Case Report

1. Case Report • 39-year-old white male, diagnosed with Rf+ rheumatoid arthritis at the age of 17, presented to his primary care physician with shortness of breath and intermittent nausea. • He had reduced his daily prednisone dose from 20 mg to 10 mg because of nausea, was on diclofenac 50 mg bid, tramadol 100 mg tid. Peter Härle et al, Department of Internal Medicine I, University of Regensburg, Franz-Josef-Strauss-Allee , 11, D-93042 Regensburg, Germany

2. In addition to steroid therapy, several different disease-modifying drugs were given over the years since diagnosis, including sulfasalazine, oral gold, chloroquine, methotrexate, and TNF-inhibitors. • A total of 16 orthopedic operations had been performed including excision of rheumatic nodules, tendon repair, and bilateral knee- and unilateral hip-replacement.

3. On exam, he showed signs of extensive rheumatoid arthritis, most marked on hand, foot, and shoulder joints as well as rheumatic nodules on both elbows. • HR regular at 105/min • BP 130/90 mm Hg • ESR 82 mm/h

4. A CT-scan and echo revealed a pericardial effusion (1.5 cm), a thickened pericardium (5 mm), and basal bilateral low-grade lung fibrosis. • Diuretic therapy and increased prednisone dose controlled his symptoms • The pericardial effusion was almost undetectable 4 weeks later.

5. Four months later, he was admitted to the hospital because of a sudden onset of abdominal pain. • A perforated NSAID/steroid-induced ulcer was diagnosed and the patient required emergency surgery. • During anesthesia, severe cardiovascular problems developed including low blood pressure, tachycardia, and pre-renal kidney failure. • A left and right heart catheterization was performed subsequently which showed a cardiac index of 2.4 l/min/m2, equalization of elevated left and right ventricular diastolic pressures.

6. Coronary angiography revealed a 75% obstruction of the diagonal branch. • An MRI-scan showed a thickened pericardium (5 mm), a small pericardial effusion, enlarged right atrium, and bilateral pleural effusions.

7. Patient was diagnosed with constrictive pericarditis without a hemodynamic relevant pericardial effusion. • He was referred to CT Surgery • Pericardectomy was suggested. • The patient was informed about the prognosis of this RA-associated complication but declined surgery and was discharged in improved physical condition.

8. Repeated hospitalizations were necessary because of clinically dominant right heart failure. • On his last admission, p/w cachexia, extensive edema, tachycardia of 122/min, blood pressure of 105/55 mm Hg, orthopnea, and ascites. • Follow-up heart catheterization revealed a reduced ventricular function with a cardiac index of 1.46 l/min/m2.

9. Surgical intervention was recommended repeatedly but the patient still declined any further procedures. • In the following weeks, the patient had three episodes of renal failure attributable to low median blood pressure (40–60 mm Hg) together with diuretic therapy • Intermittently required vasopressor medication. • The CT-scan of the chest did not show a hemodynamically relevant pericardial effusion.

10. Fig. 1.  This CT-scan was conducted without contrast because of recurring prerenal kidney failure. A thickened pericardium (~5 mm) could be seen next to a small pericardial and bilateral pleural effusion. The pericardial effusion did not seem to be of hemodynamic relevance

11. Pt was noted to be adrenally insufficient and have pancreatic insufficiency • In the following weeks, cardiovascular and renal functions were increasingly difficult to stabilize and intermittent dialysis was necessary. • He developed a DVT despite the use of prophylactic heparin and a bilateral pneumonia despite broad-spectrum antibiotic therapy. • The patient died in septic shock combined with multi-organ failure.

12. The patient died 2 years after the onset of extra-articular cardiac symptoms. • Pericarditis is a frequent extra-articular manifestation of rheumatoid arthritis showing a post-mortem prevalence of 30%–50%. • These findings correlate well with echocardiographic diagnosis in living patients. However, clinically relevant symptoms are rare with a prevalence of 0.06%–3% of all RA patients • This case demonstrates the devastating course of progressive constrictive pericarditis under sole medical therapy and emphasizes the importance of early radical pericardectomy to avoid progression of disease and secondary complications with fatal outcome.

13. Rheumatoid ArthritisJulie Schwartzman, MD

14. Rheumatoid Arthritis • A systemic, inflammatory polyarthritis that leads to joint destruction, deformity, and loss of function • Several potentially severe extra-articular manifestations • Pathology of RA involves the synovial membranes and periarticular structures of multiple joints, resulting in: • Pain • Swelling • Stiffness • Uncontrolled inflammation that can lead to irreversible damage and deformity • Functional limitation ACR Subcommittee on RA Guidelines. Arthritis Rheum. 2002;46:328–346; Goronzy JJ, Weyand CM. In: Klippel JH, et al, eds. Primer on the Rheumatic Diseases. 12th ed. Atlanta, GA: Arthritis Foundation; 2001:209–217; Anderson RJ. ibid. 218–225;Arnett FC, et al. Arthritis Rheum. 1988;31:315–324. I.2

15. ACR 1987 Classification Criteria For Rheumatoid Arthritis Patients Must have Four of Seven Criteria Morning Stiffness Lasting at Least 1 Hour* Swelling in 3 or More Joints* Swelling in Hand Joints* Symmetric Joint Swelling* Erosions or Decalcification on X-Ray of Hand Rheumatoid Nodules Abnormal Serum Rheumatoid Factor * Must Be Present at Least 6 Weeks

16. Epidemiology of RA • Prevalence 0.5 - 2% • 2 –3 times more prevalent in women • Increased prevalence with advancing age • 100,000 – 200,000 New Cases/yr • 4 –6 million current cases of RA

17. Mode of Onset • Monoarticular 21% • Oligoarticular 44% • Polyarticular 35%

18. Joint Involvement MCP, PIP Wrist Knees Shoulders Ankles Feet Elbows Hips Mean % of Patients 91 78 64 65 50 43 38 17 Site of Onset

19. Course of Disease • Clinical remission 10% • Intermittent 15 - 20% • Progressive 70 - 75%

32. Extra-articular Manifestations of Rheumatoid Arthritis

36. Sceritis

37. Secondary Sjögren’s Syndrome • SICCA • Pleuritis/ Pericarditis • Ro/La positive • Hypergammaglobulinemia

40. Vasculitis • Digital vasculitis • Cutaneous ulceration • Peripheral neuropathy • Mononeuritis multiplex

43. Pulmonary Involvement • Pleural Disease • Interstitial fibrosis • Nodules • Pneumonitis

45. Cardiac Involvement • Pericarditis • Myositis • Endocardial Inflammation • Conduction Defects

46. Articular Manifestations: Synovial Fluid Analysis • Straw colored to slightly cloudy • WBC 5000 – 25,000/ mm3 • Rheumatoid Factor • Elevated protein • Decreased glucose

47. Laboratory Presentation • Leukocytosis • Eosinophilia • Thrombocytosis • Mild Anemia • ESR > 30 mm/hr • Normal renal and hepatic function • Negative ANA • Rheumatoid Factor

48. Anti-Cyclic Citrullinated Peptide(CCP) Antibodies • High diagnostic specificity (>98%) and sensitivity • Presence in 65% of early RA, with the same specificity • Prognosis value linked to the most erosive forms

49. Seronegative polyarthritis Psoriatic Arthritis Reiter’s Syndrome Chondrocalcinosis Gout Behcet’s Syndrome Infectious Arthritis Thyroid Disease Malignancies Polymyalgia Rheumatica Hemochromatosis Differential Diagnosis

50. Baseline Evaluation • 43 yo AAF came to PMD, reports 4 mo. h/o bilateral wrist and knee pain. • Occasional swelling in wrists and “knuckles” • No PMH • Takes tylenol for pain w/o relief • BASELINE EVALUATION: Important questions