A 43-year-old Japanese woman with peripheral large vessel vasculopathy associated with systemic lupus erythematosus. She presented a 7-year history of progressive headaches and intermittent claudication, although she had no atherosclerotic risk factors...
Posted June 6,2019 in General Medicine.
Vasculopathy in systemic lupus erythematosus (SLE) is a rare form of vascular involvement characterized by arterial stenosis or occlusion, with the accumulation of immunoglobulins and complement in the arterial intima and no inflammatory change . Vasculopathy should be distinguished from vasculitis histopathologically, as vascular lesions in lupus kidney disease can be categorized as non-inflammatory vascular immune complex deposits (vasculopathy), inflammatory vasculitis, thrombotic microangiopathies, and degenerative disorders/arteriosclerosis . While previous studies have reported vasculitis in large vessels or vasculopathy in small vessels of the kidney in SLE, large vessel vasculopathy has not been reported.
Here we present, to the best of our knowledge, the first case of peripheral large vessel vasculopathy associated with SLE. The possibility of large vessel vasculopathy should be considered in a patient with lupus when the patient has peripheral arterial disease, despite having no atherosclerotic risk factors.
We made a diagnosis of large vessel vasculopathy associated with SLE based on the histopathological findings. Vascular involvements were only observed in the peripheral large vessel according to the Chapel Hill Consensus Conference nomenclature system . We initiated 120g/day of beraprost sodium and 200mg/day of cilostazol therapy orally. At 6months after discharge, re-examination by ultrasonography revealed recanalization in our patients left posterior tibial artery.
To the best of our knowledge, this is the first report of peripheral large vessel vasculopathy associated with SLE and demonstrates two important points.
First, vasculopathy, as well as vasculitis, may occur in the large vessel. Although several reports showed large vessel involvements in SLE, all of them demonstrated vasculitis or thrombosis of large vessel [4,5,6,7,8]. Takagiet al.reported a case of SLE with non-dissecting aneurysm that was successfully resected, replaced with a tube graft, and proven to be active aortitis . In this patient, focal calcification and atheroma were found within the thickened intima, which contained activated T and B lymphocytes in a perivascular lesion. However, some previous publications might include cases with large vessel vasculopathy especially when they were diagnosed only with angiography because vasculopathy is often confused with vasculitis. In contrast, the present case showed only a thickened intima with immunoglobulins and complement deposition, without any inflammatory lymphocyte infiltration or atherosclerotic change, which are typical features of vasculopathy.
Second, the present case had vasculopathy in the large vessel, not the small or medium vessel. Lupus vasculopathy is commonly found in the kidney. Pathological findings in renal lupus vasculopathy are mainly present in small vessels, such as the pre-glomerular arterioles and small arteries . In addition, although cases of extra-renal vasculopathy in lupus were rarely reported, all were shown to involve the small vessels [9,10,11]. In contrast, the present case was demonstrated to have peripheral large vessel vasculopathy based on temporal artery biopsy.
Several limitations should be noted. First, a previous use of prednisolone might modulate pathological findings. Differential diagnoses of large vessel vasculopathy include atherosclerosis and healed vasculitis. Although accelerated atherosclerosis is a common complication in SLE , the pathological findings of the present case did not show atherosclerotic changes such as fatty streak or atheroma [13,14]. In addition, healed temporal arteritis was less likely to occur in this case as there were no neovascularizations of the media or loss of the internal elastic lamina . Second, we could not speculate regarding the incidence or prevalence of vasculopathy based on the nature of the case reports. Some peripheral large vessel vasculopathy may, however, remain unrecognized among patients with SLE with peripheral arterial diseases. Thus, further studies are needed to determine the prevalence of vasculopathy among patients with SLE with peripheral arterial disease.
The present case was treated without immunosuppressive therapy. Although previous cases of lupus vasculopathy were mainly treated with immunosuppressive therapy because of coincidental active lupus involvement or its hypothesized mechanism, it has not been established whether conventional immunosuppressive agents are effective [1,10]. Sugimotoet al.reported a case of renal lupus vasculopathy without active glomerular lesions successfully treated without immunosuppressive therapy . The patient, who had a previous medical history of central nervous system lupus, developed acute renal infarction and multiple arterial stenoses in the interlobular arteries. Because no active inflammatory changes were found during renal biopsy, they did not initiate immunosuppressive therapy, which resulted in the improvement of renal dysfunction. The present case also experienced improvement of arterial occlusion without immunosuppressive therapy. Further reports should be accumulated to determine whether immunosuppressive agents are needed to treat lupus vasculopathy without active inflammation.
In conclusion, peripheral large vessel vasculopathy may occur in patients with SLE. Physicians should consider the possibility of large vessel vasculopathy in a patient with lupus when the patient has peripheral arterial disease, despite having no atherosclerotic risk factors.