Pathologic Quiz Case: An Unusual Complication of Systemic Lupus Erythematosus

Abstract

A 36-year-old Hispanic man with an 11-month history of systemic lupus erythematosus (SLE) was admitted to our hospital for shortness of breath and cough productive of white sputum, fever (38.9°C), and pain and stiffness in bilateral knees and elbows associated with warmth and swelling. The patient also complained of anterior chest wall pain for 1 week. Notably, the patient had stopped taking prednisone (prescribed dose, 30 mg daily) approximately 2 1/2 weeks prior to admission. His medical history was contributory for pneumococcal pneumonia (6 months prior to admission) and cerebral vascular accident of the right basal ganglion (4 months prior to admission). Physical examination was notable for right lung crackles, mild diffuse abdominal tenderness, and reduced range of motion in bilateral knee and elbow joints but without effusion. Chest x-ray film showed right middle lobe consolidation (Figure 1). The rapid plasmin reagin test (RPR) was negative. The patient was treated with intravenous solumedrol and hydrocortisone, antibiotics, and ibuprofen. The patient's pneumonia resolved shortly thereafter, but he subsequently developed diffuse abdominal pain with evidence of peritonitis and progressive decline in hemoglobin (to 46 g/L) despite transfusion of packed red blood cells. An exploratory laparotomy was performed. Intramesenteric and retroperitoneal hemorrhage was found, necessitating partial pancreatectomy and splenectomy. The hospital course was relatively stable until postoperative day 7 when the patient was found to have pulseless electrical activity. Resuscitation efforts were unsuccessful. A complete autopsy was performed.The autopsy revealed 400 g of freshly clotted blood within the pericardial sac and 2 transverse intimal tears (Figure 2, arrows) in the ascending aorta with medial dissection and rupture into the pericardial sac. The aorta was not dilated with a circumference of 72 mm (reference range, 60–75 mm) and thickness of 1.5 mm (reference, 1.5 mm). The cardiac valves had a normal gross appearance. Sections of the ascending aorta revealed patchy mucoid-cystic necrosis (Figure 3, arrows) and dissection (Figure 3, open arrow) of the elastic media with proliferation of the overlying intimal layer. The adjacent vasa vasorum showed active vasculitis (Figure 4), but Gram, Gomori methenamine silver, periodic acid–Schiff, Steiner, and Giemsa stains failed to reveal microorganisms. Multiple cortical infarcts were noted in the kidneys bilaterally. The resected spleen and pancreas showed focal hemorrhage and infarction grossly. Histologically, a diffuse active and healed fibrinoid necrotizing vasculitis involving medium-sized arteries, small arteries and arterioles with focal thrombi, and multiple microscopic aneurysms was noted in kidneys, pancreas, spleen, and pleura. Focal proliferative lupus glomerulonephritis was also evident. A large subacute infarct was found in the right basal ganglion and a small subependymal infarct was found in the left basal ganglion; histologic sections revealed remote infarction. The adjacent cerebral parenchyma and overlying ependyma revealed no discrete inflammation or vasculitis.What is your diagnosis?Prior to the era of antibiotics, immunosuppression, and renal transplantation, both infections and renal failure accounted for the main mortality in SLE patients.1,2 An increase in cardiovascular morbidity and mortality has been encountered in patients with SLE following an improved overall survival from therapy for other complications; it is currently the third most common cause of death in patients with SLE. However, aortic dissection associated with vasculitis of the vasa vasorum in SLE is extraordinarily rare. In a search of the English-language literature, we found only 1 previously reported case of aortic dissection with vasculitis of the vasa vasorum complicating SLE.3 There were an additional 6 cases of aortic dissection complicating SLE without a description of vasculitis4–9; all were single case reports. Aortic dissection as a complication of SLE occurs more commonly in women and at a young age (28–56 years; median, 34 years; M:F, 2:6) in patients with systemic disease and a history of lupus renal disease. All of the reported patients had received corticosteroid therapy for a relatively long period of time.3–9 The patient who had SLE associated with vasculitis was normotensive, as was the patient in this case, while those without vasculitis had a history of hypertension. Most patients manifested chest pain as the presenting symptom, but atypical manifestations, such as seizure or syncope, acute renal failure or severe hypertension, dyspnea, fever, or chill, interscapular pain, abdominal pain or diarrhea, or sudden death, have all been noted. Patchy mucoid degeneration, cystic degeneration, or medial necrosis in aortic wall was present as part of or coexisted with the dissection in some cases4,6,8,9 and the present case. Several patients showed atherosclerotic changes, aortic aneurysm, or aortic dilatation.3,7,9The mechanism of aortic dissection in SLE seems to be multifactorial. The long-term corticosteroid therapy required for SLE has increased the incidence and severity of hypertension and promoted atherosclerosis.8 In addition, corticosteroid therapy may interfere with repair of vessels due to inhibition of the formation of chondroitin sulfate, granulation tissue, and collagen.6 The variably contributing factors of hypertension, corticosteroids, and arteritis may induce vascular damage to the aortic wall and produce the rare but fatal manifestation of aortic dissection.3,8Our patient was a 36-year-old man with a relatively short history of SLE and short period of corticosteroid therapy (<12 months) and no history of hypertension. The patient had a diffuse active and healed fibrinoid necrotizing vasculitis involving medium-sized arteries, small arteries, and arterioles. A particularly active vasculitis of the vasa vasorum affected a localized segment of the aorta leading to medial destruction, aortic dissection, and rupture into the pericardium. Corticosteroid therapy may have also played a role by inhibiting repair of the damaged vascular wall or interfering with connective tissue integrity. His poor compliance with medication may have also contributed to the presence of widespread active vasculitis.Aortic dissection complicating SLE is an infrequent event, and it currently is impossible to determine the incidence of this potentially lethal complication. A high degree of suspicion in these patients is especially important as symptoms may be atypical. Early recognition of aortic dissection is important for successful surgical intervention to be achieved.10 In the present case the diagnosis of aortic dissection was not suspected antemortem.