 consecutive pts Juvenile Localized Scleroderma Age 6-17; Disease duration: 4.7 yrs (0.2-13.2) •Symptoms 7 •Pathological 24 hrs pH–monitoring •Esophagitis •No major motor abnormalities 1 Guariso G et al, Clin Exp Rheumatol 2007 •Asymptomatic •Low LES basal pressure Systemic sclerosis Esophagus High prevalence of esophageal lesions in SSc on therapy (Pts on PPI: > 75% heartburn; 30% esophagitis) No relation with disease subtype, duration, age Esophageal involvement associated with interstitial lung disease G.I. motility in SSc Small bowel 100 % 0 Esophagus Stomach Small bowel Colon Ano-rectum Systemic sclerosis Small bowel 10 pts with altered esophageal motility: 8/10 impaired SB motility (neuropathy + myopathy) Diffuse motor alterations Sjolund K et al, Eur J Gastroenterol Hepatol 2005 Systemic sclerosis Small bowel 8 SSc pts SB manometry at diagnosis and 5 yrs 5 yrs Onset: 75% pts abnormal SB manometry 5 yrs: 100% worsening of SB motor activity Marie I et al, Rheumatology 2007 Small bowel bacterial overgrowth 55 pts vs 60 HV LBT Rifaximin 10 days Small bowel involvement: Common Progressive • Bacterial overgrowth >50% • Malabsorption • Pseudobstruction Parodi A et al, Am J Gastroenterol 2008 Marie I et al, Rheumatology 2009 SSc pts Controls SIBO +ve SIBO -ve Systemic sclerosis and GI motility Subgroup of patients? •14 pts severe GI involvement within 2 yrs of onset •288 pts No GI involvement •117 pts No GI involvement within 2 yrs of onset Subgroup GI as main early manifestation (esophagus / SB) • ILD less frequent! • 50% ANA +ve nucleolar pattern Nishimagi E et al, J Rheumatol 2007 G.I. involvement in autoimmune diseases RA: impaired esophageal peristalsis, reduced LES pressure (up to 58% pts) 100 SLE: segmentary or diffuse altered esophageal motility % Polymyositis/Dermatomyositis: esophagus, small bowel 0 SSc Wegener Sjogren Beçhet RA LES Poli/Derma Mixed Mixed connective tissue disease: Smooth muscle involvement Motility Others (mucosal, vascular, side effects) Adapted: Schneider A et al: Gastrointest Endoscopy Clin N Am 2006 Salivary outflow (gr/2 min) Sjogren 27 pts -dysphagia 76% (40.6% severe) Xerostomia? •Simultaneous contractions distal (22%) and proximal (11%) esophagus •No relation with salivary function Anselmino M et al, Dig Dis Sci 1997 Fibromyalgia Chronic musculo-skelatal pain without tissue inflammation or damage Stressors IBS FD Pain Fibromyalgia processing Irritable bladder TMD Intestinal permeability Pamuk ON et al, J Rheumatology 2009 Fibromyalgia Pamuk ON et al, J Rheumatology 2009 GI motility and Rheumatic diseases GI motility alterations commonly described in rheumatic diseases, affecting > 90% pts in SSc Except for SSc, specific pattern of motor abnormalities unclear In SSc GI motility impairment may represent the most relevant internal manifestation, with potentially severe complications Difficult to identify patients and to predict severity of motor alterations only on the basis of clinical symptoms and in absence of specific “markers” (subgroups of patients?)
