Doc I’m Sick – What Do I Have?

1. Doc I’m Sick – What Do I Have? Melissa (Moe) Hagman, MD, FACP Assistant Professor, Internal Medicine University of Washington Boise VA Medical Center, Boise, ID May 12, 2012

2. The Stories of…. • #1 – Connecting the dots slowly • #2 – Can’t stand up • #3 – Your ears must be burning

3. #1 – Connecting the Dots Slowly • 43 yopreviously healthy woman presents with 2 wks of jaundice and fatigue • No stigmata of chronic liver disease, no encephalopathy

4. Hepatic Encephalopathy - Asterixis

5. Number or Figure Connection Test Normal < 30 seconds Gitlin N. Am J Gastroenterol. 1988; 83:8.

6. 134 107 15 88 4.0 23 0.8 8.4 226 36 #1 – Connecting the Dots Slowly • 43 yopreviously healthy woman presents with 2 wks of jaundice and fatigue • No stigmata of chronic liver disease, no encephalopathy AST 1040 U/L Tbili 11.6mg/dL ALT 999 U/L INR 2.3 alb 3.2g/dL

7. Acute (Fulminant) Liver Failure Severe impairment of liver function (coagulopathy) associated with encephalopathy within 26 weeks of the first symptoms

8. #1 – Connecting the Dots Slowly • DfDx for AST and ALT of >1000 U/L • Hypoperfusion • Portal vein thrombosis • Budd-Chiari syndrome – blockage of venous outflow • Ischemic (“shock liver”) • Viruses • Hepatitis A, B, D, E • HSV, EBV, (rarely CMV or VZV) • Toxins

9. Hepatic Toxins • Medications • Acetaminophen • sulfa – phenytoin • NSAIDs – valproate • halothane – TCN, erythromycin • INH – terbinafine • MAOI – herbs • antabuse – etc., etc., etc. Lee. ClinPersp Gastro. 2001; 4:101-110.

10. Hepatic Toxins • Other • mushrooms (amanita phalloides) • yellow phosphorus • CCl4 • illicit drugs • cocaine • ecstasy mushroomexpert.com

11. #1 – Acute Liver Injury due to Etodolac • Stop offending drug/toxin • Start N-acetylcysteine • Even in non-acetaminophen-induced liver failure • Improved transplant-free survival in persons with grade I-II coma (abnormal but without somnolence) at initiation • Refer to liver transplantation center • Improves survival by 20% if pts with acute liver failure are managed at specialist ICU facilities • At least 12 hrs required to do screening and to list patient for transplantation Lee WM. Gastroenterology. 2009; 137:856-64.

12. #1 – Take Home Points • Use number connection test to identify subtle hepatic encephalopathy • Start N-acetylcysteine (even in non-acetaminophen-induced liver failure) • Refer to liver transplantation center • Concern for pending acute liver failure • Synthetic dysfunction • Encephalopathy • HCC MedCon – UW 1-800-326-5300

13. #2 – Can’t Stand Up • 23 yo gentleman with DM1 presents with 9 month history of diarrhea and orthostasis • Today he cannot stand without pre-syncope • HbA1c 11.7% • Lives in Rural Washington with dog • GI work-up over past 9 months unrevealing

14. #2 – Can’t Stand Up Vitals confirm orthostasis PE significant for:

15. 127 96 27 229 6.1 26 1.0 #2 – Can’t Stand Up Random cortisol <1mcg/dL (very low) ACTH 9x upper limit of normal Diagnosis – Primary Adrenal Insufficiency

16. Autoimmune Polyglandular Syndrome Type 2 • Diabetes mellitus type 1 • Adrenal insufficiency • Autoimmune thyroid disease • Autoimmune polyglandular syndrome type 1 • Adrenal insufficiency • Hypoparathyroidism • Mucocutaneous candidiasis

17. #2 – Take Home Points • Persons with autoimmune disease are at risk for autoimmune disease • Dexamethasone does not interfere with cortisol testing • In persons with adrenal insufficiency who become ill, triple corticosteroid dose for three days • See provider if not improved after three days

18. #3 – Your Ears Must Be Burning • 73 yo gentleman with baseline CAD, AS, HTN, OSA on nightly CPAP presents with • 2 yrs papular pruritic rash on torso/extremities • 5 mo red sclera & edema/erythema eyelids • 3 mo migratory oligoarthritis (esp. MCPs, ankles) • 3 mo intermittent fevers to 103°F • 2 wks “change in voice” • Now with c/o 1 day of vertigo/trouble standing

19. #3 – Your Ears Must Be Burning • NKDA • ASA • Candesartan • Zolpidemprn • Nasacort prn • Tobradex eye oint • Triamcinolone oint • Clobetasol cream

20. #3 – Your Ears Must Be Burning • Retired computer engineer • Remote tobacco use, quit 1970’s • Rare alcohol • Dog and cat • Recent travel throughout NW and Alaska • Sister with SLE

21. #3 – Your Ears Must Be Burning • T 37°C, HR 103, BP 142/77, RR 16, O2 saturation 97% on room air • Gentleman lying in bed, looks ill, NAD • b/l red sclera • RRR, 3/6 SM at LUSB • Trace b/l ankle edema; R knee effusion • R>L MCP joints red, warmth, swollen with decreased ROM • Erythematous papules torso, UE/LE, neck

22. 129 97 15 133 4.4 23 0.8 6.4 326 31 #3 – Your Ears Must Be Burning ESR 128 mm/hr (0-20) Ferritin 402 ng/mL CRP 20 mg/dL(0-0.9) Alb 3.1 g/dL GC/Chlamydia – neg UA – normal

23. #3 – Your Ears Must Be Burning • Normal studies prior to admission included • RPR, ANA, ANCA, C3, C4 • Uric acid • SPEP/UPEP • HCV Ab, HBsAg • Extensive infectious work-up • What do you want to do?

24. #3 – Your Ears Must Be Burning • Key history then obtained… • Three times in past year one or both ears had become red • Earlobe spared • No improvement with antibiotics

25. #3 – Your Ears Must Be Burning • Diagnosis – Relapsing polychondritis • with scleritis/episcleritis • risk for myelodysplastic syndrome • ACR Criteria for Diagnosis • Chondritis in 2 of 3 sites • Auricular, nasal, laryngotracheal • At least 2 other features • Ocular inflammation • Audio vestibular damage • Sero-negative inflammatory arthritis