by Michael Westerman, M.D. Postdoctoral Fellow, Division of Geriatric Medicine, JHU
Meliha Shah, M.D. Resident in Internal Medicine
- History of Present Illness
- Physical Examination
- Laboratory Studies
- Differential Diagnosis
- Hospital Course
The patient is a 67 year-old woman with a 20-year history of rheumatoid arthritis who presented with fatigue and dyspnea. One month before her admission to Johns Hopkins, she developed pleuritic chest pain and was treated by her primary care provider for “bronchitis” with Amoxicillin. Over the next five days her pain worsened and she became short of breath. She was admitted to another hospital, where she was found to be in new-onset atrial fibrillation with an elevated troponin level of 4.4 ng/ml. Echocardiography showed a small pericardial effusion and adenosine thallium testing was negative for cardiac ischemia. Her dyspnea improved after diuresis, and she was discharged home. Less than two weeks later, she was readmitted to the same hospital with worsening dyspnea. Her pericardial effusion was somewhat larger on repeat echocardiography, and she remained in atrial fibrillation. She was anticoagulated with warfarin and again discharged to home.
A week later, she was admitted to the Medicine service at Johns Hopkins with worsening dyspnea. A 12-lead ECG showed atrial fibrillation with a controlled ventricular rate and low voltage in the limb and precordial leads.
Using pulsed-wave Doppler at the level of the mitral valve, there is exaggerated inspiratory and expiratory variation (>50%) of the mitral valve inflow E waves (arrows), representing echocardiographic evidence of pericardial tamponade physiology. Patient was in sinus rhythm during this study.
She was transferred to the Cardiac Intensive Care Unit for observation. A right heart catheter was placed, revealing high pulmonary artery pressures and an increased pulmonary capillary wedge pressure but not equalization of diastolic pressures.
The following day, however, she became more dyspneic and tachycardic, and repeat echocardiography showed further increase in the size of the pericardial effusion with right ventricular diastolic collapse. She underwent emergent pericardiocentesis with removal of 350cc of turbid brown fluid. Her dyspnea and tachycardia responded immediately to this intervention.
Past Medical History
- Rheumatoid arthritis - She was diagnosed with RA and treated by a local rheumatologist 20 years ago. Past treatments included oral gold, penicillamine, two short bouts of oral prednisone, multiple intraarticular steroid injections. At the time of admission she was receiving azathioprine and an NSAID. She had never been treated with Methotrexate, Sulfasalazine, or Minocycline. She had had no joint surgeries other than rotator cuff repair and carpal tunnel releases. Her history was positive for olecranon nodules in the past and dry mouth but not dry eyes. She denied a history of vasculitis, pulmonary nodules or pleuropericarditis.
- Hypertension for one year.
- Severe anxiety treated with chronic benzodiazepines.
- s/p total abdominal hysterectomy
She had never smoked and did not drink alcohol. She lived with her husband and daughter and was active until her current illness.
Negative for rheumatoid arthritis or connective tissue diseases.
IV contrast dye
Medications on Admission
- Azathioprine 50mg TID.
- Indomethacin 50mg TID.
- Amiodarone 400mg TID.
- Digoxin 0.125mg QD.
- Metoprolol 25mg TID.
- Cardizem 300mg QD.
- Lasix 40mg QD.
- Premarin 0.625mg QD.
- Doxepin 50mg BID.
- Xanax 0.5mg TID.
- Axid, Darvocet, Tylenol #3 and Meclizine PRN.
- Ciprofloxacin two weeks of each month prophylactically for recurrent UTIs.
- Blood pressure was 150/70 with heart rate of 110 bpm. Respiratory rate was 15-20.
- Pulsus paradoxus was 20mmHg and there was jugular venous distension to the angle of the mandible
- Heart rate was tachycardic with an irregular rhythm and distant heart sounds. There were no rubs or murmurs.
- Lungs had scattered crackles but no wheezes.
- Abdomen was obese with mild diffuse tenderness but no rebound.
- Extremities had 2+ pulses. There was 2+ pitting pretibial edema bilaterally.
- Skin had no vasculitic rashes or nodules.
- Musculoskeletal exam showed full range of motion (ROM) at the right shoulder; the left shoulder had decreased abduction and external rotation. The elbows had full ROM with no swelling. Wrists had decreased ROM but no swelling or tenderness. MCP and PIP joints had full ROM with no synovitis or deformities. Hips had full ROM. The right knee had full, painless ROM without swelling or ligamentous laxity. The left knee exhibited bony hypertrophy, crepitus and limited ROM of -5 to 100 degrees. Ankles were normal. MTPs were subluxed and swollen but nontender.
- Sodium 135. Potassium 4.3. Blood glucose 130. Hemoglobin AIC 6.4%.
- Total protein 6.0. Albumin 2.7. Liver panel – normal. TSH 1.2.
- Creatine Kinase 30 IU/L. Troponin < 0.01 ng/ml.
- WBC 12,300 with 84% neutrophils, 5% lymphocytes and 11% monocytes. Hematocrit 26.2% with MCV 91.6. Platelets 515,000.
- C3=112. C4=21. FDP – not present. Ferritin 509. Haptoglobin 430 ng/dl.
- ESR 131. Serum RF positive at 1:640 and1280 IU.
- Hep B sAg – negative
- Pericardial fluid analysis: WBCs 103,000 with 99% polys and 1% monos; RBCs17,500 RBCs. Glucose = 2mg/dl. Protein = 4.7 g/dl. LDH = 20,400 IU/L. Cytology showed acute and chronic inflammation but no malignant cells. Gram stain was negative.
- PPD with anergy panel showed her to be anergic.
- [ ] Congestive heart failure
- [ ] Rheumatoid pericarditis
- [ ] Bacterial pericarditis
- [ ] Malignant pericarditis
Her pericardial effusion was thought to be secondary to her longstanding RA, since an exudative effusion with very low glucose concentration is a hallmark of rheumatoid serositis. The presence of red blood cells in the fluid suggested that she had bled into her effusion after starting anticoagulation with warfarin. Negative aerobic and anaerobic bacterial culture, negative AFB stains and culture, and negative KOH prep and fungal cultures confirmed that there was no infectious etiology. Chest CT showed a moderate pericardial effusion but no masses or lymphadenopathy suggestive of malignancy.
Rheumatology was consulted after the pericardiocentesis and recommended initiation of prednisone 30 mg bid. A marked decrease in the size of her effusion over the next 72 hours was documented by repeat echocardiography, and she had no further complaints of dyspnea. She briefly converted back to sinus rhythm following pericardiocentesis but then atrial fibrillation/flutter recurred and persisted. Amiodarone and a low-dose beta blocker were started for rate control. In view of the fact that the pericarditis occurred while on azathioprine, it was felt to be ineffective for management of extraarticular RA and was discontinued; methotrexate was substituted.
She was discharged home without any symptoms of dyspnea, palpitations or lightheadedness.
Autopsy studies during the last century have shown that cardiac involvement with rheumatoid arthritis (RA) is common and can include granulomas or nodules in all four cardiac valves, local or diffuse myocarditis, healed or subacute arteritis and chronic endocarditis. The postmortem incidence of pericarditis in patients with RA is reported to be in the range of 11 to 50%. During the last few decades, echocardiography has allowed the antemortem detection of pericardial effusions and other sequelae of pericarditis in about a third of patients with RA. Nevertheless, symptomatic rheumatoid pericarditis, with cardiac tamponade or constrictive pericarditis, is uncommon. Escalante et al. found that 12 out of 960 patients (1.25%) admitted to a hospital over 11.5 years for complications of RA had clinically-apparent pericarditis, and 5 of these 12 had cardiac tamponade physiology with elevated jugular venous distension and/or pulsus paradoxus on exam in the presence of pericardial effusion or thickening. Surgical and non-surgical series have shown that RA is one of the least common etiologies of cardiac tamponade and constrictive pericarditis.
Symptomatic rheumatoid pericarditis tends to occur in older patients with longstanding disease, but there is significant variability in the age, duration of disease and extent of extra-articular disease in these patients. (see American Colloge of Rhematology Highlights) The pericardial effusions usually have the characteristics of an exudate with high levels of protein and lactate dehydrogenase and extremely low glucose concentrations; cellular content is variable but tends to be higher than 2,000/ul with a predominance of neutrophils. Because the pericardial fluid can be loculated and difficult to aspirate, pericardiocentesis should be reserved for relief of life-threatening cardiac tamponade. Treatment often includes corticosteroids, but data proving the efficacy of this approach are sparse. In the case of cardiac compression (constriction and tamponade) and chronic pericarditis, the preferred long-term treatment is pericardial resection. Without surgical intervention, the two-year mortality of rheumatoid pericarditis can be in excess of 50%, but these deaths were frequently attributed to causes other than recurrent cardiac tamponade.
Our patient had classic rheumatoid pericarditis with high white cell count, elevated LDH and profoundly low glucose concentration in the pericardial fluid. Her articular disease was surprisingly mild and largely inactive but marked elevations in inflammatory indices (sedimentation rate, ferritin and haptoglobin) and marked anemia and thrombocytosis were all consistent with active systemic disease. Her dyspnea responded effectively to pericardiocentesis in the short-term. Lack of reaccumulation of the pericardial effusion was presumably due to corticosteroid and/or, methotrexate treatment.
- Escalante A, Kaufman RL, Quismorio FP, and Beardmore TD. Cardiac Compression in Rheumatoid Pericarditis, Sem Arthritis Rheum, 20(3): 148-163, 1990.
- Nomeir A-M, Turner R, Watts E, Smith D, West G, and Edmonds J. Cardiac Involvement in Rheumatoid Arthritis, Annals of Internal Medicine 79: 800-806, 1973.
- Prakash R, Atassi A, Poske R, and Rosen KM. Prevalence of Pericardial Effusion and Mitral-Valve Involvement in Patients with Rheumatoid Arthritis Without Cardiac Symptoms, New Engl J Med 289(12): 597-600, 1973.
- Thadani U, Iveson JMI, and Wright V. Cardiac Tamponade, Constrictive