Hemorragia alveolar como manifestación de síndrome antifosfolípido primario / Alveolar Hemorrhage as a Manifestation of Primary Antiphospholipid Syndrome

RESUMEN El síndrome antifosfolípido (SAF) es una entidad caracterizada por fenómenos trom bóticos (arteriales y/o venosos), pérdidas fetales y elevación sérica persistente de anticuerpos antifosfolípidos. Las complicaciones pulmonares más frecuentes del SAF son: tromboembolismo pulmonar, hipertensión pulmonar tromboembólica. La hemorragia alveolar es una ma nifestación infrecuente y potencialmente mortal (SAF catastrófico). El diagnóstico se confirma cuando en una muestra tomada mediante lavado bronquioalveolar (BAL), más del 20 % de los macrófagos son positivos para hemosiderina. Los hallazgos radiográfi cos más comúnmente muestran opacidades en vidrio deslustrado o de consolidación que suelen ser difusas, bilaterales más centrales que periféricas. La DLCO es otro método diagnóstico, con valores por encima del 120 % del valor predicho. Presentamos el caso de un paciente con SAF con antecedentes de trombosis venosa profunda (TVP) y tromboembolismo pulmonar (TEP) anticoagulado, que ingresa con diagnóstico de hemorragia alveolar (HA).

ABSTRACT Antiphospholipid syndrome (APS) is an entity characterized by thrombotic phenomena (arterial and/or venous), fetal losses, and persistent increase in the serum level of antiphospholipid antibodies. The most common pulmonary complications of APS are pulmonary thromboembolism and thromboembolic pulmonary hypertension. Alveolar hemorrhage is a rare, potentially life-threatening manifestation (catastrophic APS). The diagnosis is confirmed when more than 20 % of the macrophages in a sample taken by bronchoalveolar lavage (BAL) are positive for hemosiderin. Radiographic findings most commonly show ground-glass opacities or consolidations that are usually diffuse, bilateral, more central than peripheral. The DLCO is another diagnostic method, with values above 120 % of the predicted value. We present the case of a patient with APS with a history of deep vein thrombosis (DVT) and anticoagulated pulmonary thromboembolism (PTE), who was admitted with a diag nosis of alveolar hemorrhage (AH).

Síndrome antifosfolípido (SAF): diferencias clínicas e inmunoserológicas entre SAF primario y secundario en una cohorte colombiana / Antiphospholipid syndrome (APS): Clinical and immunoserological differences between primary and secondary APS in a Colombian cohort

RESUMEN Introducción: El síndrome antifosfolípido (SAF) es una enfermedad autoinmune sistémica, caracterizada por trombosis recurrente, que puede afectar la circulación arterial y venosa. Objetivo: Analizar las diferencias inmunológicas y farmacológicas, así como los desenlaces clínicos de una cohorte de pacientes con SAF primario y secundario. Materiales y métodos: Estudio de corte transversal que incluyó 352 pacientes con diagnóstico de SAF atendidos entre los arios 2014 y 2018. Se analizaron variables sociodemográficas, clínicas e inmunológicas y se realizó un análisis univariado y un análisis bivariado mediante la prueba chi-cuadrado para determinar diferencias entre los pacientes con SAF primario y SAF secundario. Finalmente, se hizo un análisis multivariado para buscar asociaciones con los desenlaces clínicos trombóticos en los pacientes con SAF. Resultados: La edad promedio de la población fue de 42,4 ± 14 años; el 84,6% correspondió a sexo femenino. El 67,6% de los pacientes tenía diagnóstico de SAF primario y un 32,4% de SAF secundario, siendo el lupus eritematoso sistémico (LES) la enfermedad asociada en un 84%. Dentro de los eventos trombóticos, el más frecuente fue la trombosis venosa profunda (17,3%), seguida por el ataque cerebrovascular (9,9%). En los eventos obstétricos existió una prevalencia del 39,4% para abortos. No se encontraron diferencias en el perfil sociodemográfico ni en el perfil inmunoserológico entre los pacientes con diagnóstico de SAF primario y aquellos con SAF secundario. Los eventos trombóticos tuvieron mayor frecuencia en el grupo de SAF primario, pero solo la tromboembolia pulmonar alcanzó significación estadís tica. Eventos obstétricos como los abortos no fueron diferentes entre ambos grupos. Dentro de los factores asociados a los eventos trombóticos, se encontró que el sexo femenino tiene una probabilidad 5 veces mayor de accidente cerebrovascular y 3 veces mayor de trombosis venosa profunda. Los anti- β2GPI tipo IgM aumentaron alrededor de 3 veces la probabilidad de presentar abortos en mujeres con SAF. Conclusión: Se presenta una de las cohortes colombianas más grandes de pacientes con SAF reportadas hasta el momento en la literatura. La población es comparable clínica y sociodemográficamente con lo encontrado en otros estudios, aunque la prevalencia de SAF primario fue mayor y las complicaciones trombóticas fueron menores. La tromboembolia pulmonar fue significativamente mayor en el grupo de SAF primario.

ABSTRACT Introduction: Antiphospholipid syndrome (APS) is a systemic autoimmune disease charac terized by recurrent thrombosis that can affect the arterial and venous circulation. Objective: To analyze the immunological and pharmacological differences, as well as the clinical outcomes of a cohort of patients with primary APS and secondary APS. Materials and methods: A retrospective cohort study was conducted that included 352 records of patients diagnosed with APS and treated between 2014 and 2018. A description is pre sented of the sociodemographic, clinical, and immunological profile of the population. A bivariate analysis performed using the chi-squared test to determine differences between groups with primary APS and secondary APS, and finally a multivariate analysis to search for associations with thrombotic clinical outcomes in patients with APS. Results: The mean age was 42.4 ± 14 years, and 84.6% were females. Two-thirds (67.6%) of the patients had a diagnosis of primary APS, and 32.4% of secondary APS, of which 84% were associated with systemic lupus erythematosus (SLE). Among the thrombotic events, the most frequent were deep vein thrombosis (17.3%) and stroke (9.9%). Obstetric events were frequent, with a prevalence of 39.4% for miscarriages. No differences were found in the sociodemographic or immunoserological profile when comparing the group of primary vs. secondary APS. Thrombotic events were more frequent in the primary APS group, although only pulmonary embolism reached statistical significance. There were no differences bet ween the two groups as regards obstetric events, such as miscarriages. Women were found to be 5 times more likely to have a stroke and 3 times more to have deep vein thrombosis. The anti-β2GPI type IgM increased the probability of presenting miscarriages about 3 times in women with APS. Conclusion: The study contains one of the largest Colombian cohorts with APS reported so far, and although it is both clinically and sociodemographically similar to other cohorts, there is a higher prevalence of primary APS. There was a lower frequency of thrombotic complications compared to other cohorts. Patients with primary APS had a tendency to develop thrombosis, as has already been reported in the literature.

Clinical and laboratory characteristics of Brazilian versus non-Brazilian primary antiphospholipid syndrome patients in AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) clinical database and repository

Abstract Background: Antiphospholipid syndrome (APS) is characterized by episodes of thrombosis, obstetric morbidity or both, associated with persistently positive antiphospholipid antibodies (aPL). Studying the profile of a rare disease in an admixed population is important as it can provide new insights for understanding an autoimmune disease. In this sense of miscegenation, Brazil is characterized by one of the most heterogeneous populations in the world, which is the result of five centuries of interethnic crosses of people from three continents. The objective of this study was to compare the clinical and laboratory characteristics of Brazilian vs. non-Brazilian primary antiphospholipid syndrome (PAPS) patients. Methods: We classified PAPS patients into 2 groups: Brazilian PAPS patients (BPAPS) and PAPS patients from other countries (non-BPAPS). They were compared regarding demographic characteristics, criteria and non-criteria APS manifestations, antiphospholipid antibody (aPL) profile, and the adjusted Global Antiphospholipid Syndrome Score (aGAPSS). Results: We included 415 PAPS patients (88 [21%] BPAPS and 327 [79%] non-BPAPS). Brazilian patients were significantly younger, more frequently female, sedentary, obese, non-white, and had a higher frequency of livedo (25% vs. 10%, p < 0.001), cognitive dysfunction (21% vs. 8%, p = 0.001) and seizures (16% vs. 7%, p = 0.007), and a lower frequency of thrombocytopenia (9% vs. 18%, p = 0.037). Additionally, they were more frequently positive for lupus anticoagulant (87.5% vs. 74.6%, p = 0.01), and less frequently positive to anticardiolipin (46.6% vs. 73.7%, p < 0.001) and anti-ß2-glycoprotein-I (13.6% vs. 62.7%, p < 0.001) antibodies. Triple aPL positivity was also less frequent (8% vs. 41.6%, p < 0.001) in Brazilian patients. Median aGAPSS was lower in the Brazilian group (8 vs. 10, p < 0.0001). In the multivariate analysis, BPAPS patients still presented more frequently with livedo, cognitive dysfunction and sedentary lifestyle, and less frequently with thrombocytopenia and triple positivity to aPL. They were also less often white. Conclusions: Our study suggests a specific profile of PAPS in Brazil with higher frequency of selected non-criteria manifestations and lupus anticoagulant positivity. Lupus anticoagulant (not triple positivity) was the major aPL predictor of a classification criteria event.

Primary antiphospholipid syndrome in children: experience from two tertiary centres in South India

Background: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by the presence of episodes of vascular thrombosis, recurrent fetal loss and other clinical features in the presence of antiphospholipid antibodies. The aim of the study was to analyze the clinical manifestations and immunologic profile of children presenting with APS.Methods: Authors did a retrospective case record study of patients admitted with thrombotic events between September 2013 and August 2018 and identified patients with positive antiphospholipid antibodies. Children who had clinical features of active lupus were not included.Results: The clinical and immunologic profile of 7 pediatric patients presenting with APS over 5 years from 2013 to 2018 were analysed. Symptoms secondary to vascular thrombosis were limb swelling, stroke, gangrene of toes and Budd Chiari syndrome.Conclusions:APS though rare should be considered in the differential diagnosis of children presenting with thrombotic events. They need long term anticoagulants to prevent further episodes.

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.

Avaliação clínico-laboratorial de pacientes com síndrome antifosfolípide primária segundo a frequência de anticorpos antinucleares (FAN Hep-2) / Clinical and laboratory evaluation of patients with primary antiphospholipid syndrome according to the frequency of antinuclear antibodies (ANA Hep-2)

OBJETIVO: Avaliar a frequência de manifestações clínicas e laboratoriais em pacientes com síndrome antifosfolípide primária (SAFP) com anticorpos antinucleares positivos (FAN Hep-2+), comparados àqueles com esses anticorpos negativos (FAN Hep-2 -). PACIENTES E MÉTODOS: Estudo transversal em 58 pacientes (82,8 por cento mulheres) com SAFP. Foram avaliados os dados demográficos, clínicos, comorbidades, medicações e anticorpos antifosfolípides. RESULTADOS: Dos 58 pacientes incluídos no estudo, vinte (34,5 por cento) apresentaram presença de FAN Hep-2. Comparando-se o grupo de pacientes FAN Hep-2+ com aqueles FAN Hep-2 -, verificou-se que ambos os grupos de pacientes com SAFP não diferiram estatisticamente em relação aos dados demográficos, bem como em relação ao tempo de doença. Em relação às manifestações clínicas e laboratoriais, o grupo com FAN Hep-2 + apresentou maior frequência de trombose venosa profunda (85 versus 52,6 por cento, P = 0,04), uma frequência estatística e significativamente maior de anticardiolipina IgG (85 versus 52,6 por cento, P = 0,02) e uma tendência para anticardiolipina IgM (80 por cento versus 52,6 por cento, P = 0,05), bem como maiores medianas desses anticorpos [33 (0-128) versus 20 (0-120) GPL, P = 0,008] e [33 (0-120) versus 18,5 (0-120) MPL, P = 0,009]. Tal diferença não foi observada no que se refere a outras manifestações da SAF, presença de comorbidades, estilo de vida e uso de medicações. CONCLUSÃO: Pacientes com SAFP que apresentam FAN Hep-2+ têm maior frequência de trombose venosa profunda e anticardiolipinas IgG e IgM.

OBJECTIVE: To evaluate the frequency of clinical and laboratory manifestations in patients with primary antiphospholipid syndrome (PAPS) with positive antinuclear antibodies (ANA Hep-2+) compared to those in whom this antibody is negative (ANA Hep-2-). PATIENTS AND METHODS: This is a transversal study with 58 patients (82.8 percent females) with PAPS. Demographic and clinical data, comorbidities, medications, and antiphospholipid antibodies were evaluated. RESULTS: Twenty (34.5 percent) out of 58 patients were positive for ANA Hep-2. Comparing the group of patients ANA Hep-2+ with those that were ANA Hep-2-, it was observed that both groups of patients with APS did not show statistically significant differences regarding demographic data, as well as the duration of the disease. As for clinical and laboratorial manifestations, the ANA Hep-2+ group showed higher frequency of deep venous thrombosis (85 versus 52.6 percent, P = 0.04), a statistically higher frequency of anticardiolipin IgG (85 versus 52.6 percent, P = 0.02), and a tendency for anticardiolipin IgM (80 percent versus 52.6 percent, P = 0.05), as well as greater medians of those antibodies [33 (0-128) versus 20 (0-120) GPL, P = 0.008] and [33 (0-120) versus 18,5 (0-120) MPL, P = 0.009]. Such difference was not observed regarding other manifestations of APS, presence of comorbidities, lifestyle, and medications used. CONCLUSIONS: Patients with PAPS with ANA Hep-2+ have a higher frequency of deep venous thrombosis and anticardiolipin IgG and IgM.

Síndrome antifosfolípide primária e infecções por hepatites B e C / Primary antiphospholipid syndrome and hepatitis B and C infections

OBJETIVO: Avaliar a prevalência de sorologia positiva para hepatites B e C em pacientes com síndrome antifosfolípide (SAF) primária. PACIENTES E MÉTODOS: Estudo transversal de 47 pacientes com SAF primária (critérios de Sapporo). Foram avaliados os dados demográficos, clínicos, medicações e sorologias para hepatites B e C e PCR nos resultados positivos. RESULTADOS: A média de idade da população estudada foi de 38 ± 11 anos, sendo 80,8 por cento do sexo feminino e 68 por cento da cor branca. A média de duração da doença foi de 67 ± 61 meses (variando de 1 - 240 meses). Os eventos arteriais foram vistos em 61,7 por cento dos casos, os venosos em 51 por cento e os obstétricos em 38,3 por cento. Cinco (10,6 por cento) pacientes com SAF primária apresentaram sorologia positiva para hepatite B ou C. Desses, três pacientes foram positivos para anti-HBs, com anti-HBc positivo em apenas um deles, e os outros dois foram positivos para hepatite C. A análise da PCR qualitativa não detectou RNA do vírus C em nenhum desses dois pacientes positivos. CONCLUSÃO: Uma pequena percentagem de pacientes com SAF primária apresenta sorologia positiva para hepatites B e C, sendo em todos os casos pós-vacinal ou cicatriz sorológica.

OBJECTIVE: The objective of the present study was to evaluate the prevalence of hepatitis B and C serology in patients with primary antiphospholipid syndrome (APS). METHODS: This is a transversal study with 47 patients with primary APS (Sapporo's criteria). Demographic and clinical data, medications, and hepatitis B and C serologies, with PCR in positive cases, were evaluated. RESULTS: The study population had a mean age of 38±11 years, 80.8 percent were females, and 68 percent Caucasian. The mean duration of the disease was 67 ± 61 months (ranging from 1 to 240 months). Arterial events were seen in 61.7 percent of the patients, venous events in 51 percent, and obstetric events in 38.3 percent. Five (10.6 percent) patients with primary APS had positive serologies for hepatitis B or C. Three of them were positive for anti-HBs, and only one was anti-HBc positive; the other two patients were positive for hepatitis C. Qualitative PCR did not detect hepatitis C viral RNA in neither of the positive patients. CONCLUSION: A small percentage of patients with primary APS had positive serology for hepatitis B and C, and all represented cases post-vaccine or serology scar.

Hyperhomocysteinemia and primary antiphospholipid syndrome / Hiper-homocisteinemia e síndrome antifosfolípide primária

OBJETIVOS: Investigar a prevalência de hiper-homocisteinemia e suas possíveis associações clínicas e laboratoriais em pacientes com síndrome antifosfolípide primária (SAFP). PACIENTES E MÉTODOS: Estudo transversal de 27 pacientes (88% mulheres) com SAFP (critérios de Sapporo). Foram avaliados dados demográficos, clínicos, comorbidades, medicações, anticorpos antifosfolípides, colhendo-se uma amostra de sangue para dosagem da homocisteína, pela técnica de cromatografia líquida de alta resolução. RESULTADOS: Seis (22%) dos 27 pacientes apresentaram níveis de homocisteinemia acima dos valores normais (98,7 ± 8,9 versus 8,0 ± 2,9 »M, P = 0,0008). Comparando-se o grupo de pacientes com hiper-homocisteinemia com aquele que apresentava níveis normais, verificou-se que ambos os grupos de pacientes com SAFP não diferiram estatisticamente em relação aos dados demográficos (idade, cor branca, peso, altura e índice de massa corporal), bem como ao tempo de duração de doença (64 ± 39,6 versus 77,9 ± 61,3 meses, P = 0,32). Nenhum dos grupos apresentou diferenças quanto às manifestações da doença (eventos arteriais, venosos e obstétricos, trombose venosa profunda, tromboembolismo pulmonar, plaquetopenia, infarto agudo do miocárdio, angina e acidente vascular cerebral), comorbidades (hipertensão arterial e dislipidemia), ao estilo de vida (atividade física e tabagismos atual e pregresso), bem como ao uso de medicações (corticoide atual e pregresso, estatina, cloroquina e ácido acetilsalicílico). A prevalência e os títulos de anticorpos anticardiolipina foram semelhantes em ambos os grupos. CONCLUSÃO: A hiper-homocisteinemia pode ser detectada em cerca de um quarto da população com SAFP, sem associação com características distintivas clínicas e laboratoriais dessa doença.

OBJECTIVES: The objective of this study was to investigate the prevalence of hyperhomocysteinemia and its possible clinical and laboratorial associations in patients with primary antiphospholipid syndrome (PAPS). PATIENTS AND METHODS: This is a transversal study with 27 patients (88 percent women) with PAPS (Sapporo criteria). Demographic and clinical data, as well as comorbidities, medications, antiphospholipid antibodies, and blood concentrations of homocysteine, measured by high resolution liquid chromatography, were evaluated. RESULTS: Six (22 percent) out of 27 patients had high levels of homocysteine (98.7 ± 8.9 versus 8.0 ± 2.9 »M, P = 0.0008). Comparison between the group of patients with hyperhomocysteinemia and the group of patients with normal serum homocysteine levels did not show differences in the demographic data (age, white, weight, height, and body mass index) or in the duration of the disease (64 ± 39.6 versus 77.9 ± 61.3 months, P = 0.32). Differences in disease manifestations (arterial, venous, and obstetric events, deep venous thrombosis, pulmonary thromboembolism, thrombocytopenia, acute myocardial infarction, angina, and stroke), comorbidities (hypertension and hyperlipidemia), life style (physical activity, past and present smoking), as well as the use of medications (past and present use of corticosteroids, statins, chloroquine, and acetylsalicylic acid), were not observed between both groups. The prevalence and titers of anticardiolipin antibodies were similar in both groups of patients. CONCLUSION: Hyperhomocysteinemia can be detected in approximately one fourth of the PAPS patients, and it is not associated with distinct clinical and laboratorial characteristics of this disorder.

Retinal Vein Occlusion in Two Patients with Primary Antiphospholipid Syndrome

Primary antiphopholipid syndrome (APS) is a disease producing vascular thrombus with antiphospholipid antibody without association with autoimmune diseases as systemic lupus erythematosus. Retinal vein occlusion is a rare vascular manifestation in primary APS. We describe 2 cases of primary APS presenting with developing blurred vision. Each had central retinal vein occlusion and high titer of IgG anticardiolipin antibody.

A Case of Primary Antiphospholipid Syndrome with a Cerebrovascular Attack in a Child

The antiphospholipid syndrome is a multisystemic disorder comprising of venous and arterial thrombotic events, recurrent unexplained fetal losses, moderate thrombocytopenia, and a high frequency of neurologic events with laboratory findings of a positive lupus anticoagulant test or anticardiolipin antibody. It may occur in association with other disorders, particularly autoimmune diseases, in which case it is referred to as secondary antiphospholipid syndrome. But when it occurs without obvious underlying disease, it is primary antiphospholipid syndrome. We report a case of primary antiphospholipid syndrome in a 5 year old female child who had a cerebrovascular attack, moderate thrombocytopenia and splenomegaly.

A Case of Primary Antiphospholipid Syndrome Presented with Membranoproliferative Glomerulonephritis pattern / 대한신장학회잡지

Though the renal abnormality is usually accompanied in the patient with antiphospholipid syndrome, it is not frequent that the symptoms of antiphospholipid syndrome are confined to kidney. We experienced a case of 40-year-old woman suffered from intermittently developed nephrotic range proteinuria combined with generalized edema during last 10 years. She had no past history of thrombosis, spontaneous abortion, or symptoms related to SLE. Laboratory findings revealed that she had nephrotic syndrome. Her antinuclear antibody was negative, but her serum antiphospholipid IgG antibody level was 32 GPL IU. In the renal biopsy specimen, half of the glomeruli showed global sclerosis and remaining glomeruli showed chronic thrombotic microangiopathy and membranoproliferative glomerulonephritis without immune deposit. Combined therapy with steroid and aspirin improve the amount of proteinuria, and she is free from edema without deterioration of renal function during follow up. Though this case lacked the typical symptoms of antiphospholipid syndrome, her glomerulonephritis presented with membranoproliferative pattern may be associated with it. We report this case with a review of literatures.

A Case of Primary Antiphospholipid Syndrome associated with Chronic Hepatitis C / 대한내과학회지

Hepatitis C was known to be associated with many autoimmune disease, but the pathophysiology was not well understood. Antiphospholipid antibodies, which are autoantibodies detected to negatively charged phospholipids, are sometimes detected in infectious diseases, including sypilis and autoimmun diseases, such as systemic lupus erythematosus and unknown etiology. A few reports suggested that there is a relation between primary antiphospholipid syndrome and hepatitis C. We recently experienced a case of primary antiphospholipid syndrome with chronic hepatitis C in 20 years old womam who developed cerebral infartion. A brief review of related literature is presented.

Primary antiphospholipid syndrome associated with non-ischemic central retinal vein occlusion

Antiphospholipid syndrome is a disease producing vascular thrombosis with antiphospholipid antibody and usually associated with systemic lupus erythematosus. It is called primary antiphospholipid syndrome(PAPS) if it does not have the clinical features of collagen vascular disorder. Ocular manifestations of antiphospholipid syndrome include retinal vascular disorder, anterior ischemic optic neuropathy, and amaurosis fugax. Ocular manifestations are much less common in primary antiphospholiped syndrme than antiphospholipid syndrome associated with systemic lupus erythematosus. We experienced two cases of non-ischemic central retinal vein occlusion which were associated with primary anti phospholipid syndrome in two female patients, who complained sudden decrease of visual acuity in one eye. We report the cases with review of the literatures.