1. Vasculitises

2. Outline Basics Small groups Review

3. Definition Inflammation of blood vessel walls

4. Symptomatology The clinical (and pathological) features are variable. Depend on the site and type of blood vessels that are affected.

5. How does this affect inflammation affect vessels?

6. What is common between Vasculitides? Fever night sweats Malaise Myalgia arthralgia

7. Copyright ©2000 BMJ Publishing Group Ltd. Savage, C O S et al. BMJ 2000;320:1325-1328

8. Large Vessel Vasculitis Takayasu’s Arteritis –Primarily affects the aorta and it’s branches Temporal Arteritis –Large and medium vessels –Most prominently involves the cranial branches of arteries arising from the aorta

9. Medium Vessel Vasculitis Polyarteritis Nodosa –Systemic vasculitis –Typically affects small and medium vessels Kawasaki disease –Large, medium and small –Particularly the coronary arteries

10. Small Vessel Vasculitis Churg-Strauss Arteritis –Medium and small vessels –Classically involves lung and skin Wegener Granulomatosis –Medium and small vessels –Venules and Arterioles as well –Usually involves the upper/lower respiratory tract and kidneys Henoch-Schonlein Purpura –Small vessels

11. Small Group Cases Go through your case with your group (5 min) Come up with a diagnosis (5 min) Quick Research (5-10 mins) –Signs/Symptoms –Labs/DI –Complications Present to Group (3-5 mins per group)

12. Our Focus today Wegener HSP Kawasaki’s Churg-Strauss

13. Wegener Granulomatosis Usually caucasian Most frequently in 5th generation Males and Females equally affected

14. Symptoms Upper Respiratory –Sinusitis, otitis, oral ulcers Lower Respiratory –Cough, hemoptysis, dyspnea Kidneys –Blood, protein, casts Other (ophth., GI, cardiac, neuro)

15. First Presentation Unexplained Constitutional Sx Upper airway –Sinusitis present in 1/2 to 2/3 Lower Airway Other symptoms unusual at presentation

16. Investigations Elevated ESR N. N. anemia, +/- low plts. Urinalysis: hematuria, active sediment, proteinuria C-ANCA (ANA usually neg.) CXR: nodular densities (LL’s), pleural effusions, opacities

17. Treatment Cyclophosphamide +/- prednisone Methotrexate +/- prednisone +/- septra prophylaxis, +/- plasmapheresis IVIg, TNF blockers, etc…

18. Acute Complications Infection Pulmonary Hemorrhage Renal Failure

19. HSP Children represent 90% of cases Association with recent URTI and drugs Can affect any age IgA deposition in organs

20. Signs/Symptoms Palpable purpura Hematuria Abdominal pain Arthralgia Bloody diarrhea Fever

21. The Rash Begins as a maculopapular erythema Coalesces to form ecchymoses, petechiae, and purpura Usually symmetric and in gravity dependent areas

22. Investigations CBC, lytes, Cr Urinalysis Coagulation studies Plain radiography Ultrasound +/- joint aspiration, biopsy, etc.

23. Complications Renal (usually not severe) Occasionally, Intussusception, GI bleeding Rarely, Neurologic and pulmonary involvement

24. Treatment Disease is self-limiting Supportive treatment Hospitalization (dehydration, bleeding, other complications)

25. Adults Rarely get intussusception More likely to have renal involvement (including ESRD)

26. Outpatient Monitoring Urinalysis and BP q 1-2 weeks for first 2 months Then monthly extending to bi-monthly Then during well-child visits

27. Kawasaki Disease More than 85% are less than 5 yrs

28. Signs and Symptoms Fever for 5 days or more –Bilateral conjunctival injection –Mucous membrane involvement –Polymorphous rash –Extremity involvement –Cervical adenopathy

29. Investigations CRP/ESR CBC Urinalysis ALT Albumin Cultures +/- echocardiography

30. Complications Coronary Artery Aneurysms Atherosclerosis/MI Peripheral arterial obstruction (less common) Other: hyponatremia, dehydration, pancreatitis, arthritis, renal involvement

31. Treatment IVIg –Good evidence showing decrease incidence of CAA (2g/kg IV over 12 hours) ASA –Standard practice? (80-100 mg/kg/d PO divided qid for 2 wk initial) –Lack solid evidence Steroids –Evidence in refractory cases –No good studies for initial management

32. Churg-Strauss Usually follows a predictable pattern: –Prodromal Phase: –Eosinophilic Phase: –Vasculitic Phase:

33. Prodrome Usually 2nd-3rd decade –Atopic disease –Allergic rhinitis –Asthma

34. Eosinophilic Peripheral infiltration of organs –Especially lungs and GI tract

35. Vasculitic Life threatening May be heralded by the onset of constitutional symptoms

36. Symptoms Asthma (95%) Allergic Rhinitis –Or recurrent sinusitis, polyosis, etc Skin (2/3) –Palpable purpura, erythema, hemorrhagic lesions, subcutaneous nodules

37. Investigations CBC CXR biopsy

38. Complications Cardiovascular disease –Pericarditis, CHF, MI Neurologic –Mononeuritis Renal –Failure rare GI –Abdominal pain

39. Treatment Prednisone +/- cyclophosphamide

40. THP’s Know the basic presentations Be aware of complications Talk with rheumatology

41. The End