Calm before the Storm: Cholesterol Pericarditis in Rheumatoid Arthritis Nadil Zeiadin MD, Matthew Woo MD, Gursimran Chandokee MD Department of Internal Medicine Queen s University Kingston, ON
Learning Objectives Recognize cholesterol pericarditis as an extraarticular complication of rheumatoid arthritis Diagnose cholesterol pericarditis Recognize that quiescent intraarticular rheumatoid arthritis does not preclude patients from developing life threatening extraarticular complications
A 78 year-old man presents to the emergency room with a 4-day history of general malaise associated with rigors, diaphoresis, nausea, vomiting and pleuritic chest pain
100/59mmHg, 108bpm, RR18, O2 96% RA, T38.1 GCS15, Unwell looking, dry MM/axilla JVP 15cm ASA, +Kussmaul sign Normal auscultation Bibasilar Crackles Rheumatoid nodules
PMHx BPH, OSA, RA, HTN, DM2 Med Adalimumab, Alendronate/Cholecalciferol, Azathioprine, Calcium Carbonate, Metformin, Prednisone, Rabeprazole, Tamsulosin, Valsartan/HCTZ SHx Married, 2 children Retired school custodian 25py previous smoker FHx Lung Ca brother, sister (both heavy smokers) Father ACS
RA disease activity over time 10 9 8 1-yr hx of joint pain/swelling, RA nodules RF+, anti-ccp +ve Dx: RA MTX and Prednisone 7 DAS28 Score 6 5 4 3 2 1 0 Oct-07 Jan-08 Apr-08 Jul-08 Oct-08 Jan-09 Apr-09 Jul-09 Oct-09 Jan-10 Apr-10 Jul-10 Oct-10 Jan-11 Apr-11 Jul-11 Oct-11 Jan-12 Apr-12 Jul-12 Oct-12 Jan-13 Apr-13 Jul-13 Oct-13
10 9 Leflunomide IM Gold Etanercept DAS28 Score 8 7 6 5 4 3 2 1 Adalimumab & Azathioprine 0 Oct-07 Jan-08 Apr-08 Jul-08 Oct-08 Jan-09 Apr-09 Jul-09 Oct-09 Jan-10 Apr-10 Jul-10 Oct-10 Jan-11 Apr-11 Jul-11 Oct-11 Jan-12 Apr-12 Jul-12 Oct-12 Jan-13 Apr-13 Jul-13 Oct-13
10 9 8 7 Quiescent disease DAS28 Score 6 5 4 3 2 1 0 Oct-07 Jan-08 Apr-08 Jul-08 Oct-08 Jan-09 Apr-09 Jul-09 Oct-09 Jan-10 Apr-10 Jul-10 Oct-10 Jan-11 Apr-11 Jul-11 Oct-11 Jan-12 Apr-12 Jul-12 Oct-12 Jan-13 Apr-13 Jul-13 Oct-13
DAS28 Score 10 9 8 7 6 5 4 3 2 1 Bilateral pleural effusions Small pericardial effusion 0 Oct-07 Jan-08 Apr-08 Jul-08 Oct-08 Jan-09 Apr-09 Jul-09 Oct-09 Jan-10 Apr-10 Jul-10 Oct-10 Jan-11 Apr-11 Jul-11 Oct-11 Jan-12 Apr-12 Jul-12 Oct-12 Jan-13 Apr-13 Jul-13 Oct-13
10 9 8 7 A 78 year-old man presents to the emergency room with a 4- day history of general malaise associated with rigors, diaphoresis, nausea, vomiting and pleuritic chest pain 6 5 4 3 2 1 0 Oct-07 Jan-08 Apr-08 Jul-08 Oct-08 Jan-09 Apr-09 Jul-09 Oct-09 Jan-10 Apr-10 Jul-10 Oct-10 Jan-11 Apr-11 Jul-11 Oct-11 Jan-12 Apr-12 Jul-12 Oct-12 Jan-13 Apr-13 Jul-13 Oct-13
Serum Pericardial Fluid Hemoglobin (g/l) 118 - Nuclear cell (cells/mm 3 ) - 36 Leukocytes (x10 9 /ml) 12 - Neutrophils (x10 9 /ml / %) 10.9 95% Monocytes (x10 9 /ml / %) 0.7 - ESR (mm/hr) 140 - Glucose (mmol/l) 8.4 0.4 Creatinine (Umol/L) 271 - Urea (mmol/l) 17.4 - Total protein (g/l) 76 38 Albumin (g/l) 28 Sparkling gold paint Amylase (U/L) 51 17 LDH (U/L) 120 10580 Cholesterol (mmol/l) 3.26 6.5 Triglycerides (mmol/l) 0.93 0.56 CRP (mg/l) >340 -
Giemsa stain x 40 Cholesterol crystals with characteristic rhomboid plates and notched corners Giemsa stain x 400 Raposo L, Joao Andrade M, Ferreira J, et al. Cholesterol Pericarditis Relapsing Pericardial Effusion in a Patient with Rheumatoid Arthritis. Rev Port Cardiol 2005 ; 24 (5) : 737-745
Cholesterol Pericarditis RARE type of pericardial effusion rich in LDL-cholesterol First described in 1919 only 77 cases in literature ( 15 related to RA) Etiology: Idiopathic (>55%) RA TB Hypothyroidism (myxedema) Other less common causes: hypercholesterolemia, hemopericardium, acute MI, heart failure refractory to diuretic therapy Diagnostic Criteria 1. Presence of crystals of cholesterol OR 2. Elevated concentration of cholesterol in the pericardial fluid (above 70mg/dL or 3.9mmol/L)
Cholesterol Pericarditis in RA Pericardial disease most common cardiac manifestation of RA Approx 40-50% in post-mortem studies Characterized by chronic relapsing, usually large, pericardial effusions Typically asymptomatic, with only 2% becoming clinically significant Potentially fatal sequelae: cardiac tamponade and constrictive pericarditis Early diagnosis and treatment critical: mean delay of 10 months from first cardiac symptom Echocardiography reliable first investigation for any RA patient with suspicious symptoms Definitive diagnosis relies on pericardial fluid sampling Occurrence of rheumatoid pericarditis does not seem to correlate with disease duration or severity and may even occur when the disease is quiescent CP is typically associated with seropositive, extra-articular, nodular type
Pericardial cell membranes, necrotic rheumatoid nodules, lysis of red blood cells, lymphatic obstruction? Effusion
Management Surgery is the only definitive treatment option Radical vs limited pericardiectomy Concomitant medical management may provide further benefit Moderate dose glucocorticoids (15-20 mg) DMARD therapy Cyclophosphamide or additional immunosuppresion No clinical trials conducted to assess benefit of standard drug regimes on cardiac manifestations of rheumatoid arthritis Most studies concluded that pericardiectomy should be performed as early as possible to avoid deleterious haemodynamic consequences close long-term follow-up is imperative due to the high likelihood of re-accumulation
Back to our case Continued adalimumab, azathioprine Prednisolone 40mg IV, then 20mg PO Recurrent constrictive pericarditis necessitating definitive pericardiectomy
Conclusion Cholesterol pericarditis rare extraarticular manifestation of RA Need for clinicians to be vigilant for extra-articular manifestations of rheumatoid arthritis even in the presence of quiescent joint disease Definitive management of cholesterol pericarditis is surgical Further studies are required to elucidate optimal medical management strategy with regards to cardiac manifestations of RA
Barcin C, Yalcinkaya E, Kutsi Kabul H. Cholesterol pericarditis associated with rheumatoid arthritis: A rare cause of pericardial effusion. International Journal of Cardiology. 2013; 166: e56 e58 Fernandes F, Vieira GS, Arteaga E, et al. Cholesterol Pericarditis. A Specific but Rare Cause of Pericardial Disease. Arq Bras Cardiol 2001; 76: 393-4 Jordan AD, Khan MEA, Hoey ET, et al. A Clinico-pathological Conference on Constrictive Pericarditis Secondary to Rheumatoid Arthritis: A Case Report with Expert Commentary and Review of the Literature. Heart, Lung and Circulation 2011; 20:24 29 Raposo L, Joao Andrade M, Ferreira J, et al. Cholesterol Pericarditis Relapsing Pericardial Effusion in a Patient with Rheumatoid Arthritis. Rev Port Cardiol 2005 ; 24 (5) : 737-745