Diffuse alveolar haemorrhage and Libman-Sacks endocarditis: a rare presentation of antiphospholipid syndrome.

A 26-year-old woman with a history of idiopathic thrombocytopaenic purpura and a 1-year history of blood-streaked sputum presented after a severe episode of haemoptysis with dyspnoea. Chest imaging revealed diffuse ground glass and bronchovascular nodules. Bronchoscopy revealed bilateral diffuse alveolar haemorrhage (DAH). Sputum and bronchoalveolar lavage studies were negative for infectious aetiologies. A transthoracic echocardiogram revealed Libman-Sacks endocarditis with severe mitral regurgitation and physical examination revealed retinal artery occlusion and Osler's nodes. The patient had an increased anticardiolipin Immunoglobulin IgG and anti-B2 glycoprotein IgG, suggesting antiphospholipid syndrome (APLS). The patient was then started on high-dose methylprednisolone and had an improvement in her dyspnoea and haemoptysis. She was also started on anticoagulation as treatment for Libman-Sacks endocarditis. APLS should be considered as a possible underlying aetiology for unusual presentations of DAH with concurrent Libman-Sacks endocarditis in non-intravenous drug users with existing autoimmune disorders.

The role of angiogenic biomarkers and uterine artery Doppler in pregnant women with systemic lupus erythematosus or antiphospholipid syndrome.

OBJECTIVE: To evaluate the usefulness of the uterine artery mean pulsatility index (mPI-UtA) and the sFlt-1/PlGF ratio in women with systemic lupus erythematosus (SLE) or antiphospholipid syndrome (APS) for the prediction of placental dysfunction-related adverse outcomes (AO), namely pre-eclampsia (PE) and intrauterine growth restriction (IUGR), and for differential diagnosis between PE and SLE flares. STUDY DESIGN: Observational prospective cohort study of 57 pregnant women with SLE or APS. MAIN OUTCOME MEASURES: mPI-UtA and sFlt-1/PlGF ratio in maternal serum were obtained at four gestational age periods (11-14, 19-22, 24-29 and 32-34 weeks). Comparisons among pregnancies with normal outcome, SLE flare and AO were performed. RESULTS: Overall, we had 44 ongoing pregnancies (36 with SLE and 8 with APS) of which most (n = 35, 80%) were uncomplicated. The overall rate of AO was 9% (n = 4), that was diagnosed at a mean (SD) gestational age of 34.1 (7.5) weeks. Five SLE patients (14%) suffered a SLE flare. No differences for these markers were found between normal pregnancies and those affected by SLE flare. mUtA-PI values were significantly higher in the AO group when compared with normal and SLE flare groups, at 19-22 weeks (1.52, 0.95 and 0.76) and 32-34 weeks (1.13, 0.68 and 0.65), respectively. The sFlt-1/PlGF ratio was significantly higher in the AO group at 24-29 weeks (191.1, 3.1 and 9.2), respectively. CONCLUSION: Our preliminary results indicate that mPI-UtA and sFlt1/PlGF ratio may be useful to predict AO in women with SLE, and to make the differential diagnosis with a lupus flare.

Carotid and femoral atherosclerosis in antiphospholipid syndrome: Equivalent risk with diabetes mellitus in a case-control study.

BACKGROUND: Antiphospholipid syndrome (APS) may carry a worse prognosis for vascular complications when co-existing with subclinical atherosclerosis; however, the association between the two conditions remains ambiguous. METHODS: We evaluated ultrasonographic markers of subclinical atherosclerosis in carotid and femoral arteries of 86 patients with thrombotic APS [43 primary APS (PAPS), 43 systemic lupus erythematosus-associated APS (SLE/APS)], 86 patients with diabetes mellitus (DM) and 86 healthy controls, individually matched for age and gender, and investigated their associations with traditional and disease-related factors in APS. RESULTS: Carotid plaques were found in 28% of PAPS, 23% of SLE/APS, and 30% of DM patients versus 9% of controls (p = 0.006). Femoral plaques were found in 33% of PAPS, 19% of SLE/APS, 20% of DM, and 9% of controls (p = 0.032). Multivariate regression-derived relative risk estimates for atherosclerotic plaques in any location were 2.72 for PAPS, 2.63 for SLE/APS, and 1.98 for DM (p = 0.004, 0.009, and 0.032 respectively), after adjusting for age, gender, hypertension, dyslipidemia, smoking, BMI, and family history of coronary disease. Among patients with APS, atherosclerotic plaques were associated with the number of traditional CVD risk factors in both PAPS (RR = 2.75, p < 0.001) and SLE/APS (RR = 1.84, p < 0.001), and with IgG anti-beta2-glycoprotein I antibodies in SLE/APS. CONCLUSIONS: Patients with PAPS and SLE/APS have a nearly 2.5-fold risk of atherosclerotic plaques in carotid and femoral arteries compared to healthy controls, similar to DM patients. Atherosclerotic plaques are associated with the number of traditional risk factors in both APS and SLE/APS, and with IgG anti-beta2-glycoprotein I antibodies in SLE/APS.

Catastrophic antiphospholipid syndrome (CAPS)-induced ischemic pancreatic ducts injury mimicking intraductal papillary mucinous neoplasm (IPMN).

OBJECTIVES: Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening disease characterized by multiple small-vessel occlusions of rapid onset. Ischemic pancreatic duct lesions secondary to CAPS have never been reported. METHODS: We describe 4 patients who presented lesions suspected to be intraductal papillary mucinous neoplasm (IPMN) of the pancreas following a CAPS. RESULTS: All patients had a history of CAPS months or years before the IPMN diagnosis. They had abdominal pain or abnormal liver test results and had undergone radiography. In a 36-year-old man, endoscopic ultrasonography and magnetic resonance cholangiopancreatography demonstrated parietal thickening, stenoses and dilatations of the main pancreatic duct, which suggested IPMN. A pancreatic resection was performed because of presumed risk of malignancy. Histology revealed pancreatitis and thrombosis of small pancreatic vessels but no IPMN. The 3 other cases had lesions consistent with IPMN disclosed on MRI. From the first case experience, regular radiography surveillance was decided for the 3 other patients. After more than 4 years of follow-up, lesions remained unchanged. CONCLUSION: Physicians must be aware that these lesions may be encountered in CAPS and may closely mimic IPMN, with subsequent risk of performing unnecessary pancreatectomy.

Increased heterogeneity of brain perfusion is an early marker of central nervous system involvement in antiphospholipid antibody carriers.

OBJECTIVE: The non-criteria neuropsychiatric manifestations of antiphospholipid syndrome include headache, dizziness, vertigo, seizure, depression and psychosis. There were still no objective methods qualified to detect the early central nervous system involvement in non-criteria antiphospholipid syndrome. We evaluated the effectiveness of Tc-99m ECD SPECT in assessing circulatory insufficiency in the brains of patients with antiphospholipid antibodies and neuropsychiatric symptoms but without thromboembolism. MATERIALS AND METHODS: Patients with a history of positive antiphospholipid antibodies and neuropsychiatric symptoms composed the case group; patients without antiphospholipid antibody served as the control group. Subjects with a history of thromboembolism or autoantibodies to extractable nuclear antigens were excluded. All patients received Tc-99m ECD SPECT studies and were classified by the number of positive antiphospholipid antibodies they carried. The heterogeneity of brain perfusion was defined as the coefficient of variation of the SPECT signals. Analysis of variance (ANOVA) was applied to evaluate the differences between the groups. RESULTS: Total 60 adult patients were included in this study. There were 54 patients in the case group and 6 patients in the control group. The mean age was 38.3 ± 11.5 years. There were 52 women and 8 men. There was no significant difference in the mean brain perfusion between groups (P = 0.69). However, Tc-99m ECD SPECT demonstrated significant heterogeneity of brain perfusion in relation to the number of antiphospholipid antibodies (P = 0.01). CONCLUSIONS: This is the first study demonstrating that Tc-99m ECD SPECT can early detect the increased heterogeneity of brain circulation in non-criteria antiphospholipid antibody carriers.

Síndrome antifosfolípido catastrófico: caso clínico / Catastrophic antiphospholipid syndrome: clinical case

El síndrome antifosfolípido (SAF) fue descrito y caracterizado durante la segunda mi-tad del siglo XX inicialmente como un fenómeno protrombótico secundario en con-texto de otras enfermedades del tejido conectivo, principalmente lupus. Sin embargo, el estudio de pacientes con enfermedad primaria impulsó a distintos consensos, tan-to clínicos como de laboratorio para su correcta identificación. Entre los pacientes con SAF destaca la forma de presentación catastrófica, de baja prevalencia, pero impor-tante por su mal pronóstico, caracterizada por el compromiso de múltiples sistemas en corto tiempo. Presentamos el caso de una paciente del Hospital Clínico San Borja-Arriarán con diag-nóstico de SAF primario, que presentó en su evolución la forma catastrófica. Este caso sirve de base para una revisión del proceso diagnóstico del SAF en relación a otras patologías reumatológicas y las características propias del SAF catastrófico.

Antiphospholipid syndrome (APS) was described and characterized during the second half of the 20th century initially as a secondary prothrombotic phenome-non in the context of other connective tissue diseases, mainly lupus. However, the study of patients with primary disease prompted different consensus, both clin-ical and laboratory for their correct identification. Among patients with APS, the catastrophic presentation is of low prevalence, but important because of its poor prognosis, characterized by the commitment of multiple systems in a short time. We present the case of a patient from the San Borja-Arriaran Clinic Hospital with di-agnosis of primary APS, which presented the catastrophic form in its evolution. This case serves as a basis for a review of the diagnostic process of APS in relation to other rheumatologic pathologies and the characteristics of catastrophic APS.

The Place of Nailfold Capillaroscopy Among Instrumental Methods for Assessment of Some Peripheral Ischaemic Syndromes in Rheumatology.

Micro- and macrovascular pathology is a frequent finding in a number of common rheumatic diseases. Secondary Raynaud's phenomenon (RP) is among the most common symptoms in systemic sclerosis and several other systemic autoimmune diseases including a broad differential diagnosis. It should be also differential from other peripheral vascular syndromes such as embolism, thrombosis, etc., some of which lead to clinical manifestation of the blue toe syndrome. The current review discusses the instrumental methods for vascular assessments. Nailfold capillaroscopy is the only method among the imaging techniques that can be used for morphological assessment of the nutritive capillaries in the nailfold area. Laser-Doppler flowmetry and laser-Doppler imaging are methods for functional assessment of microcirculation, while thermography and plethysmography reflect both blood flow in peripheral arteries and microcirculation. Doppler ultrasound and angiography visualize peripheral arteries. The choice of the appropriate instrumental method is guided by the clinical presentation. The main role of capillaroscopy is to provide differential diagnosis between primary and secondary RP. In rheumatology, capillaroscopic changes in systemic sclerosis have been recently defined as diagnostic. The appearance of abnormal capillaroscopic pattern inherits high positive predictive value for the development of a connective tissue disease that is higher than the predictive value of antinuclear antibodies. In cases of abrupt onset of peripheral ischaemia, clinical signs of critical ischaemia, unilateral or lower limb involvement, Doppler ultrasound and angiography are indicated. The most common causes for such clinical picture that may be referred to rheumatologic consultation are the antiphospholipid syndrome, mimickers of vasculitides such as atherosclerosis with cholesterol emboli, and neoplasms.

Catastrophic Antiphospholipid Syndrome: Scintigraphic Demonstration With Correlated Cross-Sectional Imaging.

We present the case of catastrophic antiphospholipid syndrome occurring in a 44-year-old woman with a recent history of coronary artery bypass surgery. Postoperatively, she was urgently readmitted for a left middle cerebral artery stroke, and during workup she was found with a left ventricular thrombus on echocardiogram. Subsequently, the patient was diagnosed with antiphospholipid syndrome. Multimodality imaging, including bone and myocardial perfusion scintigraphy, CT, and MR, during her hospitalization, depicted all the characteristic features constituting the catastrophic form of antiphospholipid syndrome, also known as Asherson syndrome.

Neurosurgical management for complicated catastrophic antiphospholipid syndrome.

Antiphospholipid syndrome (APS) is an autoimmune condition involving arterial and venous thrombosis. An unusual APS variant, catastrophic antiphospholipid syndrome (CAPS), includes rapid multi-organ failure from widespread small vessel thrombosis. Central nervous system complications arise in one-third of CAPS patients. In rare cases, CAPS co-manifests with cerebellar hemorrhage presenting a neurosurgical emergency. We present a 65-year-old woman with CAPS-related cerebellar hematoma, co-morbid idiopathic thrombocytopenic purpura, deep vein thrombosis and altered mental status, with treatment complicated by thrombocytopenia. The patient suddenly deteriorated, secondary to a cerebellar subdural hematoma, and underwent decompression and excision of the hematoma. After recovery in the intensive care unit, she developed a new spontaneous epidural hematoma requiring additional surgery. Management of these patients is hematologically complex and often requires a multi-disciplinary team of physicians. This patient provides an important learning point for clinicians - consider CAPS when hemorrhage and thrombosis are present.

Mitral valve surgery for marantic endocarditis and multiple cerebral embolisation.

Valvular involvement is common in antiphospholipid syndrome (APS) with increased risk of thrombo-embolic events. We report a patient with APS and multiple cerebral infarcts. Echocardiography demonstrated verrucous vegetations of the mitral valve in keeping with marantic endocarditis. The patient underwent successful mitral valve replacement. Post-operative clinical and echocardiographic follow-up showed excellent short term results.

Thin-section chest CT findings in systemic lupus erythematosus with antiphospholipid syndrome: a comparison with systemic lupus erythematosus without antiphospholipid syndrome.

PURPOSE: To assess thin-section chest CT findings in systemic lupus erythematosus (SLE) with antiphospholipid syndrome (APS), in comparison with SLE without APS. MATERIALS AND METHODS: We retrospectively reviewed the medical records and thin-section CT findings of 17 consecutive patients with an established diagnosis of SLE with APS, comparing with 37 consecutive SLE patients without APS, between 2004 and 2008, and patients who had other autoimmune disease, such as Sjögren syndrome, were excluded. No significant differences were seen between the two groups in age, gender, smoking habits, or history of steroid pulse and biological therapy. CT images of 2mm thickness obtained with a 16- or 64-detector row CT were retrospectively evaluated by two radiologists in consensus on ultra high-resolution gray-scale monitors. RESULTS: The frequency of thin-section CT abnormalities was higher in SLE with APS group (82%) than in SLE without APS group (43%). Ground-glass opacity (59%), architectural distortion (47%), reticulation (41%), enlarged peripheral pulmonary artery (29%), and mosaic attenuation (29%) were significantly more common in the SLE with APS group than in the SLE without APS group (Fisher's exact test, p<0.01). CONCLUSION: SLE patients with APS have increased prevalence of thin-section chest CT abnormalities than those without APS.

Coronary pseudoaneurysm and superior vena cava occlusion in a young patient with multicentric Castleman's disease and antiphospholipid antibody positivity.

Castleman disease is a non-malignant proliferative disease of the lymphoid system. It can be unicentric or multicentric. In this report, we present a case of multicentric Castleman disease to which coronary artery pseudoaneurysm, vena cava superior occlusion, and antiphospholipid antibody positivity was accompanying.

Acute fibrinous and organizing pneumonia in systemic lupus erythematosus: a case report and review of the literature.

Pulmonary manifestations of systemic lupus erythematosus (SLE) typically include pleuritis, alveolar hemorrhage, and infectious pneumonia due to immunosuppression with less common entities including bronchiolitis, interstitial pneumonia, and pulmonary fibrosis. More rare manifestations include organizing pneumonia (OP) and diffuse alveolar damage (DAD). A similar but distinct entity of acute fibrinous and organizing pneumonia (AFOP), characterized by intra-alveolar fibrin deposition and associated organizing pneumonia, has been reported in association with connective tissue disorders, but has not been described in association with SLE. Reported herein is a patient with SLE and accompanying antiphospholipid syndrome with recent pulmonary embolism, persistent respiratory symptomology, and persistent radiographic abnormalities who underwent lung biopsy displaying features of AFOP. This case in conjunction with previous literature indicates that AFOP can be a manifestation of connective tissue disease including SLE and may be an underreported variant of medical lung disease due to overlap in histological characteristics with OP and DAD.

Right atrium myxoma coexisting with antiphospholipid syndrome: a case report.

In this case report we describe a rare case of right atrium myxoma that coexisted with antiphospholipid syndrome in a young woman. We describe the unusual findings and diagnostic challenges combined with a review of the literature.