1/16. Primary antiphospholipid syndrome masquerading as diabetic retinopathy.BACKGROUND: Primary antiphospholipid syndrome (PAPS) is a thrombotic condition which characteristically includes ocular vaso-occlusive pathology. CASE: We present the case of a 47-year-old woman with PAPS presenting with retinal vascular leakage. OBSERVATIONS: Vascular leakage was an incidental finding on fundoscopy in a myopic Chinese woman, and initially this was diagnosed as diabetic retinopathy. However, a glucose tolerance test was negative. Subsequently, signs of retinal vaso-occlusion developed. Previous subclinical cerebral thrombosis, mild thrombocytopaenia and positive tests for antiphospholipid antibodies were consistent with the diagnosis of PAPS. Anticoagulation therapy with warfarin prevented further thrombotic episodes. CONCLUSION: The possibility of PAPS should be considered in patients presenting with retinal vascular disease without obvious vascular risk factors.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/16. Wegener's granulomatosis associated with antiphospholipid syndrome.We report a case of Wegener's granulomatosis (WG) with pulmonary hemorrhage also satisfying the criteria for antiphospholipid antibody syndrome (APS). This association has, to the best of our knowledge, never been described before. Pulmonary hemorrhage may be an early manifestation of several immune and idiopathic disorders such as ANCA-associated vasculitis. Several case-reports of APS patients with capillaritis have been described. A possible explanation is that microvascular thrombosis with subsequent increase in vascular permeability facilitates perivascular IgG and complement deposition leading to development of capillaritis. Whether the vascular disease is secondary to thrombosis or vasculitis or both is important in choosing the proper management strategy. We suggest that anticardiolipin antibodies (aCL) should be detected in ANCA-associated vascularitis because they may contribute to life-threatening events superimposed on vascular damage.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
3/16. hypertension due to renal artery occlusion in a patient with antiphospholipid syndrome.We report an unusual case of renovascular hypertension in a 16-year-old boy with primary antiphospholipid syndrome (PAPS), admitted to our clinic for severe drug-resistant hypertension and hypokalemia. Hormonal investigation revealed secondary aldosteronism and positive captopril test for renovascular disease. aortography confirmed the occlusion of the left renal artery. After nephrectomy, normalization of blood pressure and secondary aldosteronism occurred. Presently the patient remains in good health, receiving warfarin anticoagulant therapy. PAPS is defined by the presence of antiphospholipid antibodies and recurrent thrombosis. Arterial thrombosis (29%) appears to be less prevalent than venous thrombosis. Thrombotic microangiopathy of the kidney is frequently observed but renal artery occlusion, as seen in our patient, is unusual.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
4/16. Membranous glomerulonephritis in a patient with inherited activated protein c resistance.We present a patient with membranous glomerulonephritis, several clinical complications of the antiphospholipid syndrome and ulcerative colitis, but without lupus anticoagulant and antiphospholipid/cofactor antibodies. Immunological studies--other antibodies--were negative and failed to show enough criteria for any autoimmune diseases. Evaluation of her laboratory tests for hereditary thrombophilia revealed a heterozygous form of the Leiden mutation that might be associated with widespread vasculopathy. An interesting possibility is that the inherited activated protein c resistance could be an additional risk factor for vaso-occlusive manifestations appearing as a clinical sign of cardiovascular diseases and nephropathy.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
5/16. Myocardial infarction with normal coronary arteries in a patient with primary antiphospholipid syndrome--case report and literature review.Primary antiphospholipid syndrome is associated with an increased risk of vascular thrombosis. The authors describe a young patient without any risk factor for coronary artery disease who was admitted to the hospital because of a transient cerebral ischemic attack. Standard EKG showed signs of a previous silent inferior wall myocardial infarction, confirmed by echocardiography, technetium-99 scintigraphy, and left ventricular angiography. Coronary arteries appeared normal at angiography. Blood tests showed the presence of antiphospholipid antibodies and lupus anticoagulant. Since there is evidence that these antibodies are associated with an increased risk of microvascular thrombosis, the authors conclude that this silent myocardial infarction could be caused by a cardiac microvascular disease accompanying the antiphospholipid syndrome.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
6/16. Familial lupus anticoagulant.The antiphospholipid antibody syndrome (APS) is defined by widespread arterial and venous thromboses associated with elevated plasma levels of antiphospholipid antibodies (APLA). The primary antiphospholipid antibody syndrome (PAPS) appear to be a fairly homogeneous disease, and HLA, family and other studies provide new insights into this cause of thrombosis and vascular disease. We describe two patients with PAPS (lupus anticoagulant positive), whose family members were analyzed for clinical and laboratory abnormalities associated with APS. Familial screening seems to be important, in order to prevent the thrombotic events. Low dose aspirin is the first line treatment in asymptomatic subjects with APLA, previous or present thrombosis requiring long-term, possibly life-long anticoagulation.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
7/16. Renal thrombotic microangiopathy in patients with systemic lupus erythematosus and the antiphospholipid syndrome.Current studies indicate that a thrombotic microangiopathy (TMA) identifies patients with systemic lupus erythematosus (SLE) who are at high risk of progressing to end-stage renal disease. We have observed two patients with SLE and one patient with a primary antiphospholipid syndrome (APS) who developed acute renal insufficiency with thrombocytopenia. Renal biopsies showed a TMA characterized by thrombi or by cellular and mucoid intimal hyperplasia of small arteries and arterioles. No arterial or arteriolar immune-complex deposits were detected by immunofluorescent or electron microscopy. Biopsies from one SLE patient and the APS patient showed no immune-complex glomerular disease. Both had serum antiphospholipid antibodies (aPL). aPL were not detected in the serum of the other SLE patient who had an active lupus nephritis. Acute renal failure and thrombocytopenia resolved in each case following treatment by plasmapheresis or prednisone and heparin. None of the patients were initially treated with cytotoxic drugs. As more knowledge is gained, the accurate identification of renal vascular lesions in SLE or related diseases could influence renal prognosis and choice of therapy. The cases reported here provide further evidence that a TMA can cause acute renal failure independent of lupus nephritis. TMA should be distinguished from other forms of renal vascular disease, particularly a noninflammatory lupus microangiopathy, which is probably mediated by subendothelial immune-complex deposits. The absence of immunoglobulin deposits in vessels involved by a TMA indicates that microvascular thrombosis is promoted by mechanisms other than those usually attributed to immune-complex disease. Phospholipid reactive antibodies may be pathogenetic in some cases.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
8/16. Recurrent acute transverse myelopathy: association with antiphospholipid antibody syndrome.A seven-year-old boy presented with a second episode of acute transverse myelopathy. The first episode had responded dramatically to methylprednisolone. The manifestations of the second episode did not respond to methylprednisolone or IVIG. He showed persistently raised levels of antiphospholipid antibodies in the serum. Primary conditions like collagen vascular diseases, malignancy, exposure to drugs and hiv infection, which are known to be associated with the raised titers of these antibodies were ruled out clinically and by investigations. Recurrent transverse myelopathy is a rare event in childhood and reports of its association with Antiphospholipid Antibody Syndrome (APLAS) are scanty. The etiological role for these antibodies remains to be established. However, once the diagnosis is established, it may be prudent to treat the condition with agents and procedures to bring about a decrease in their titers. Long-term therapy to prevent thromboembolic complications of APLAS may also be instituted.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
9/16. vasculitis in the antiphospholipid syndrome. A cause of ischemia responding to corticosteroids.A 63-year-old woman with atherosclerotic peripheral vascular disease and the lupus anticoagulant developed ischemia of the right lower extremity, requiring progressive amputations. Pathologic specimens revealed inflammatory vasculitis in multiple arteries. Her serum showed anticardiolipin antibodies in high titer. Treatment with high-dose corticosteroids reversed the ischemic process. In patients with antiphospholipid antibodies, thrombosis is the most common pathologic finding associated with cutaneous lesions and/or gangrene. vasculitis, although uncommon, is known to occur and may respond to corticosteroid therapy.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
10/16. The antiphospholipid antibody syndrome in the emergency department setting--livedo reticularis and recurrent venous thrombosis.We present the case of a 26-year-old man with an exacerbation of apparent chronic asthma with chronic peripheral vascular disease due to recurrent venous thrombosis. Localized livedo reticularis, new cutaneous infarctions, severe venous insufficiency, thrombocytopenia, renal failure, and cerebral supratentorial dysfunction were noted. During hospital admission, antibodies to phospholipids in high titer were present by three different testing methods. Renal biopsy demonstrated significant renal vasculature abnormalities characteristic of hemolytic endovasculopathy, and magnetic resonance imaging showed multiple cerebral infarctions. This case exemplifies the spectrum of presentations and management of the primary antiphospholipid antibody syndrome. The clue to its presence in this patient was the livedo reticularis rash, a cutaneous marker for this syndrome that was evident in the emergency department.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
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