A 64-year-old man was referred to internal medicine consultation with a 6-month history of anorexia, weight loss (22 % of body weight) and progressive pain in the abdomen radiating to the lower back. He had a past strong medical history of cardiovascular disease with systemic hypertension, coronary disease and open surgical repair of a ruptured ascending thoracic aortic aneurysm 4 years before. Relevant medical background also included two episodes of deep venous thrombosis (DVT) in the last year. On physical examination there was an abdominal pulsatile mass. Initial laboratory findings showed microcytic anemia (8.9 g/dL; N 12-13 g/dL), elevated erythrocyte sedimentation rate (133mm 1st hour; N<20mm 1st hour) and C-reactive protein (13 mg/dL; N<5 mg/L), and renal dysfunction (creatinine 2.8 mg/dL; N<1 mg/dL) without proteinuria or red blood cells casts. The serum proteinogram showed polyclonal hypergammaglobulinemia with raised level of IgG (1945 mg/dl) but normal serum IgG4 concentrations. Serological testing for EBV, CMV, HSV, parvovirus B19, HIV, hepatitis B and C, syphilis and tuberculosis skin testing were negative. Immunological studies revealed persistent high levels of IgM anticardiolipin (aCL) antibody (159.5 MPL, positive >11) and IgM β2-glycoprotein (B2G) antibody (168 U/mL, positive >11), with negative lupus anticoagulant, measured on two occasions 4 months apart. Workup for other autoimmune disorders was unremarkable, including negative anti-neutrophil cytoplasmic antibodies and antinuclear antibody panel. A thoracoabdominal computed tomography (CT) scan was made and showed an aneurysm of the infrarenal abdominal aorta with 4.1 cm in diameter and bilateral hydronephrosis ( figure 1 -C). In addition, there was a 'sleeve' of abnormal material of soft tissue density surrounding the right brachiocephalic trunk, aortic arch, descending aorta, abdominal aorta and common iliac arteries (Figure 1 -A and 1-B
AbstractChronic periaortitis (CP) is a rare fibro-inflammatory disease characterized by periaortic fibrosis and/or aortic aneurysms formation, mostly localized in retroperitoneum and occasionally in the mediastinum. Recent studies have shown its common association with autoimmune diseases, therefore autoimmunity has been proposed as a contributing factor. Herein, we describe the second case in the literature of CP associated with antiphospholipid syndrome. A 64-year-old man with history of open surgery for inflammatory thoracic aortic aneurysm and recurrent deep vein thrombosis was referred for abdominal pain and weight loss in the last 6 months. Further investigation revealed elevated acute-phase reactant levels, positive antiphospholipid autoantibodies, radiological and histological evidence of periaortic fibrosis and inflammation causing abdominal aortic aneurysm and ureteral obstruction. Diagnosis of CP and antiphospholipid syndrome were made and steroid therapy was implemented with clinical and radiological improvement. The present report further supports the potentially immune-mediated origin of ...