A Case of Cauliflower Ears

1. A Case of Cauliflower Ears Hilary Rowe, BScPharm VIHA Pharmacy Resident 2009-10 Pain Clinic Rotation

2. Outline • Objectives • Background • Patient Case • Clinical Question • Review of Evidence • Recommendation • Monitoring

3. Objectives • Describe 1 way inflammation destroys cartilage in relapsing polychondritis (RP) • Name 3 risk factors for addiction in a pain patient • Be familiar with the evidence of disease modifying agents in RP

4. Relapsing Polychondritis • Destruction of cartilage and replacement with fibrous tissue • Autoantibodies to type II, IX, XI collagen causes inflammatory infiltration • Produce Th1 cytokines (TNF-α) by T-cell clones reactive to Type II collagen • Lysosomal enzyme release eventually results in destruction of the cartilage

5. Diagnostic Criteria Presence of 3 or more: • Recurrent chondritis both auricles • Non-erosive inflammatory polyarthritis • Nasal chondritis • Ocular inflammation • Respiratory tract chondritis • Cochlear &/or vestibular dysfunction

6. Symptoms

7. Treatment ? Methotrexate, Colchicine, Dapsone, Hydroxychloroquine Treat inflammation-Prednisone Treat pain-NSAIDS Diagnosis

8. Mrs. MJ • ID: 40 yo female, ht 155cm, wt 62kg • CC: Acute decline in functioning with widespread pain and stiffness in joints • HPI Nov 2009:Current RP flare of longest duration; walking this summer and now in motorized wheel chair since September • RP diagnosed Aug 2009, polyarthritis since 2005

9. Mrs. MJ • PMHx:Transposition of ureters 1983- Recurrent UTI’s (prior to surgery 8-9/year, after surgery 1-2/year) • Allergies: Lactose (hives & difficulty breathing)

12. Mrs. MJ • Social & Family Hx: • Lives with husband & two teenagers • Prior to attack was running an event planning business • Both parents were alcoholics • Discharge Plan from Pain Clinic: • Improve pain control & function

13. Medical Problem List Active: • Prolonged flare of RP • Pain • Constipation Chronic: • Depression • Osteopenia • RP • Graves disease • Pain

14. Review of Systems

15. Score is 5: • 3 points family history • 1 point age • 1 point depression • Other factors: • Drug seeking • Altering routes • Running out early • Rx forgery • Stealing • ↑ dose with no change in disease state

16. Review of Systems

17. Review of Systems

18. Review of Systems

19. Review of Systems

20. Review of Systems

21. Pain History Paroxysmal attacks: • Left side more affected then right • Described: red-hot poker stabbing and digging into her • 20/10 causing her to sob, occurs with flares • What makes it better-? more medication • What makes it worse- Nothing

22. Pain History Baseline aches: • Widespread: Nose, chest, sternum, jaw, elbows, back, shoulders, wrists, hands, hips, ankles • Described: ache • What makes it better-baths, medication • What makes it worse- > 300-400 steps per a day

23. DRPs • MJ has a prolonged polychondritis flare and is experiencing additional pain not controlled by her current therapies • MJ is experiencing constipation secondary to narcotics and immobility and could benefit from a regular bowel routine

24. DRPs • MJ has a prolonged flare of polychondritis and could potentially benefit from re-evaluation of her disease modifying agents

25. Question • Are there any disease-modifying therapies that might be helpful for Mrs. MJ’s prolonged flare of relapsing polychondritis, taking into consideration the medications she has already tried?

26. Therapeutic Options • No change in therapy • Infliximab • Rituximab • Azathioprine • Cyclophosphamide

27. Clinical Question

28. Search Strategy • PubMed, Embase, Google • Search terms: • Relapsing polychondritis • Disease modifying agents • Autoimmune diseases • Found • 3 case reports, 1 retrospective review

29. Leroux et al. Arthritis & Rheumatism 2009

30. Leroux et al. Arthritis & Rheumatism 2009

31. Leroux et al. Arthritis & Rheumatism 2009 • Results: • 2 partial remissions • 4 stable • 3 worsened • 2 added new immunosuppressants • 2 increased steroid dose • 6 benefitted- at 12 months 2 remained stable & 4 were worse

32. Leroux et al. Arthritis & Rheumatism 2009

33. Marie et al. Rheumatology 2009

34. Buonuomo et al. Rheumatol Int 2009

35. Richez et al. Rheumatol Int 2009

36. Goals of Therapy Patients Goals • Improve pain control • Increase mobility and ADL • Return to work Team Goals • Improve pain control • Increase mobility and ADL • Slow progression of disease • Decrease morbidity & mortality • Minimize adverse drug events

37. Recommendation • No definitive evidence to support suggesting a disease-modifying agent • Risks and benefits of infliximab should be discussed with patient • Patient should make an informed decision to start therapy

38. Recommendation • Improve pain control • Discontinue Codeine Contin • Start Morphine 30mg long acting q 12h • Start Morphine IR 5mg prn for breakthrough pain

39. Recommendation • Codeine Contin ineffective pain 20/10, poor sleep, dose above ceiling effect of 400mg/day • Morphine is effective for breakthrough pain • Morphine less potential for abuse then hydromorphone and oxycodone • SR formulation less potential for abuse

40. Monitoring

41. Monitoring

42. Follow Up- Feb 2010 • Patient switched from Codeine Contin to Morphine (↓ IR 2 daily to 2-3 nights/wk) • Currently ↓ prednisone dose • Patient wanted to trial dapsone & colchicine 1st (DMARD was not started) • Patient now considering DMARD option • Constipation improving

43. Questions?

44. References • Kahan M, Srivastava A, Wilson L et al. Misuse of and dependence on opioids: study of chronic pain patients. Canadian Family Physician 2006;52:1081-87. • Marie I, Lahaxe L, Josse S, Levesque H. Sustained response to infliximab in a patient with relapsing polychondritis with aortic involvement. Rheumatology 2009 Oct;48(10):1328-33. • Leroux G, Costedoat-Chalumeau N, Brihaye B, et al. Treatment of relapsing polychondritis with rituximab: a retrospective study of nine patients. Arthritis Rheumatology 2009 May 15;61(5):577-82. • Buonuomo PS, Bracaglia C, Campana A, et al. Relapsing polychondritis: new therapeutic strategies with biological agents. Rheumatology International. 2009 Aug 15. [Epub ahead of print]. • RichezC, Dumoulin X, Schaeverbeke T. Successful treatment of relapsing polychondritis with infliximab. Clinical and Experimental Rheumatology 2004;22:629-31. • PorroGB, Lazzaroni M, Imbesi V et al. Efficacy of pantoprazole in the prevention of peptic ulcers, induced by non-steroidal anti-inflammatory drugs: a prospective, placebo-controlled, double-blind, parallel-group study. Digestive and Liver Disease 2000 April; 32(3): 201-208.