Intracardiac thrombosis and fever possibly triggered by ovulation induction in a patient with antiphospholipid antibodies

We report on a 28-year old patient with polycystic ovary syndrome (PCOS) who presented with fever and laboratory markers of inflammation. Her medical history was relevant for multiple ovulation inductions (OI) and ovarian hyperstimulation syndrome (OHSS). She had two miscarriages and one preterm delivery. Intracardiac thrombosis was diagnosed in the presence of antiphospholipid antibodies. We suggest that primary antiphospholipid syndrome (APS) was possibly triggered by OI.     

Outcome of primary antiphospholipid syndrome in childhood

The objective of this paper is to investigate the long-term outcome of primary antiphospholipid syndrome (APS) in the paediatric age. The features of unselected patients with primary APS who had disease onset before the age of 16 years were retrospectively analysed in three Italian referralcentres. Clinical and laboratory manifestations were assessed to establish whether, at the end of follow-up, the final diagnosis was still primary APS or whether they had developed definite SLE or lupus-like syndrome. Fourteen patients, nine boys and five girls, who had the presenting clinical manifestation of APS between three and 13 years of age (median nine years) and were followed for two to 16 years (median six years). Six patients presented with deep vein thrombosis, five with cerebral stroke, two with peripheral artery occlusion and onewith myocardial infarction. During follow-up, four patients had one or more recurrences of vascular thrombosis. At last observation, 10 patients could still be classified as having primary APS, two had developed SLE, one lupus-like syndrome and one Hodgkin's lymphoma. In conclusion; our analysis suggests that some children who present with the features of primary APS may progress to develop SLE or lupus-like syndrome.     

Primary adrenal insufficiency and primary antiphospholipid syndrome

Primary adrenal insufficiency (PAI) is a rare complication of antiphospholipid syndrome (APS). The hypercoagulable state in the APS may lead to adrenal vein thrombosis and subsequently to hemorrhagic necrosis of the adrenal glands. This complication of APS is important to recognize because it may be fatal if untreated. We describe one case of PAI and primary APS, with magnetic resonance studies consistent with hemorrhagic necrosis of the adrenal glands.     

Cardiovascular abnormalities of the antiphospholipid antibody syndrome

The antiphospholipid antibody syndrome (APS) may present with serious cardiovascular complications which should be recognised by the cardiologist. The authors report a series of 6 cases of APS diagnosed after thrombotic events and the finding of antiphospholipid antibodies. The APS was primary in 5 cases and associated with tuberculous lymphadenitis in 1 case. There was cardiac involvement in 5 patients with pericardial effusion in 3 cases, complicated by tamponade as the presenting sign of primary APS in the other 2, valvular disease in one case (moderate mitral stenosis with aortic valve disease) and pulmonary embolism in one case. Five patients developed recurrent deep vein thrombosis of the legs. One patient had a transient ischaemic cerebral attack.     

Acute adrenal failure as the heralding symptom of primary antiphospholipid syndrome: report of a case and review of the literature

Acute adrenal failure is a potentially fatal condition if overlooked. Occasionally, acute adrenal insufficiency may ensue from bilateral adrenal haemorrhage in patients with known antiphospholipid syndrome (APS). APS is characterized by recurrent arterial and venous thrombosis, pregnancy complications and detection of autoantibodies to phospholipids. This syndrome may be associated with non-organ specific diseases (e.g. connective tissue disorders) or with malignancies, but it may also appear in isolated form (primary APS). In a very few cases the heralding manifestation is given by adrenal failure. We report here a 63-year-old man presenting with acute adrenal insufficiency as the opening clinical manifestation of an APS. We also carried out a computer-aided search of the literature to identify all cases of primary adrenal failure as the first-recognized expression of a primary APS, a condition that not so infrequently may be tackled by endocrinologists. 20 patients fulfilled the inclusion criteria. The great majority of them were males (75%) with a mean age of 42 years. Abdominal pain was present in 14 patients, followed by fever (13 patients) and hypotension (12 patients). The main morphological findings by computed tomography or magnetic resonance were consistent with bilateral adrenal haemorrhage in 11 patients. Lupus anticoagulant was present in all of the 19 tested patients. Our observations emphasize the importance in the assessment of clotting times, and possibly of antiphospholipid antibodies, in all patients with diagnosis of rapidly progressive adrenal failure and concurrent abdominal pain.     

A vitamin K antagonist rapidly reverses a blue toe syndrome in a patient with lupus anticoagulant and antiprothrombin antibodies

A 30-year old male was admitted to the hospital with extremely painful blueish discoloration of his toes. After clinical and laboratory evaluation the diagnosis of a blue toe syndrome due to primary antiphospholipid syndrome (APS) was made. Complete resolution of the blue toe syndrome occurred within 72 hours following 9 mg phenprocoumon. APS consists of the association of lupus anticoagulant or antiphospholipid antibodies with arterial or venous thrombosis, thrombocytopenia, and spontaneous abortion. The exact pathways leading to thrombosis are still unknown. Our group has previously proposed that membrane-associated immune complexes contribute towards clinical symptoms in the antiphospholipid syndrome. The case presented strengthens that concept.     

Renal involvement in primary antiphospholipid syndrome and its response to immunosuppressive therapy

Antiphospholipid syndrome (APS) is the association between antiphospholipid antibodies, venous and arterial thrombosis and pregnancy morbidity. Although the kidney may be affected in APS, the treatment of renal involvement is yet to be elucidated. This report describes the clinical and laboratory features of four patients with primary APS nephropathy, and the beneficial effect of immunosuppressive therapy accompanied by warfarin and angiotensin-converting enzyme inhibitor. We also briefly discuss the possible mechanisms of the beneficial effects of immunosuppressives on primary APS nephropathy.     

Influence of hypergammaglobulinemia on antiphospholipid antibodies titres

PURPOSE: Antiphospholipid antibodies (aPL), anticardiolipin antibodies (aCL) or lupus anticoagulant (LA), are indispensable for the diagnosis of antiphospholipid syndrome (APS). However, antiphospholipid assays can generate false positive results. MATERIALS: We have studied the influence of hypergammaglobulinemia (HG) on aPL antibodies titers in 232 patients twice as positive for aPL antibodies. RESULTS: Out of 232 patients, 93 have an APS (76 primary APS, 17 secondary APS). Thrombosis occurred 138 times in APS patients. Of 139 patients without APS, 95 have an auto-immune disease, 28 have an isolated prolonged KCT and 16 an evolutive neoplasia. LA seems to be the best marker of APS. On the other hand aCL IgG and M, anti-beta2-GP1 IgM titers are significantly higher in patients without APS but with HG. CONCLUSION: Those results suggest that biological APS diagnosis should be carefully performed in patients with HG. In this case, other additional risk factors must be considered for the etiological diagnosis of thrombosis.     

Portal and mesenteric vein and inferior vena cava thrombosis associated with antiphospholipid syndrome

We report a 48-year-old man with thrombosis of the portal and superior mesenteric vein and inferior vena cava associated with primary antiphospholipid syndrome (APS). Primary APS was diagnosed by a positive reaction with anticardiolipin antibody (aCL) and the absence of any evidence suggesting the presence of other disease states known to be associated with aCL. A coeliac angiography showed obstruction of the portal and superior mesenteric vein with prominent collaterals and cavernous transformation. Femoral vein angiography showed total obstruction of the external iliac vein and inferior vena cava, and dilation of the pelvic veins, with contrast medium in the lumbar vein. This case is noteworthy as a report of primary APS accompanied by extensive abdominal and pelvic venous thrombosis.     

Diffuse alveolar hemorrhage in the antiphospholipid syndrome: spectrum of disease and treatment

OBJECTIVE: To describe clinical manifestations, laboratory findings, and treatment of patients with the antiphospholipid syndrome (APS) who develop diffuse alveolar hemorrhage. METHODS: Diffuse alveolar hemorrhage is an occasionally reported manifestation of the APS. The diagnosis, however, may be overlooked or manifestations attributed to another disease process. Seven episodes in 5 patients with primary APS were identified and retrospectively reviewed for presenting symptoms and signs, laboratory findings, and response to treatment. RESULTS: The severity of the condition varies, and diffuse alveolar hemorrhage may be the initial manifestation of APS. Patients may present with symptoms ranging from cough, dyspnea, and fever with or without hemoptysis, to symptoms of acute respiratory failure. Hypoxemia and anemia are usually present. Other causes need to be excluded. Bronchoscopy and bronchoalveolar lavage with or without biopsy often aid in confirming the diagnosis. The pathologic abnormality appears to be microvascular thrombosis with or without capillaritis. Treatment with corticosteroids usually leads to marked improvement. CONCLUSION: Patients with APS may present with diffuse alveolar hemorrhage resulting in mild to life threatening symptoms. Prompt and thorough evaluation to confirm the diagnosis and treatment with corticosteroids usually leads to rapid improvement. The clinical setting will dictate whether other therapies such as immunosuppressive agents or intravenous immunoglobulin are required.     

Morphology of vascular, renal, and heart lesions in the antiphospholipid syndrome: Relationship to pathogenesis

A growing body of evidence suggests that aPL are not only serological markers of the antiphospholipid syndrome (APS), but may also directly contribute to the development of thrombosis and other manifestations, including the APS vasculopathy. The latter has been documented in leptmeninges, lung, skin, myocardium, peripheral arteries, and kidney. Renal lesions, a common feature of primary antiphospholipid syndrome (PAPS), include occlusion of principal renal arteries or their main branches, TMA, cortical ischemia, and renal vein thrombosis. Within the cardiac manifestations associated with aPL, valvular involvement is the most common. Histologic findings in valve specimens are consistent with a noninflammatory lesion characterized by intravalvular capillary thrombosis, laminar or verrucous superficial thrombosis, vascular proliferation, fibrosis, and calcification. Even though there is general consensus that endothelial damage triggers the chain of events that results in valve thickening, fusion, rigidity, and ultimately functional abnormalities, we believe that more experimental work remains to be done on the initial valve insult in APS.     

Primary antiphospholipid syndrome associated with mesenteric inflammatory veno-occlusive disease

Summary We describe a 24-year old Caucasian man with gangrene of small bowels and intestinal resection due to mesenteric inflammatory veno-occlusive disease (MIVOD) who later developed deep vein thrombosis in his left leg. He had no clinical evidence of an underlying symptomatic connective tissue disease or Behçet's disease. An IgG anticardiolipin antibody titre above 60 GPL unit/mL and thrombocytopenia confirmed the diagnosis of primary antiphospholipid syndrome (APS). This is the first known case of APS associated with MIVOD.     

A case of primary antiphospholipid antibody syndrome presenting with dysfunctional uterine bleeding and cerebral infarction

We report a 34-year-old woman who developed primary antiphospholipid antibody syndrome (APS) presenting with dysfunctional uterine bleeding and cerebral infarction. Antiphospholipid antibody syndrome presenting with bleeding manifestations is rare. We should recognize that APS may be associated with not only thrombosis but also bleeding.     

Mesenteric and portal venous obstruction associated with primary antiphospholipid antibody syndrome

A 58-year-old Korean man who had a past history of appendicitis, superior mesenteric vein thrombosis and intestinal obstruction presented 7 years later suffering from colicky right upper quadrant pain, epigastric discomfort after fatty meals, nausea and vomiting. He was found to have thrombosis of the superior mesenteric and portal veins, portal hypertension with oesophageal varices, cholangitis, and a biliary stone. The serum anti-cardiolipin antibody (aCL) titres were 103 immunoglobulin (Ig)G antiphospholipid units (GPL) and 50 IgM antiphospholipid units (MPL) and the aCL-IgG titre was still high at 106 2 years after the initial diagnosis. No evidence of disease states known to be associated with antiphospholipid antibodies was found. We report a patient with mesenteric and portal venous obstruction associated with the primary antiphospholipid syndrome (APS).     

A case of primary antiphospholipid antibody syndrome presenting with dysfunctional uterine bleeding and cerebral infarction

Abstract

We report a 34-year-old woman who developed primary antiphospholipid antibody syndrome (APS) presenting with dysfunctional uterine bleeding and cerebral infarction. Antiphospholipid antibody syndrome presenting with bleeding manifestations is rare. We should recognize that APS may be associated with not only thrombosis but also bleeding.     

Possible episodes that trigger thrombotic events in patients with antiphospholipid syndrome

The presence of antiphospholipid antibodies and/or lupus anticoagulant (LA) increase the risk of thrombosis, while the onset of thrombosis is usually sudden. The objective of this study was to determine whether or not some episodes triggered thrombotic events in patients possessing antiphospholipid antibodies. Fifteen patients who presented with thrombosis (primary antiphospholipid syndrome (APS), six cases; secondary APS, nine cases) were retrospectively examined to discover whether or not any specific episodes occurred prior to a total of 21 thrombotic events. In five events occurring in five female patients, specific episodes were identified, including the wearing of tight underwear, dehydration due to fever and standing in hot and humid weather, fever following the extraction of a carious tooth, steroid pulse therapy, toxemia during pregnancy, and intrauterine fetal death. To prevent the occurrence of thrombosis in patients possessing antiphospholipid antibodies, it appears to be important to avoid such triggering episodes and also to reduce the risk factors for thrombosis. Received: May 18, 1999 / Accepted: October 14, 1999     

Antiphospholipid syndrome presenting as unilateral renal artery occlusion: case report and literature review

The objective of this study is to report a case of primary antiphospholipid syndrome (APS) presenting as complete renal artery occlusion, and to review the literature on the subject. We describe the clinical presentation, course and outcome of one patient who presented with resistant hypertension later found to be due to thrombosis and complete occlusion of the left renal artery. We review the medical literature registered in the Medline PubMed database from 1966 to 2004 using keywords: antiphospholipid, Hughes syndrome, kidney, renal, renal artery thrombosis. We describe one patient and analyzed ten well-documented cases of renal artery thrombosis due to APS. Most of the patients were women, at a mean age of 32 years. All but one case had primary APS. The presenting symptom was hypertension in ten cases. Most patients had both lupus anticoagulant and anticardiolipin antibodies. Arterial occlusion was left sided in 55%, right sided in 27% and bilateral in 18%. Renal artery thrombosis is an uncommon presentation of APS. This entity should be considered in the differential diagnosis of severe hypertension.     

Primary antiphospholipid syndrome presented with portal vein thrombosis]

Antiphospholipid Antibody Syndrome (APS) is defined by arterial and venous thrombosis, recurrent spontaneous abortions and thrombocytopenia associated with persistence of antiphospholipid antibodies. Thrombosis may involve virtually all arterial or venous sites, but deep vein thrombosis of the lower limbs are the most common; however, unusual thrombi that involve the portal vein have been described. We report females with documented portal vein thrombosis and primary APS. The treatment of these patients is difficult because of the risk of bleeding and the recurrent thrombosis if they don't receive appropriate long-term anticoagulant therapy.     

Antiphospholipid syndrome: state of the art with emphasis on laboratory evaluation

Antiphospholipid antibodies (aPLs) are associated with arterial and venous thrombosis, recurrent pregnancy loss and thrombocytopenia. Although aPLs have not yet been conclusively shown to be causal in thrombosis and miscarriage, they are useful laboratory markers for the antiphospholipid syndrome (APS). The syndrome can complicate another autoimmune disease, most commonly systemic lupus erythematosus, but more often occurs alone -- primary APS. Identification of the syndrome is clinically important because of the risk of recurrent thrombosis and the need for antithrombotic therapy in many cases. Diagnosis and treatment of APS represent significant challenges, however, owing to the protean clinical manifestations and associations, limitations of currently available laboratory tests for aPLs, and the lack of clear evidence-based guidance on optimal management.     

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.