An unusual case of pacemaker endocarditis in a patient with antiphospholipid syndrome

We report the case of a 50-year-old woman with systemic lupus erythematosus who had previously undergone pacemaker implantation. She developed recurrent pulmonary thromboembolism and was diagnosed with antiphospholipid syndrome. During investigation, pacemaker endocarditis was discovered, and the system was surgically explanted. Surprisingly, all microbiological studies, including culture of the extracted material and extensive serological analysis, were negative and she remained well with anticoagulation plus her usual immunosuppressant regimen. The data indicate that her pacemaker endocarditis could be an equivalent of the nonbacterial thrombotic endocarditis often described in native valves.     

Intracerebral hemorrhage in a patient with SLE and catastrophic antiphospholipid syndrome (CAPS): report of a case

A 31-year-old woman was admitted to the hospital for investigation of left lower limb thrombophlebitis. History, physical examination, and laboratory investigations led to the diagnosis of systemic lupus erythematosus (SLE), complicated by secondary antiphospholipid syndrome (APS). Treatment included steroids, azathioprine, aspirin, and low molecular weight heparin. Sixty-three days later, she was admitted to the hospital again because of high fever, macroscopic hematuria, and dyspnea. Laboratory testing showed anemia and impaired renal function. High-resolution chest computed tomography (CT) revealed bilateral multiple peribronchial infiltrates with hemorrhage. Magnetic resonance imaging (MRI) angiography of the kidneys revealed left renal vein thrombosis combined with ischemia of the left kidney. Cyclophosphamide and methylprednisolone pulse treatment as well as intravenous immunoglobulins were started immediately. Despite intensive immunosuppressive and supportive treatment, she suffered three relapses of alveolar hemorrhage and died on day 40, due to severe intracerebral bleeding. The final diagnosis was catastrophic APS with diffuse alveolar hemorrhage and kidney involvement. The unusual combination of recurrent alveolar hemorrhage and death from intracerebral hemorrhage rather than thrombosis in a CAPS patient is discussed.     

Autoantibodies involved in neuropsychiatric SLE and antiphospholipid syndrome

OBJECTIVE: We sought (1) to identify and (2) to define the association of all reported antibodies (Abs) with neuropsychiatric lupus (NPSLE), (3) to search for possible mechanisms that are involved in NPSLE, and (4) to determine whether we can recognize a panel of Abs associated with specific neuropsychiatric (NP) manifestations. METHODS: A MEDLINE search (1975 to 2005) was performed utilizing the following terms: neuropsychiatric lupus, antiphospholipid syndrome, or central nervous system systemic lupus erythematosus matched with the term antibodies. RESULTS: Twenty Abs (11 brain-specific and 9 systemic) were described in NPSLE patients. These include Abs that target brain-specific antigens (neuronal, ganglioside, synaptosomes, glia, methyl-d-aspartate receptors, lymphocytotoxic) and systemic antigens (nuclear, cytoplasmic, phospholipid, endothelial cells). Cognitive impairment, psychosis, and depression were associated with many Abs. Elevated titers of anticardiolipin Abs (aCL) were reported most often and found in patients with cognitive impairment, psychosis, depression, seizures, chorea, and migraine. No specificity was encountered among brain-specific or systemic Abs for any single NP manifestation. No studies evaluated a specific NP manifestation with the full panel of 20 Abs. A panel of brain-specific and systemic Abs may be helpful in establishing the diagnosis of NPSLE. Postulated mechanisms in experimental models included vascular occlusion and injury by pathogenic Abs in a disrupted blood brain barrier. CONCLUSIONS: NPSLE is associated with brain-specific and systemic Abs. Cognitive impairment, psychosis, and depression were associated with many Abs, including aCL Abs. Possible mechanisms include vascular occlusion and injury by pathogenic Abs in a disrupted blood brain barrier.     

Libman-sacks endocarditis and primary antiphospholipid syndrome

Cardiac involvement is a not uncommon complication in patients with antiphospholipid syndrome (APS). Herein, the case is reported of cardiac failure in a female patient with Libman-Sacks endocarditis and with primary APS diagnosed eight years previously. Aggressive anticoagulation therapy and medical treatment for the cardiac failure over a 12-month period resulted in a partial regression of the severe mitral regurgitation. Close clinical and echocardiographic surveillance during the follow up of patients with APS and heart valve disease is mandatory. Optimal treatment, including adequate aggressive anticoagulation therapy and specific treatment for heart failure, may play a pivotal role in reducing the severity of valve dysfunction in these patients.     

Heparin-induced thrombocytopenia associated with interleukin-8-dependent platelet activation in a patient with antiphospholipid syndrome

Platelet factor 4 heparin enzyme immunoassay, platelet aggregation test, and serotonin release assay are commonly used to diagnose and confirm heparin-induced thrombocytopenia. We describe a case of recurrent thrombocytopenia appearing in a few hours after each heparin administration and who tested negative for the three assays. Further analysis revealed anti-interleukin (IL)-8 antibodies and IL-8-dependent platelet activation facilitated by heparin, which may explain this unusual case of heparin-induced thrombocytopenia.     

Severe thrombophilia with antiphospholipid syndrome and hyperhomocysteinemia in a patient with Schnitzler's syndrome

Schnitzler's syndrome is a rare condition with chronic urticaria, intermittent fever, bone pain, and a monoclonal IgM gammopathy. Most patients have a chronic and indolent course, but a small number ultimately progress to a lymphoplasmacytic malignancy. We describe a patient with Schnitzler's syndrome who entered an accelerated phase of clinical deterioration with repeated thromboses of the cerebral and coronary arteries that ultimately led to a fatal outcome. We found that the he fulfilled the Sapporo criteria for definite antiphospholipid syndrome and had elevated blood levels of homocysteine during the active clotting phase of the disease. We suggest that the association with immune-mediated or metabolic disorders, such as the antiphospholipid syndrome and hyperhomocysteinemia, may unfavorably affect the otherwise chronic and stable course of patients with Schnitzler's syndrome. The systemic clotting disorder, the association with the antiphospholipid syndrome and hyperhomocysteinemia, and the biclonal rather than monoclonal IgM gammopathy were striking features of this atypical case of Schnitzler's syndrome.     

Libman-Sacks endocarditis and cerebral embolization in antiphospholipid syndrome

In antiphospholipid syndrome (APS), there is a high prevalenceof valvular heart disease which leads to increased risk of thrombo-embolicevents, in particular, cerebrovascular events. We present apatient with cerebral infarction, previous deep-vein thrombosis,and miscarriages with positive lupus anticoagulant and anticardiolipinantibodies. Echocardiographic examination revealed mitral valveleaflet thickening and verrucous vegetations consistent withLibman–Sacks endocarditis, which is commonly associatedwith APS. In patients with combined Libman–Sacks endocarditisand antiphospholipid antibodies, anticoagulation therapy withwarfarin is indicated due to high risk of valvular thrombusformation and subsequent embolization.     

Liver transplantation in a patient with primary antiphospholipid syndrome and Budd-Chiari syndrome

The antiphospholipid syndrome (APS) is an acquired thrombophilic disorder in which autoantibodies are produced to a variety of phospholipids determinants of cell membranes or phospholipid binding proteins. There are few reports about association between antiphospholipid antibodies and development of Budd-Chiari syndrome (BCS). We report the case of BCS development in young Russian male with primary APS. The patient underwent orthotopic liver transplantation on August 26, 2012. At present time his state is good, the blood flow in the liver restored and its function is not impaired. We report about the first time the successful use of dabigatran etexilate for prolonged anticoagulation therapy in APS patient with BCS. In addition patient is managed with immunosuppressive drugs.     

Bacterial endocarditis in a patient with Marfan's syndrome.

A patient with Marfan's syndrome and subacute bacterial endocarditis is presented. Echocardiographic studies demonstrated dilatation of the aortic root, prolapse of the posterior leaflet of the mitral valve, and the appearance of shaggy echoes on the anterior miltral leaflet, of the kind previously described as representing bacterial vegetations.     

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.     

Severe neurological and obstetrical complications in a patient with antiphospholipid syndrome

Antiphospholipd syndrome (APS) is a disease characterised by venous and arterial thrombosis or recurrent foetal loss, which are associated with antiphospholipid antibodies or/and lupus anticoagulant. Clinical symptoms ofAPS are assocciated with presence of noninflammatory thrombosis ocluding arteries or venes. Symptoms of APS are often very dramatic what can be illustrated by the presented case of 40 year patient. At the age of 25 after a miscarriage the patient developed tetraparesis and motor aphasia in the course of thrombosis in central nervous system vessels. In physical examination besides neurological symptoms reticular livedo was found on the trunk and limbs. Serological tests have revealed presence of high titre of IgG and IgM anticardiolipin antibodies and lupus anticoagulant (LA). CT examination revealed hypodensic foci in left parietal lobe, and abnormal EEG findings were observed in fronto-temporal leads of left hemisphera of brain. At that point the patient did not meet the criteria of connective tissue diseases, including lupus. The diagnosis of primary APS was suggested. The patient received anti-aggregation treatment and also immunosuppressive drugs (azathioprine and prednison) due to progression of neurological manifestations. 3 years later, the second pregnancy ended in the 27th week with intrauterine fetal death. During the 3rd pregnancy, 2 years afterwards, the patient was treated with heparin, aspirin and intravenous immunoglobulin. The pregnancy finished with a successful delivery at term, the newborn was in good condition. During the following pregnancy the symptoms of preeclampsia occurred at 36/37th week but the newborn was delivered in a good condition after a caesarean section. At the age of 36 patient developed ischemic brain stroke with left-side hemiparesis inspite of anti-aggregation and immunosupressive (prednison and azathioprine) treatment. At that time homogenic type antinuclear antibodies (ANA) 1:2560, anti-beta2-glycoprotein antibodies (beta2-GPI), aCL antibodies in medium titer and thrombocytopenia were found. The patient was treated with heparin, cyclophosphamide and methyloprednisolon intravenously. During rehabilitation process gradual improvement of cognitive functions, speech and motorical functions was observed. Recently high titre of ANA and recurent thrombocytopenia < 100,000/mm3 were present. Maybe during the further follow-up, 14 years after the first symptoms of APS, the patient will develop full blown symptoms of SLE.