Poop Pourri of Oral Manifestations of Pediatric Gastrointestinal Disease 13th Annual Fall Pediatric Conference Susan Maisel MD St. Vincent PMCH Pediatric Gastroenterology 317 338-9450 Objective • To provide an update for three pediatric gastrointestinal diseases that can initially manifest themselves in, and profoundly affect the oral cavity. Dermatitis herpetiformis Endoscopic Findings • Frequently normal appearing endoscopy • Can be associated with mild gastritis Dental Enamel Defects and Celiac Disease • Affects 89% • Childhood onset of disease occurs during enamel formation • Characterized by: – – – – demarcated opacities undersized teeth yellowing grooves and pitting • Can occur in asymptomatic Celiacs Dental Enamel Defects and Celiac Disease • Involves permanent dentition • Symmetrical – incisors – Molars • Damage is irreversible • Treatment is cosmetic – bonding – veneers Celiac Disease Histology Normal Partial atrophy I Partial atrophy II Partial atrophy III Subtotal atrophy Total atrophy Oral Cavity and Celiac Disease • Cavities – Calcium and vitamin D deficiencies common • • • • Aphthous stomatitis Atrophic glossitis Dry mouth syndrome Squamous carcinoma Iritis/Uveitis Erythema Nodosum Pyoderma Gangrenosum Inflammatory Bowel Diseases (IBDs) INFLAMMATORY BOWEL DISEASE Ulcerative Colitis (UC) Crohn’s Disease (CD) Mucosal Ulceration in Colon Transmural Inflammation Proctitis Left-sided Extensive Colitis Colitis Upper Small Bowel Colonic Gastrointestinal Anorectal Stenson WF, et al. Inflammatory bowel disease. In: Yamada T et al., eds. Textbook of Gastroenterology Philadelphia, PA: Lippincott Williams & Wilkins;4th Ed. 2003:1699. Worldwide Geographical Prevalence of IBD High Intermediate Low Loftus EV. Gastroenterology. 2004;126:1504. Epidemiology of IBD in North America • Incidence (per 100,000 person-years) – UC: 2.2 to 14.3 cases – CD: 3.1 to 14.6 cases • Prevalence (per 100,000 persons) – UC: 37 to 246 cases – CD: 26 to 199 cases • New diagnoses (per year) – UC: 7,000 to 46,000 cases – CD: 10,000 to 47,000 cases Loftus EV. Gastroenterology. 2004;126:1504. • Population experiencing IBD – 1,400,000 Demographic Features of IBD in North America • Slight male predominance in UC – Incidence of UC seems to have stabilized overall but continues to rise in males • Slight female predominance in CD – Especially in late adolescence and early adulthood – Hormonal factors might play a part • Mean age at diagnosis 15 – 35 • Late onset 50’s-60’s Loftus EV. Gastroenterology. 2004;126:1504. Recurrent Aphthous Ulcers • 48-80% incidence in Crohn’s, less in UC • Parallel or predate intestinal disease • Biopsies can often diagnose Crohn’s • Treatment – topical, intralesional, systemic steroids; aminosalycilate preps • Orofacial granulomatosis • Chronic swelling of the lips and lower half of the face • Oral lesions • Hyperplastic gingivitis Potential Risk Factors Associated With IBD Risk Factors With IBD Association • Cigarette smoking – + risk factor for CD – - risk factor for UC • Appendectomy – + risk factor for CD – - risk factor for UC Risk Factors With Questionable IBD Association • Perinatal and childhood factors • Measles infection or vaccination • Mycobacterial infection • Oral contraceptives – Weak association with IBD • Diet – Increased sugar intake Loftus EV. Gastroenterology. 2004;126:1504. Evolution of Crohn's Disease Behavior Over Time Cumulative probability % 100 90 80 70 Penetrating 60 50 40 30 Stricturing Inflammatory 20 10 0 years 1 3 5 7 9 11 13 15 17 19 Established percentage of CD patients remaining free of penetrating complications (upper curve) and free of stricturing and/or penetrating complication (lower curve) in 2002 patients with Crohn’s disease since onset (diagnosis) of the disease. Adapted from Cosnes J, et al. Inflammatory Bowel Dis. 2000;8:244. The Role of Proinflammatory Cytokines in Crohn’s Disease IL- 6 Inflammation and Tissue Damage B Cell Plasma Cell IL- 12 Antigenpresenting Cell Activation of T cells Inflammatory Cell Adhesion Humoral Immune Response TNF IL- 1 Antigen IL- 8 GM-CSF Leukotrienes, Superoxides, Nitric Oxide, and Prostaglandins Sands BE. Inflammatory Bowel Diseases. 1997; 3:95-113. Feldman M, et al. Advances in Immunology. 1997; 64:283-350. Key Actions Attributed to TNF Mechanism for Antibody Neutralization of TNF van Deventer S. Gut. 1997; 40:443-48. Scallon BJ. Cytokine. 1995; 7:251-59. Feldman M. et al. Advances in Immunology. 1997; 64:283-350. Results of Infliximab Use The use of Infliximab is no longer restricted to patients who have severe disease, not responsive to conventional therapy. 1. A single infusion can induce remission in ~60% of patients with active Crohn’s Disease (Targan et al., 1997). 2. Three infusions over six weeks led to closure of fistulae in 50% of patients (Present et al., 1999). 3. Before Treatment Week 2 Repeated infusions maintains remission in >60% of patients (Hanauer et al., 2002). Figure: Closure of an abdominal fistula in a 60year old man with treatment of Infliximab (5mg/kg). Present et al., 1999. Week 18 Ringed Esophagus Esophageal nodules Whitish exudates Esophageal Stricture with Food Impaction Diagnosis: Endoscopic Features of EE Vertical Lines Rings White Specks Epidemiology • Described in 1978; not recognized until late 1990’s • 15 fold increase in last 16 years • Male to female 2:1 (Allergic) Eosinophilic Esophagitis • 50 – 75% atopic • Food sensitization common • Aero-allergens may also play a role GERD and Dental Erosions 4/7/2015 38 Dental Erosions and GERD • Critical pH of enamel – 5.5 • Gastric refluxate - 2.2 • Salivary protective factors vary – rate of salivary flow – pH – viscosity – protein and mineral content • Fluoride – unproven to help • Brushing after GERD without rinsing first Diagnosis: Clinical symptoms in EE Symptom Median Age of Presentation (years) Feeding disorders 2 Vomiting/reflux 8 Abdominal pain 12 Dysphagia 13.4 and adults Food impaction 16 and adults Noel NEJM;351, 2004 EE vs. GERD Characteristic Atopy Food sensitization Histology Peripheral eosinophilia Esophageal pH PPI Steroids Food allergen elimination EE High High >24 eos/hpf ~50% Normal Usually not helpful Helpful Sometimes helpful GERD Nml Nml 0-7 eos/hpf rare Abnormal Helpful Not helpful Not helpful