A Case of Behçet's Disease with Pericarditis, Thrombotic Thrombocytopenic Purpura, Deep Vein Thrombosis and Coronary Artery Pseudo Aneurysm
A 37-year-old woman was transferred to our emergency room with the history of syncope. Three days previously, she experienced chest discomfort, dyspnea, palpitations and a low grade fever, but had no relevant past medical history or co-morbidity. At presentation her blood pressure was 80/50 mmHg, pulse rate 76/min, respiratory rate 36/min and body temperature 37.5℃. The physical examination was remarkable for: dyspnea, orthopnea, absence of normal heart sounds, and jugular vein dilatation; Additional tests revealed: low voltage ECG, sinus tachycardia and a large cardiac shadow on X-ray. Laboratory findings showed a pH of 7.37, PaCO2 18.8 mmHg, PaO2 88.5 mmHg, HCO3- 10.8 mmol/L on ABGA, hemoglobin 9.2 g/dL, white blood cell of 12900/µL, and platelet 476000/µL on CBC. ANA, rheumatoid factor, and VDRL were all negative and non- reactive. The patient had a suspected cardiac tamponade therefore a transthoracic echocardiography (TTE) was performed immediately and revealed moderate pericardial effusion and a hypokinetic LV at the anterior, anterolateral and apex portions of the heart (). Pericardiectomy with a window formation was performed to remove the effusion and hematoma. The drained fluid had a white blood cell 225 (PML 100%), red blood cell 90,000/µL LDH 1064U/L, and glucose 190 mg/dL; there were no organism on gram stain and culture. A class I cytology, indicating acute and chronic inflammation was revealed. On postoperative day 3, fever, dyspnea, oliguria and mental status changes developed and the patient deteriorated rapidly. A follow-up evaluation showed the serum hemoglobin to be 6.9 g/dL, white blood cell 14900/µL, platelets 18000/µL and AST, ALT and CPK of 272, 151, and 247 U/L, respectively. Blood urea nitrogen was 40.6 mg/dL, creatinine 4.9 mg/dL, LDH 2279 U/L, PT 11.6 seconds and aPTT of 32 seconds.\nA chest X-ray revealed a ground glass appearance in both lungs. The brain MRI was reported as normal. A peripheral blood smear was remarkable for schistocytosis, anisocytosis, normoblasts and a thrombocytopenia, suggestive of microangiopathic hemolytic anemia (). All of these findings were compatible with the diagnosis of thrombotic thrombocytopenic purpura (TTP). Treatment with methylprednisolone pulse therapy was started (1 g/day for 3 days). Investigation for the etiology of TTP revealed that the patient had suffered from severe recurrent oral and genital ulcers. The ulcers were reported to be as frequent as 3 to 4 per month and were exacerbated by menstruation after the age of 20. The patient also reported taking medications intermittently for painful skin indurations and eruptions as well as arthralgia. Considering the clinical symptoms in total the diagnosis, of Behcet's disease complicated by TTP and hemorrhagic pericarditis, was made. Treatment with methylprednisolone pulse therapy resulted in a dramatic response with improvements of the CBC, LFT, azotemia, and improved mental status. The patient went on to fully recover and returned to normal activities of daily living within a few days. Follow-up evaluation with TTE after complete recovery showed a normal right and left ventricular heart function and a minimal pericardial effusion. After discharge, the patient continued taking medication including colchicine 1.2 mg/day and prednisolone 5 mg/day. The patient continued to be followed.\nSix months after discharge, the patient required readmission to the hospital. She presented with a sudden onset left leg swelling and pain that was diagnosed as a left proximal femoral vein thrombosis by duplex scan. There were no associated signs or symptoms such as dyspnea or fever. A venogram demonstrated that a filling defect obstructed the left proximal femoral vein. the patient was treated with catheter directed thrombolysis using urokinase, thrombectomy and venoplasty and then started on combination anticoagulation therapy (). Six months after maintenance therapy with coumadin, colchicines, and sulfasalazine was started, a homogenous soft tissue mass was found, incidentally, on routine laboratory and radiologic follow up. The soft tissue density was attached to the left perihilar area by the pulmonary conus (). The laboratory tests showed a hemoglobin of 11.1 g/dL, platelet 324000/µL, WBC 6000/µL, ESR 39 mm/H, CRP 1.59 mg/dL, albumin 4.5 g/dL, LDH 503 U/L, BUN 11.8 mg/dL, creatinine 1.0 mg/dL, PT 29.7 second, INR 2.95.\nChest CT angiography and cardiac MRI revealed a 5x5cm sized round aneurysmal sac between the left atrium and left ventricle adjacent to the proximal pulmonary artery, but no definite stalk from the origin could be found (). A doppler echocardiography demonstrated holosystolic ejection flow from the surface of the left ventricle (). Despite the fact that the origin of aneurysmal stalk could not be identified, the pressure gradient calculated by the color doppler flow from the aneurysmal stalk to lumen was about 9 mmHg. The dilated vascular sac appeared to be a pseudo aneurysm with a coronary artery origin. A Tc99m sestamibi scan was performed to investigate the possibility of myocardial ischemia. The scan revealed a reversible perfusion defect on the antero-septal wall (). Subsequent coronary angiography allowed us to confirm the diagnosis of a pseudo aneurysm from the mid-left anterior descending artery (LAD), which interrupted distal flow by creating indentations and diffuse stenotic lesions along the whole length of the right coronary artery (RCA). In addition, a well developed collateral circulation was observed from the LAD and the left circumflex artery (LCX). An emergency operation was planned for aneurysmal resection and coronary artery bypass grafting (CABG). However, because of the size and thus the risk of rupture, we opted instead for balloon angioplasty with a U-pass and graft stent. Implantation proceeded using a Graftmaster 3.0×19 mm at the mid LAD the origin of pseudo aneurysm (). Because of the compromised distal flow of the RCA, the LAD would be of no advantage to the outcome of the CABG. If packed completely, it was expected that the aneurysmal sac would not grow any more and would regress by resorption and fibrosis. The administration of high dose glucocorticoid therapy (prednisolone 1 mg/kg/day) with azathioprine (150 mg/day) in addition to the mechanical intervention were both highly effective management as demonstrated by a follow-up TTE and coronary angiography which showed no further diversion of flow from the LAD and regression of the aneurysmal size to 3.9×3.8 cm from 5×5 cm Initially. and a preserved heart function (LVEF 60%) (). Six months after discharge, the patient was asymptomatic and only a vestige of the aneurysm was evident by routine chest X-ray ().