İnflammatory bowel disease

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Inflammatory bowel disease and lung Prof Dr Berrin Ceyhan Marmara Univ School of Medicine, ISTANBUL, TURKEY. İnflammatory bowel disease. IBD is a chronic inflammatory disease commonly involving the gastrointestinal system characterised by mucosal inflammation and ulcers Etiology İnfection - PowerPoint PPT Presentation

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  • Inflammatory bowel disease and lung

    Prof Dr Berrin CeyhanMarmara Univ School of Medicine, ISTANBUL, TURKEY

  • nflammatory bowel disease

    IBD is a chronic inflammatory disease commonly involving the gastrointestinal system characterised by mucosal inflammation and ulcers

    EtiologynfectionFood allergyEnvironmental factorsGeneticsImmunologic factorsNormalUCCD

  • nflammatory bowel disease

    The incidence of ulcerative colitis is 1.5-20.3/100.000 person-yearsThe incidence of Crohns disease is 0.7-9.8/100.000 person-years

    Frequency of extraintestinal manifestations occur in %21-41 of patients with IBD

    Lung and gastrointestinal system are originated from primitiv gut and they have same pathogenetic changes

  • Ulcerative colitisCrohns diseaseDistributionDiffuse inflammation extending from rectumRectal sparing, frequent skip lesionsInflammationDiffuse, with mucosal granularity or friabilityFocal and asymmmetric; cobblestoning, granularity and friability less commonUlcerationSmall ulcers in a diffusely inflamed mucosa; deep ulcers in severe diseaseAfphtoid ulcers; linear/serpiginous ulceration; intervening mucosa often normalColonic lumenOften narrowed in long-standing chronic disease; strictures very rareStrictures common

  • Extraintestinal manifestations of inflammatory bowel diseaseMusculoskletalPeripheral arthropathyAnkylosing spondylitisSacroileitisHipertrophic osteoarthropathyOsteopeniaOsteoporosisOsteomalaciaOsteonecrosisRelapsing polychondritis

    DermatologicEr. NodosumPyoderma gangrenosumStomatitisPsoriasisErt. MultiformeMetastatic Crohns diseaseSweets syndromeEpidermolysisHematolojgicAnemiaLeucocytosisLeucopeniaThrombocytosisThrombocytopeniaCoagulation abnormalities and hypercoaguable stateNeurologicNeuropathyMyopathyVasculopathyMeningitisSeizures

  • Extraintestinal manifestations of inflammatory bowel disease

    OpthalmologicUveitisScleritisEpiscleritisRetinal vascular diseaseConjunctivitis

    GenitourinaryNephrolithiasisObstructive uropathyFistulasAmiloidosisGlomerulitisMembranoglomerulonephritis

    Cardiovascular

    Pleuropericarditis

    Cardiomyopathy

    Endocarditis

    Myocarditis

    PancreaticGranulomatous pancreatitis

    HepatobiliaryPrimary sclerosing cholangitisPericholangitisCirrhosisCholangiocarcinomaSteatosisGranulomatous hepatitisCholelithiasisAutoimmune hepatitisHepatic abscessPortal vein trombosis

  • Ulcerative colitis and lung involvement

    Upper airway obstructionTracheobronchitisBronchiectasisConstrictive bronchiolitisPanbronchiolitisNecrobiotic noduleLung bullaeINterstitial lung diseaseBOOPSarcoidosisPulmonary vasculitisPulmonary eosinophiliaWegener granulomatosis?(lack of kidney involvement)Apical fibrosis

  • Crohns disease and lung involvement BOOPILDSubclinical lymhocyctic alveolitisChronic bronchitisChronic bronchial suppurationBronchiectasisGranulomatous infiltration and peripheral eosinophiliaNecrobiotic nodule

  • 33 IBD cases(21 F, 12 M, 17-80 years old) , (27 ulcerative colitis and 8 Crohns disease) with lung involvement were reported and 57 IBD cases in literature were reviewed

    Camus et al Medicine 1993;151.

    400 IBD cases from literature and a review was published in 2003

    Storch I et al Inf Bowel Dis 2003; 9:104-115Milestones in the literature of lung involvement in IBD Firstly, Kraft et al reported 6 IBD cases (5 ulcerative colitis) with lung involvement in 1976 (1400 cases were screened in 40 years) Kraft et al Arch Intern Med 1976;454.

  • 28/33 IBD diagnosis preceded lung involvement, 5/33 lung disease preceded IBD diagnosis Inactive bowel diease 17(%60.7) active 3(%10.7) postcolectomy 8(%28.6)Camus et al 1993

    Diagnosis of lung involvement mean age: 42.72.9 ya IBD diagnosis mean age: 35.32.9 years IBD diagnosis preceded 9.35.3 years the lung involvement (range: 1 week-36 years)

  • Symptoms: Stridor, severe dyspnea during 2-3 weeks

    Differential diagnosis of subglottik stenosis: Intubation, tuberculosis, sarkoidosis, amiloidosis

    Bronchoscopy: Fragile, bleeding tissue and %50-80 obstruction in the lumen

    Airway Disease Upper airway obstruction Subglottic inflammation and stenosis in tracheal obstruction (3/33 cases in Camuss cases)

  • Treatment: Good response to inhaled and oral steroids, one case stayed in remission state during 8 years

    Rupture following biju dilatation was noted in one case who was unresponsive to previous steroid treatment and he died with pneumomediastinumBiopsy: Granulation tissue and inflammation (plasma cells, lymphocytes, neutrophils, and erythrocytes) in airway, epithelial ulceration and thin fibrin layer Clinical progress: IBD was diagnosed 10 and 20 years before in two cases , IBD was diagnosed 1 month after pulmonary disease in the other case

  • Large airway involvement Chronic bronchitis and bronchiectasis

    Symptoms; cough, sputum and dyspnea( mucopurulent sputum up to 800 ml/day)

    15/33 cases in Camus cases (13UC, 2 Crohn) Camus et al Medicine 1993;151.9 cases with colectomy and 1 case with asthma Airway disease was diagnosed in 9/22 pulmonary involvement cases with Crohns disease

    Omori H et al Inf Bowel Dis 2004

  • Radiology: Tubuler bronchial opacities, bronchiectasis in CT

    Lung function test: FEV1/FVC

    Bronchoscopy: Eritematous and edematous mucosa and inflammation in the wall

  • Bronchial biopsy revealed submucosal lymphocytes, plasma cells, neutrophilic infiltration, mucosal ulceration, chronic inflammation with abundant plasma cells=Bronchial biopsy is mimicking colon biopsy

  • BAL commonly reveals leucocytes, but lymhocytes in one case 9 cases were in postcolectomy state, airway disease occured in days and weeks after colectomy The mean interval after IBD diagnosis was 7.41.9 years Extraintestinal manifestations were detected in 10/15 cases Camus et al Medicine 1993;151.

    In the other study of 7 cases with large airway disease, interval was 12 years (4 months-35 years)Spira et al Chest 1998

  • Airway involvement was not associated with intake of sulphasalazin and 5-ASA

    Symptoms of bronchiectasis and bowel were activated at the same time

    Bronchiectasis was activated after colectomy (3/7 cases 1-4 months after colectomy)

  • Bronchial steroid lavage is helpful, (40-80 mg metil prednisolon in serum physiologic in 2-3 days)

    There was no response in 2 cases of Camus cases, they were in waiting list of transplantation

    Immuran and cyclophosphamide were not effective treatment choicesTREATMENTSteroids are more effective in chronic bronchitis than bronchiectasis, therefore inhaled steroids should be started in the early stage and progression to bronchiectasis should be slowed. Lung function test and bronchoscopy show response with inhaled +/- oral steroid

  • Small airway disease (Chronic bronchiolitis)

    Less common presentationThis disease was diagnosed in 2 UC patients in Camus cases

    Both of them had inactive disease and open lung biopsy revealed chronic and stenotic chronic bronchiolitis=diffuse panbronchiolitis

  • Chronic bronchiolitis

  • Diffuse panbronchiolitis (DPB) is diagnosed in non Asian people 1 patient with DPB has been reported 5 years before UC diagnosis

    Limited treatment, mild-moderate response to oral steroids 1 patient of Camus cases responded to steroid, the other one was transplanted Macrolid effect??

  • Bronchiolitis obliterans organised pneumonia (BOOP)6 patients in Camus cases (5UC, 1 Crohns disease) Interval 2 months- 36 years BOOP was diagnosed 6 months before the onset of IBD in one case LFT: Restriction in all subjects BAL: One case with neutrophilia, one with lymphocytosis

  • TREATMENT: Steroids were used in 4 patients and led to complete remission, two patients with mild symptoms improved without any tretment in 2 and 6 months There was no relation to sulphasalazine and 5-ASA intake, remission occured in one case while taking the drug There was no association with colectomy

  • Pyoderma gangrenosum in skin (lung and skin biopsies are similar) have been reported

    4 cases with necrobiotic nodules are reported in the literature

    2 new cases were included in Camus serie, in one of them had p. gangrenosum and pANCA(+).

    IBD has been diagnosed 11 ve 25 years earlier, both of them were inactive and one was taking low dose steroid and sulfasalazine

    Nekrobiotic parenchymal nodules Rounded and cavitated nodules Biopsy: Neutrophilic infiltration in necrotic area

  • Early nodules show neutrophils and fibrinous exudate These nodules undergo central necrosis and cavitated to form large necrotic nodules resembling necrotic granulomas There are no giant cells, no severe vasculitis (there were secondary inflammatory cells in vascular walls), and no non-necrotizing granuloma

    IBD complicated by pulmonary vasculitis (Wegener granulomatosis?)

    There are vasculitis( inflammatory cells in vascular walls, giant cells, granuloma, cANCA, pANCA and intestinal manifestations (25 cases)

  • Parencymal necrobiotic nodules

    Relationship with skin neutrophilic dermatosis?

    Pyoderma gangrenosum was reported in a patient with cavitated lung nodules,who had no IBD history 1 of the 8 patients with neutrophilic dermatosis originally described by Sweet had ulcerative colitis, however pulmonary lesions could not be found

  • Churg Strauss syndrome was considered in a case with face swelling, arthralgia, wheezing, nasal congestion, and skin vasculitis

    Oral steroids ar