1/308. Lupus anticoagulant syndrome: case report.A twenty seven year old female was referred to our department with deep vein thrombosis, abnormal activated partial thromboplastin time (aPTT) ratio 1:60 and prothrombin time (PT) INR of 3:11. She had history of loss of pregnancies previously. Coagulation tests with pooled normal fresh plasma did not correct a PTT because of a coagulation inhibitor and only partially corrected PT. kaolin clotting time (KCT) of patient plasma (PP) and a mixture of PP/normal plasma (NP) detected the lupus anticoagulant (LA). Venereal disease Laboratory (VDRL) test on the patient's serum was positive with low titre 1:8 while treponema pallidum haemaglutination test (TPHA) was negative. Anticardiolipin antibodies IgG were raised while IgM levels were within normal levels. This was a case of lupus anticoagulant syndrome. The patient was treated with unfractionated heparin and warfarin and later started on salicylates and prednisone.- - - - - - - - - - ranking = 1keywords = lupus (Clic here for more details about this article) |
2/308. Valvular deposition of antiphospholipid antibodies in the antiphospholipid syndrome: a clue to the origin of the disease.In this report we present an unusual case of a 45-year-old female patient with systemic lupus erythematosus (SLE) who was hospitalized for mitral valve replacement. In her childhood she presented with mitral stenosis and chorea on which grounds a preliminary diagnosis of rheumatic fever was established. After a quiescent period lasting two decades her disease erupted with mitral stenosis, thromboembolic phenomena, and nephritis. Due to severe malfunctioning of her mitral valve, the patient eventually underwent mitral valve replacement. The antibodies involved in the pathogenesis of our patient's valvular disease were studied by immunohistochemical analysis, applying rabbit polyclonal anti-human IgG and IgM anti-human C3c and anti-idiotypes to a mouse monoclonal naturally occurring polyspecific human monoclonal anti-cardiolipin antibody termed S2.9, and to the 16/6 Id which defines a common Id on anti-dna antibodies in patients with SLE. Immunoperoxidase staining using an anti-idiotype mAb to anti-cardiolipin antibodies demonstrated the deposition of these anti-bodies in the subendothelial layer of the valve. We believe that anti-phospholipid syndrome (APS) with SLE was the initial and primary disease in this patient. These findings clearly indicate that APS must be considered in the differential diagnosis of rheumatic fever, particularly in young female patients who present with mitral stenosis and chorea.- - - - - - - - - - ranking = 6.5591108595059keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
3/308. Acquired C1 inhibitor deficiency associated with systemic lupus erythematosus, secondary antiphospholipid syndrome and IgM monoclonal paraproteinaemia.In this case report, a woman is described who developed systemic lupus erythematosus at the age of 36 years affecting the central nervous system and kidneys, showing the serological evidence of an acquired C1 inhibitor deficiency, but also developing an antiphospholipid syndrome and IgM lambda type paraproteinemia. During the 25 years follow-up she did not show any signs of angioedema. Although the immunological abnormalities are still constantly present, the disease has been quiescent since the past three years. The possible pathophysiology of clinical and laboratory features are discussed.- - - - - - - - - - ranking = 32.79555429753keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
4/308. plasma exchange for thrombocytopenia in antiphospholipid syndrome: a case report.The remarkable effect of plasma exchange (PE) was observed on thrombocytopenia in a patient with antiphospholipid syndrome (APS) associated with systemic lupus erythematosus. Three times PE led to recovery from severe thrombocytopenia refractory to treatment with corticosteroid, anticoagulant, and antiplatelet drugs accompanied by a decrease in the serum cardiolipin beta2 glycoprotein I antibody level. This result suggests that PE is a valuable therapeutic tool for refractory thrombocytopenia in APS.- - - - - - - - - - ranking = 6.5591108595059keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
5/308. Cryosupernatant plasma exchange in the treatment of antiphospholipid antibody syndrome with lupus nephritis.We report a case of a 22-year-old female with antiphospholipid antibody syndrome (APS) associated with systemic lupus erythematosus in whom cryosupernatant plasma exchange was effective and improved both the refractory venous thrombosis in her legs and relapsing thrombocytopenia. A renal biopsy specimen showed not only features of active lupus nephritis but also renal arteriolar thrombosis which is considered to be a type of thrombotic microangiopathy (TMA). Because a pathological role of unusually large von willebrand factor (vWF) multimers has been reported in patients with TMA including thrombotic thrombocytopenic purpura, plasma exchange using replacement with cryosupernatant, which is free of unusually large vWF multimers, is likely to be an option of treatment modality for patients with refractory and chronic relapsing APS manifesting TMA.- - - - - - - - - - ranking = 9.0591108595059keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
6/308. Cutaneous papules and nodules in the diagnosis of the antiphospholipid syndrome.Of 11 patients with primary or secondary antiphospholipid syndrome (APS), four exhibited papules or nodules on the finger, sole or leg as the initial cutaneous manifestation. Histological examination demonstrated thrombosed vessels or vessels containing organized thrombi in the dermis or in the subcutaneous fat tissue. Cutaneous papules and nodules should be recognized as skin manifestations of APS. Screening tests for antiphospholipid antibodies and lupus anticoagulant are required in patients with cutaneous papules or nodules of unknown aetiology. In cases of positive antiphospholipid antibodies and/or lupus anticoagulant, histological examination is critical in the establishment of the diagnosis of APS.- - - - - - - - - - ranking = 1keywords = lupus (Clic here for more details about this article) |
7/308. Characterization of IgG monoclonal anti-cardiolipin/anti-beta2GP1 antibodies from two patients with antiphospholipid syndrome reveals three species of antibodies.Antiphospholipid antibodies (aPL), including antibodies detected in anti-cardiolipin (aCL) enzyme-linked immunosorbent assays and in lupus anticoagulant (LA) tests, are strongly associated with recurrent thrombosis and recurrent fetal loss, i.e. the antiphospholipid syndrome (APS). Although recent studies suggest that most APS-associated aCL are directed against the phospholipid (PL)-binding plasma protein beta2-glycoprotein 1 (beta2GP1), the precise nature of aCL binding specificities remains controversial. To address the issue of aCL specificity we generated five new monoclonal IgG aCL from two patients with APS. Characterization of these five aCL, as well as two previously published IgG aCL, revealed three patterns of reactivity: (1) four antibodies reacted strongly with human beta2GP1-cardiolipin (CL) complexes and weakly with human beta2GP1 alone; (2) two antibodies recognized bovine beta2GP1, but not human beta2GP1; (3) one antibody reacted with complexes of human beta2GP1 and CL, but not with human beta2GP1 alone. Only one monoclonal displayed weak LA activity. These patient-derived IgG monoclonal antibodies, and additional ones to be generated, may help define varying species of antibodies detected in aCL assays and identify the specific antibodies that may be pathogenic.- - - - - - - - - - ranking = 0.5keywords = lupus (Clic here for more details about this article) |
8/308. Systemic lupus erythematosus-associated catastrophic antiphospholipid syndrome occurring after typhoid fever: a possible role of Salmonella lipopolysaccharide in the occurrence of diffuse vasculopathy-coagulopathy.We report a case of well-documented typhoid fever in a 30-year-old woman with inactive systemic lupus erythematosus with asymptomatic lupus anticoagulant and high-titer anticardiolipin antibody (aCL). Despite prompt eradication of the salmonella typhi obtained with appropriate antibiotic therapy, multiple organ system dysfunction occurred. The central nervous system was involved, with ischemic infarcts in the occipital lobes. High-dose corticosteroid therapy failed to improve the neurologic manifestations, which responded to repeated plasmapheresis. A sharp fall in aCL and anti-beta2-glycoprotein I antibody titers was recorded before the start of plasmapheresis. At the same time, IgM and IgG antibodies to Salmonella group O:9 lipopolysaccharide became detectable; the IgM antibodies disappeared within 4 months, whereas the IgG antibodies remained detectable during the next 13 months. Despite treatment with high-dose corticosteroids and cyclophosphamide, rapidly progressive glomerulonephritis developed, leading to chronic renal failure. There is convincing evidence of a link between the S. typhi infection and the ensuing catastrophic syndrome in this patient, probably precipitated by bacterial antigens.- - - - - - - - - - ranking = 28.278353112089keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
9/308. Optic neuropathy in systemic lupus erythematosus and antiphospholipid syndrome (APS): clinical features, pathogenesis, review of the literature and proposed ophthalmological criteria for APS diagnosis.Optic neuropathy is a well-known ocular manifestation occurring in patients with systemic lupus erythematosus (SLE), and it remains one of the major causes of blindness in these patients. We report data from six SLE patients with optic neuropathy, one of whom was considered to have antiphospholipid syndrome (APS). This patient had monolateral optic neuropathy, whereas the other five SLE patients had bilateral optic nerve disease. We believe that the monolateral occurrence of optic neuropathy in our patient can be considered as a 'focal' neurological disease due to a thrombotic event involving the ciliary vasculature. Conversely, bilateral optic nerve damage in SLE could be considered to be a 'general' neurological disease due to different immunological mechanisms, such as vasculitis. Additionally, the literature on SLE patients affected by optic neuropathy is reviewed to evaluate the major clinical features, particularly neurological features. In reviewing the literature, it appears that bilateral optic neuropathy in SLE occurs more frequently than monolateral optic neuropathy, and the main neurological manifestation seen in these patients is transverse myelitis, particularly in SLE patients with bilateral optic nerve disease. Finally, we propose a clinico-ophthalmological spectrum of APS and outline the ocular clinical manifestations that can be considered as diagnostic for the syndrome.- - - - - - - - - - ranking = 32.79555429753keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
10/308. Recurrent thrombo-embolic episodes: the association of cholangiocarcinoma with antiphospholipid syndrome.antiphospholipid syndrome is a disorder of recurrent vascular thrombosis, pregnancy loss and thrombocytopenia associated with persistently elevated levels of antiphospholipid antibodies. It was first described in a group of patients with systemic lupus erythematosus but has since been associated with a wide range of conditions, including other autoimmune disorders and malignancy. It can also occur in isolation, the so-called primary antiphospholipid syndrome. We describe an elderly woman with the antiphospholipid syndrome thought to be associated with a cholangiocarcinoma.- - - - - - - - - - ranking = 6.5591108595059keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
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