Antıphospholıpıd Syndrome Wıth Lupus Erythematosus Presentıng Wıth Myocardıal Infarctıon.

 Kubra Kaynar 1, Sukru Ulusoy 2, Semih Gul 3, Fatma Kılıcarslan 4, Funda Oztuna 5, Ali Ahmetoglu 6, Serdar Bedii Omay 7

1. Assistant Professor, Department of Nephrology, School of Medicine, Karadeniz Technical University.

2. Associate Professor, Department of Nephrology, School of Medicine, Karadeniz Technical University.

3. Assistant Professor, Department of Nephrology, School of Medicine, Karadeniz Technical University.

4. Assistant Professor, Department of Internal Medicine, School of Medicine, Karadeniz Technical University.

5. Assistant Professor, Department of Chest Disease, School of Medicine, Karadeniz Technical University.

6. Associate Professor, Department of Radiology, School of Medicine, Karadeniz Technical University.

7. Professor, Department of Hematology, School of Medicine, Karadeniz Technical University.

Correspondence to : Dr. Kubra Kaynar, Karadeniz Teknik Üniversitesi, Tip Fakültesi, Nefroloji Bilim Dali, 61080 Trabzon, TURKEY

Fax number                  : +90 462 325 05 18

SMJ 2007 52(2): 56

  

Abstract 

Arterial and venous thrombosis is a prominent feature of antiphospholipid syndrome together with antiphospholipid antibodies. We report generalised thrombosis in a 28 years old male patient with antiphospholipid syndrome associated with lupus erythematosis. Firstly the patient had myocardial infarction. Eight months later he started to complain about oedema which was found to be secondary to nephrotic syndrome. In his third hospital day he developed pulmonary emboli. Tomographic angiography revealed left renal vein thrombosis, portal vein thrombosis, right renal infarct, pulmonary emboli. His tests for antinuclear antibody, anti dsDNA antibody and antiphospholipid antibody IgM were positive. After anticoagulation therapy and immunosupressive therapy he could have been disconnected from mechanical ventilator and his oedema nearly disappeared. This case clearly highlights the fact that antiphospholipid syndrome with systemic lupus erythematosus can present with myocardial infarction in young patients. 

Key words: Myocardial infarction, antiphospholipid syndrome,systemic lupus erythematosus.

  

Introduction 

Arterial and venous thrombosis is a prominent feature of antiphospholipid syndrome together with antiphospholipid antibodies and miscarriages of pregnancy.1 Antiphospholipid antibodies are associated with autoimmune diseases such as systemic lupus erythematosus (SLE), but when they occur in isolation, this is known as primary antiphospholipid syndrome (PAPS). The main antiphospholipid antibodies implicated in thrombosis and atherosclerosis are the anticardiolipin antibody, the lupus anticoagulant, and IgG antibodies against plasma phospholipid-binding proteins such as b2-microglobulin I and prothrombin.2,3

 

The mechanism for thrombosis in this syndrome is complex and not well understood. A mild thrombocytopenia is a common finding in antiphospholipid antibody syndrome (APS), though this does not seem to correlate with thrombosis.4 However there is in vitro evidence that the anticardiolipin antibody increases platelet adhesiveness.5

 

We report generalised thrombosis in a patient with antiphospholipid antibody syndrome associated with lupus erythematosis.

 

Case presentation

A 28 year old male patient presented to our department with fatigue and oedema since last month. He had anterior myocardial infarction 8 months ago. In his coronary angiography, there was 40% obstruction in his left anterior descending artery secondary to thrombus. He was treated medically with ramipril, acetylsalicylic acid and metaprolol. His renal function tests (including urinary protein excretion) were normal at that time. He had no complaint until he had progressive oedema worsening prior to presentation. On physical examination, his body temperature was 39°C, pulse was 96/min, blood pressure was 150/90 mmHg. Laboratory investigations on admission to our department confirmed the diagnosis of nephrotic syndrome (serum albumin 10g/l, serum LDL cholesterol 4.6mmol/l, urine protein-to-creatinine ratio of 20). Renal vein doppler was normal. The patient developed tachypnea and hypotension during his third hospital day while the renal biopsy was being planned. Clinical examination was unremarkable except decreased lung sounds in the basal zones. Arterial blood gas analysis revealed very low oxygen pressure (Po2: 45 mmHg). Chest x-ray showed bilateral pleural effusion. To rule out the possibility of pulmonary thromboembolism, computed tomographic pulmonary angiography was performed. In the distal part of left pulmonary artery and in the subsegmental branches of the right pulmonary artery multiple emboli were present (figure 1). Abdomen sections of tomography obtained in the meantime showed right renal infarct and left renal vein thrombosis and portal vein thrombosis (figure 2). The level of fibrin degradation products was elevated, to 12.48  (normal range, less than 0.5 µg/ml). The patient’s respiration was supported by mechanical ventilation. The heparin intravenous infusion (5000u bolus, 30000u/24 hour) was started. The laboratory results of  ANA, antidsDNA, anticardiolipin IgM came out as positive. Antithrombin III was decreased, to 16mg/dl (normal range ,22-39). As the patient was treated with heparin, a renal biopsy was cancelled and the patient was diagnosed as antiphospholipid  syndrome secondary to lupus (as he had multiple thrombosis and positive ANA, antidsDNA and anticardiolipin antibody test results). The pulse steroid and cyclophosphamide therapy (0.75gr/m2) was started. In 2 days the patient was disconnected from mechanical ventilation. His blood pressure normalised and fever subsided. The steroid dose was tapered to 1 mg/kg/day and after 3 weeks the albumin level increased to 20g/l without any albumin replacement. Oedema of the patient disappeared considerably. 

 

Discussion

The main complaint of our patient was oedema due to nephrotic syndrome. But he had myocardial infarction 8 months ago and thrombus was found in coronary angiography then. Acute myocardial infarction is rare in teenagers and young adults. The pathophysiology of infarcts is varied but not usually due to atherosclerotic plaque except for those with genetically predetermined or familial hyperlipidemias. There was no family history of cardiac diseases in our patient. Coronary thrombosis can be seen in hypercoagulable states such as in the nephrotic syndrome, antiphospholipid syndrome and protein S and factor XII deficiencies.6 Proteinuria associated with the nephrotic syndrome results in the loss of low molecular weight proteins, which in turn alters the concentration and activity of coagulation factors (factors IX, XI, XII are decreased due to urinary excretion and there is an increased synthesis of factors II,VII,VIII and fibrinogen resulting in raised blood levels). While the patient had myocardial infarction (MI), he had no proteinuria. He developed nephrotic syndrome nearly 8 months after he had MI. So nephrotic syndrome could not have been the aetiology of his MI. After his admission to our department, he developed bilateral pulmonary emboli, so immediate anticoagulation had to be started and tomographic angiography revealed multiple thrombosis in renal vein, portal vein and pulmonary artery, and renal infarct. Common risk factors for renal infarction include valvular heart disease, atheroembolic events. Hypercoagulopathy, systemic vasculitis, blunt trauma, collagen vascular disorders, vascular intervention procedures and endocarditis are other less common risk factors associated with renal infarct.7 Our patient was found to have APS secondary to SLE. So all these arterial and venous thrombosis and nephrotic syndrome were features of this syndrome. Since our patient had to be given anticoagulation therapy due to life threatening pulmonary emboli, renal biopsy could not be performed. Presence of serositis, antinuclear antibody, anti dsDNA antibody and renal involvement distinguished the diagnosis of SLE from PAPS (when antiphospholipid antibodies occur in isolation, it is known as primary antiphospholipid syndrome).

 

In conclusion, young adults with myocardial infarction should be investigated for SAPS (secondary antiphospholipid syndrome is known as antiphospholipid antibodies associated with autoimmune diseases such as systemic lupus erythematosus), and it should not be forgotten that antiphospholipid syndrome with SLE could present with myocardial infarction in male patients.

           

References 

  1. Hughes GR. Thrombosis, abortion,cerebral disease and the lupus anticoagulant. BMJ 1983;287:1088-9.     

  2. Vaarala O. Antiphospholipid antibodies and atherosclerosis. Lupus 1996;5:442-7.

  3. Vaarala O, Puurunen M, Manttari M et al. Antibodies to prothrombin imply a risk of myocardial infarction in middle aged men. Thromb Haemost 1996;75:456-9.

  4. Cuadrado MJ, Mujic F, Munoz E, et al. Thrombocytopenia in the antiphopholipid syndrome. Ann Rheum Dis 1997;56:194-6.

  5. Reverter JC, Tassies D, Font J,et al. Effects of human monoclonal anticardiolipin antibodies on platelet function and on tissue factor expression on monocytes. Arthritis Rheum 1998;41:1420-7.

  6. Osula S, Bell GM, Homung RS. Acute myocardial infarction in young adults: causes and management. Postgrad. Med J 2002;78:27-30.

  7. Bemanian S, Motallebi M, Nosrati SM. Cocaine-induced renal infarction: report of a case and review of the literature. BMC nephrology 2005;6:10-16.

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