DIAGNOSING CHRONIC PANCREATITIS WITHOUT THE CLASSIC TRIAD

1. DIAGNOSING CHRONIC PANCREATITIS WITHOUT THE CLASSIC TRIAD DR P BADENHORST

2. Patient history • Mr J – A 39 year old black male from Bloemfontein • Presented with: • Chronic abdominal pain – 3 years • Worsening of pain over past 3 days • Nausea and vomiting • Malaise • Pain: • Epigastric which radiates to the back • Multiple similar episodes (never admitted) • Slightly relieved by sitting

3. Patient history (continued) • Systemic: • GIT • No heart burn • Stools foul smelling and greasy • Weight loss – 4 kg in past year • RESP • No complaints • CVS • No complaints • CNS • No complaints

4. Patient history (continued) • Medical: • Diabetes mellitus diagnosed in 2009 • Treatment: • Protaphane 28U nocte • Actrapid 10U before each meal • Surgical: • No previous surgery • Social: • Strong alcohol history (20 years) • Five (5) smoking pack years • Allergies: • No known allergies

5. General BP: 130/80 mmHg Pulse: 104/min Temperature: 36.2 C Acute on chronically ill No dehydration No jaundice/cyanosis/anaemia/ lymphadenopathy GIT Epigastric tenderness No acute abdomen No mass No hepatosplenomegaly No ascitis CVS Normal examination RESP Normal examination No signs of basal pneumonia CNS Normal examination Clinical Examination

6. Special investigations

7. Special investigations (continued)

8. Further investigations • Random glucose 14mmol/L • HBA1c 13% • s- Amylase 344 IU/L (High) • u- Amylase 1623 IU/L (High) • CXR Normal • AXR

9. Abdominal X-ray Pancreatic calcifications

10. Diagnosis • CHRONIC PANCREATITIS WITH AN ACUTE UPFLARING • CLASSIC TRIAD • CALCIFICATIONS • STEATORRHEA • DIABETES

11. FOCUS OF DISCUSSION • WORK UP OF A PATIENT WITH SUSPECTED CHRONIC PANCREATITIS WHERE CLASSIC TRIAD IS NOT PRESENT

12. BACKGROUND (EPIDEMIOLOGY) • 70% DIAGNOSED AT AGE 35-60 • MALE 4:1 FEMALE • 23/100 000 PEOPLE WORLDWIDE • INCIDENCE RISING –INCREASED ALCOHOL CONSUMPTON • RECENT POST MORTEM STUDIES SHOWS EVIDENCE OF CHRONIC PANCREATITIS IN UP TO 45% OF ASYMTOMATIC ALCOHOLICS

13. CLINICAL MANIFESTATIONS • PAIN - DOMINANT FEATURE • NO PAIN IN 30% OF PATIENTS • PANCREATIC INSUFFICIENCY • PANCREATIC DIABETES # LATE IN COURSE OF DISEASE • MALABSORBTION (90% PANCREAS DESTROYED) # LIPOLYTIC ACTIVITY DECREASES FASTEST WITH STEATORRHEA # VIT A,D,E,K , B12 RARE AND LATE

14. DIFFERENTIAL DIAGNOSIS OF ABDOMINAL PAIN( EPIGASTRIC) • PEPTIC ULCER DISEASE • GALLSTONES • DISEASES OF BILIARY TRACT • PANCREAS CA • OTHER ABDOMINAL MALIGNANCIES • OTHER • TB ABDOMEN • MESENTERIC ISCHAEMIA • NON ULCER DYSPEPSIA • MEDICAL CAUSES= DKA BASAL PNEUMONIAE INFERIOR MYOCARDIAL INFARCTION ETC.

15. WORK-UP • DIAGNOSING CHRONIC PANCREATITIS • ENDOCRINE INVOLVEMENT • EXOCRINE INVOLVEMENT • ETIOLOGY • COMPLICATIONS

16. MAKING THE DIAGNOSIS BLOOD AND IMAGING TESTING NOT SENSITIVE IN EARLY CHRONIC PANCREATITIS BLOOD - AMYLASE AND LIPASE NOT DIAGNOSTIC - NORMAL IN > 50% -TRYPSIN LEVELS NOT DIAGNOSTIC AND VERY EXPENSIVE GASTROSCOPY -TO EXCLUDE PUD AND GASTRITIS

18. CT PANCREAS CALCIFICATIONS IN PANCREAS ATROPHIC PANCREAS DILATED PANCREATIC DUCT

20. MRCP • PREFERRED ABOVE ERCP • BEADING OF DUCTS • PANCREAS DUCT OBSTRUCTION . DILATED PANCREATIC DUCTS

22. ENDOSCOPIC ULTRASONOGRAPHY • MOST SENSITIVE IN EARLY CHRONIC PANCREATITIS • ?? DO THIS PATIENTS DEVELOP CP • FEATURES • IRREGULAR DUCTS • SIDE BRANCHES • STONES • DILATATION OF DUCTS

23. 2. ENDOCRINE INVOLVEMENT • WORK UP FOR DIABETIS • USUALLY INSULIN DEPENDANT • NB! RISK OF HYPOGLYCAEMIA

24. 3. EXOCRINE INVOLVEMENT • DIRECT AND INDIRECT TESTS • DIRECT TESTS DONE IN VERY FEW CENTRES IN SA

25. INDIRECT TESTS • 72H FECAL FAT DETERMINATION IS GOLD STANDARD • FECAL ELASTASE BEST OPTION IN ANY SETTING • SENSITIVE IN MODERATE TO SEVERE PANCREATIC INSUFFICIENCY( LEVELS <200 UG/G) • ONLY ONE SAMPLE NEEDED • NOT INFLUENCED BY PANCREATIC ENZYME REPLACEMENT

26. DIRECT TESTS • SECRETIN STIMULATION TEST • IN VERY FEW SPECIALIZED CENTRES • ADMINASTRATION OF A MEAL • PANCREAS STIMULATED • PANCREATIC SECRETIONS OBTAINED IN DUODENUM- DETERMINE NORMAL PANCREATIC SECRETORY CONTENT

27. Toxic-metabolic Alcohol Smoking Hypercalcaemia Hyperlipidaemia Chronic renal failure Drugs Toxins Idiopathic Early Late Tropical Genetic Hereditary Cationic trypsinogen SPINK1 CFTR Autoimmune Isolated Sjogren IBD PBC Recurrent acute attacks Obstructive Pancreas divisum SOD Tumour Duodenal wall cyst 4. ETIOLOGY (TIGAR-O)

28. 4. ETIOLOGY • IF NO HISTORY OF ALCOHOL AND GALLSTONES EXCLUDED ON SONAR • ANF,ANCA,IgG 4, RF TO EXCLUDE AUTO IMMUNE PANCREATITIS • POSITIVE IgG4 IS DIAGNOSTIC OF AUTO IMMUNE PANCREATITIS • ASSOCIATED WITH # PRIMARY SCLEROSING CHOLANGITIS # PRIMARY BILLIARY CIRRHOSIS # SJOGREN SYNDROME # AUTO-IMMUNE HEPATITIS - TRIGLYCERIDES AND CALCIUM

29. GENETIC TESTING • MUTATIONS ASSOCIATED WITH CHRONIC PANCREATITIS IN: • CFTR GENE • SPINK-1 • PRSS-1 • CURRENTLY NOT PART OF NORMAL WORK-UP FOR CHRONIC PANCREATITIS # CFTR GENE MUTATION IN 44% OF PATIENTS WITH CHRONIC PANCREATITIS # ALSO PRESENT IN 22% OF HEALTHY POPULATION

30. 5. COMPLICATIONS • PAIN • DIABETES MELLITUS • EXOCRINE INSUFFICIENCY • PSEUDOCYSTS (30%) • DUODENAL STENOSIS • SPLENIC ARTERIAL THROMBOSIS • PANCREATIC ASCITIS • PANCREAS CARCINOMA

31. PANCREAS CA • EXOCRINE INSUFFICIENCY ALONE NOT DIAGNOSTIC OF CRONIC PANCREATITIS- CA CAN PRESENT SIMILARLY • SOME STUDIES SHOW 15X INCREASED RISK FOR PANCREAS CA • RECOMMENDATION IS YEARLY ENDOSCOPIC ULTRASOUND FROM 40Y OF AGE IN PATIENTS WITH CHRONIC PANCREATITIS • DIFFICULT TO DISTINGUISH BETWEEN PANCREATIC TUMOR AND CHRONIC INFLAMMATORY PROCESS

32. CONCLUSION • REMEMBER CHRONIC PANCREATITIS IN DDx OF CHRONIC ABDOMINAL PAIN EVEN IF INITIAL INVESTIGATIONS IS NORMAL

33. BIBLIOGRAPHY • UP TO DATE • FAUCI,AS ET AL, HARRISONS PRINCIPLES OF INTERNAL MEDICINE. 17TH EDITION • WWW.MEDIFOCUS. THE EVALUATION OF SURGICAL TREATMENT OF CHRONIC PANCREATITIS. ANDERSON,DK; FREY,CF • Q.LIAO ET AL. HEPATOBILIARY AND PANCREATIC DISEASE INTERNATIONAL VOLUME 1, NO3, 2006, • WWW.MEDCONSULT.COM: DIAGNOSIS OF CHRONIC PANCREATITIS

34. THANK YOU!