CHRONIC PERICARDITIS… A Rheumatologist’s Perspective

2. CHRONIC PERICARDITIS… A Rheumatologist’s Perspective Steven S. Overman, MD, MPH, FACR The Seattle Arthritis Clinic Clinical Professor of Medicine University of Washington, Seattle, Washington USA

3. The pericardial tissue that covers the heart is similar to the synovial tissue that lines the joints. The diagnostic and treatment principles for pericarditis are the same as those for chronic arthritis. This means that a physician who specializes in chronic arthritis may be able to help your chronic pericarditis patients. CHRONIC PERICARDITISA ‘Window’ to Chronic Arthritis

4. My Background • Private rheumatology practice for over 25 years • Masters Degree in Public Health • Robert Wood Johnson Clinical Scholar • Board Certified: Rheumatology, Internal Medicine, & Quality Assurance • Medical Directorships • Clinical Investigator • American College of Rheumatology committees and strategic planning

5. Clinical Professor of MedicineUniversity of Washington, Seattle

6. 5 Principles of Rheumatology Diagnosis & Chronic Pericarditis 1) Search for infections 2) Understand how rheumatology tests can be useful (or misleading!) 3) Re-examine for rheumatic- disease signs 4) Search for organ problems 5) Treat and then re-evaluate

7. Sandra’s Pericarditis • Sandra, age 49, developed a sore throat and cough after noticing an unusual smell coming from her home’s heating vents. • Three weeks later, she presented with syncope and right-sided CHF.

8. Sandra’s Pericarditis HOSPITAL • Pericardial effusion and tamponade diagnosed with echocardiography • Pericardial window placed • Cultures and tissue samples were culture (-) • Troponins suggested myocardial damage RHEUMATOLOGY TESTS • ANA, RF were negative • A family history of rheumatoid arthritis and rheumatic fever was noted

9. Sandra’s Pericarditis Outpatient • Initially improved with colchicine and tapering prednisone 8 weeks • Major flare with a 15x increase in C-reactive protein • Legionella IgM antibody found positive • Spondylitis was diagnosed based on X-ray diagnosis of sacroiliitis and a (+) HLA-B27 12 months • Recurrent flares of “reactive pericarditis” were treated with typical anti-rheumatic drugs - methotrexate, tapering prednisone, and biologic therapies. Sandra has improved.

10. MILDAcute Pericarditis • No hemodynamic problems • No fever • Not immunosuppressed • Responds to aspirin or NSAIDs and colchicine

11. • Fever >38 C • Follows trauma or myocardial infarcts • Cardiac tamponade • Does not respond to first-line therapy SEVERE Acute Pericarditis

12. Pericarditis Differential Diagnosis • Infections tuberculosis, malaria, bacterial, atypical bacterial, viral, spirocheaeteal, fungal, helminthes infections • Inflammatory • Neoplastic • Myocarditis or post-myocardial infarct • Metabolic • Traumatic • Radiation

13. 5 Principles of Rheumatology Diagnosis & Chronic Pericarditis • SEARCH FOR INFECTIONS • Routine blood and pericardial fluid examinations • Adenosine deaminase <40 means no TB 97% of the time • Serologies check for infections not easily cultured

14. Diagnosing Active Tuberculosis NEWEST TEST GeneXpertis the newest gene array identification technology that can identify the tuberculosis bacillus from body fluid in 2 hours. This test can simultaneously identify TB and resistance to rifampicin.

15. Legionnaire’s disease Acute respiratory illness after heating system smell. The organism is found in heating, cooling, and humidifying systems in communities and hospitals. Pericardial fluid culture (-) Myocarditis with pericarditis is the most common extra-pulmonary manifestation (30%). IgM Legionnella Ab positive proved the diagnosis. Sandra’s Infection

16. 5 Principles of Rheumatology Diagnosis & Chronic Pericarditis 1) Search for infections 2) UNDERSTAND HOW RHEUMATOLOGY TESTS CAN BE USEFUL (OR MISLEADING!)

17. Common Inflammation Markers • C-reactive protein and sedimentation rate are often - but not always - increased in rheumatic syndromes. • Unfortunately, they are still non-specific and are increased in infections.

18. Auto-antibody Patterns in Inflammatory Rheumatic Diseases • SERO-POSITIVE means an auto-antibody is positive • Rheumatoid Factor (RF) • Anti-cyclic citrullinated peptide (anti-CCP) • Antinuclear Antibody (ANA) • Anti-double stranded DNA (anti-dsDNA) • Extractable Nuclear Antibody (ENA), SSA, SSB, Scl-70, RNP, Sm, and others • Anti-neutrophil cytoplasmic antibody (pANCA/cANCA) • SERO-NEGATIVE means ALL auto-antibodies are negative

19. INITIAL: Legionella myocarditisand pericarditis BUT, LATER: reactive pericarditis “skyrocketed” her CRP, although all auto-antibodies were negative. Sandra’s Post-infectious, Sero-negativeInflammatory Reaction

20. 5 Principles of Rheumatology Diagnosis & Chronic Pericarditis 1) Search for infections 2) Understand how rheumatology tests can be useful (or misleading!) 3) Re-examine for rheumatic-disease signs

21. Sero-negativeSYSTEMIC LUPUS ERYTHEMATOSIS • Malar rash • Discoid rash • Photosensitivity • Oral ulcers • Arthritis • Pleuritis or pericarditis • Renal disorder • Seizures or psychosis • Hematologic disorder • Anti-dsDNA, anti-Sm, or anti-phospholipid Ab • Antinuclear antibodies

22. Sero-negativeMEDIUM VESSEL VASCULITIS • Polyarteritis nodosa • Behcet’s syndrome • Churg-Strauss syndrome

23. Sero-negativeRHEUMATOID ARTHRITIS • Rheumatoid nodules • Pericarditis • Premature cardio- vascular mortality • Vasculitis • Lung disease

24. Sero-negativeRHEUMATIC FEVER • Erythema marginatum • Subcutaneous nodules • Migratory arthritis • Carditis and valvulitis • Central nervous system involvement

25. Sero-negativeADULT STILL’S DISEASE • Arthralgias or arthritis • Nonpruritic, ‘salmon’ colored, maculopapular rash • Lymphadenopathy • Hepatomegaly or splenomegaly • Leukocytosis • Sore throat • Abnormal liver function • Negative tests for antinuclear antibody andrheumatoid factor

26. Sero-negativeSPONDYLOARTHRITIS SYNDROMESSandra’s Sacroliitis

27. Sero-negativeSPONDYLOARTHRITIS SYNDROMES • Ankylosing spondylitis • Psoriatic spondyloarthritis • Reactive spondyloarthritis • Inflammatory bowel-associated spondyloarthritis • Undifferentiated spondyloarthritis and enthesitis

28. Sero-negativePSORIASIS ARTHRITISDIP Swelling, but not osteoarthritis

29. Sero-negativePSORIASISnail-bed pitting

30. Sero-negativePSORIASISOnycholysis

31. Sero-negativeREACTIVE ARTHRITISkeratoderma blennorrhagicum

32. Sero-negativeREACTIVE ARTHRITISdactylitis and keratoderma

33. 5 Principles of Rheumatology Diagnosis & Chronic Pericarditis 1) Search for infections 2) Understand how rheumatology tests can be useful (or misleading!) 3) Re-examine for rheumatic-disease signs 4) SEARCH FOR ORGAN PROBLEMS

34. • Heart • Lungs • Gastrointestinal • Endocrine • Renal • Liver • Eyes and Brain • Large vessels • Joints SEARCH FOR ORGAN PROBLEMSSandra’s evaluation

35. 5 Principles of Rheumatology Diagnosis & Chronic Pericarditis 1) Search for infections 2) Understand how rheumatology blood tests can be useful (or misleading!) 3) Re-examine for rheumatic- disease signs 4) Search for organ problems 5) TREAT & RE-EVALUATE

36. Chronic Pericarditis Treatments are the same as Chronic Arthritis Treatments • Antibiotics • NSAIDs (ibuprofen; aspirin) and colchicine • Corticosteroids • Disease-modifying drugs • Biologic injects

37. 5 PRINCIPLES 1) Search for infections 2) Understand rheumatology tests 3) Re-examine for rheumatic disease signs 4) Search for organ problems 5) Treat and re-evaluate SANDRA’s CARE Pericardial fluid cultures were negative, but her serology for Legionella infection was positive. General inflammation tests became positive, but all auto-antibody tests were negative. She had no new signs, but showed sacroiliitis on X-ray. Kidney and liver function normalized when the tamponade was relieved. With treatment for reactive pericarditis and spondyloarthritis, Sandra is improved, but not cured. Sandra’s Chronic Pericarditis

38. The Seattle Arthritis ClinicThe Pericarditis Charts on My Desk When I Left Seattle…

39. Seattle Arthritis Clinic18 Chronic Pericarditis Cases • Ages:16 to 83 years • Gender:11 women and 7 men • Sero-positive Conditions:5 Antibodies: Anti-DNA, ANA, RNP, SS-A, SS-B Diagnoses: Systemic Lupus Erythematosis (1) Sjogren’s Syndrome (1) Undifferentiated Connective Tissue Disease (3) • Sero-negative Conditions:13 Antibodies: ALL NEGATIVE Diagnoses: Reactive Spondyloarthropathy (1) Psoriasis Spondyloarthropathy (2) Undifferentiated Spondyloarthropathy (8) No underlying disease identified (2)

40. Infections cantrigger reactive inflammatory pericarditis and arthritis. Sero-negative syndromes such as spondyloarthritis are very common. 3)The 5 Principles of Rheumatology Diagnosis will help you rule out infections, and identify the patterns of rheumatic disease. Sandra’s‘Take Home Message’

41. THANK YOU University of Washington and Northwest Medical Center