Anorectal diseases in patients with Antiphospholipid syndrome: a cross-sectional study

Abstract Background: Hemorrhoid disease (HD) is one of the most common gastrointestinal complaints worldwide, affecting 4.4% of the general population in the United States. Since antiphospholipid syndrome (APS) may lead to intra-abdominal thrombosis, one may expect that this condition can impact the risk for HD development. Additionally, as APS patients are more prone to thrombosis and treatment with anticoagulants may increase risk of bleeding, one may also infer that rates of HD complications may be higher in this scenario. Nevertheless, no data in these regards have been published until now. The objective of the present study is to evaluate frequency of HD and describe its complications rates in antiphospholipid syndrome APS patients. Methods: We consecutively invited patients who fulfilled APS criteria to undergo proctological examination. After examination, patients were divided in two groups, based on the presence of HD, and compared regarding different clinical manifestations and antiphospholipid profile. We performed the analysis of the data, using chi-square and Mann Whitney U when applicable and considering a significance level of 0.05. Multivariate regression analysis included age and variables with p < 0.10 in the bivariate analysis. Results: Forty-one APS patients agreed to undergo proctological examination. All were female and overall median age was 43 (36-49). Seventeen (41.4%) patients were diagnosed with HD, with the following frequency distribution: 7 internal (41.2%), 4 external (23.5%) and 5 mixed hemorrhoids (29.4%). Of the internal hemorrhoids, 5 patients were classified as grade I (71.4%), 1 grade II (14.3%), and 1 grade IV (14.3%). Prior gestation ( p = 0.067) and constipation ( p = 0.067) correlated with a higher frequency of HD. In multivariate analysis, constipation remained as an important risk factor (OR 3.92,CI95% 1.03-14.2, p = 0.037). Five out of 17 patients (29.4%) reported anal bleeding, but it did not correlate with warfarin dose ( p = 0.949). Surgical treatment was indicated for 10 patients (58.8%). Other anorectal findings were anal fissure, plicoma, condyloma and one chlamydial retitis. Conclusion: We found an unexpected high frequency of hemorrhoids in APS patients, with a great proportion requiring surgical treatment.(AU)

Pigmented purpura and cutaneous vascular occlusion syndromes

Abstract: Purpura is defined as a visible hemorrhage in the skin or mucosa, which is not evanescent upon pressure. Proper classification allows a better patient approach due to its multiple diagnoses. Purpuras can be categorized by size, morphology, and other characteristics. The course varies according to the etiology, as do the diagnostic approach and treatment. This review discusses pigmented purpuras and some cutaneous vascular occlusion syndromes.

Proliferative glomerulonephritis and primary antiphospholipid syndrome

Little is known regarding the association of primary antiphospholipid syndrome APLS and proliferative glomerulonephritis GN. We describe a biopsy-documented case with primary APLS and proliferative GN with no evidence of thrombotic microangiopathy TMA, and in the absence of other manifestations of systemic lupus erythematosus SLE. She presented initially with left popliteal deep venous thrombosis and nephrotic syndrome. Her first pregnancy at the age of 26 years resulted in intra-uterine fetal death at term. Two subsequent pregnancies ended up with miscarriages at 3 and 4 months of gestation. Urinalysis revealed glomerular red blood cells of 1.0000.000/ml and granular cast; proteinuria of 13.4 grams/24 hours, which was non-selective; hemoglobin 12 gm/dl, normal white blood cell and platelets; serum albumin 2.6 gm/dl; anti-nuclear antibody ANA and anti DNA were negative and complement levels normal. Lupus anticoagulant was positive leading to a diagnosis of primary APLS. The biopsy findings were consistent with membranoproliferative GN. She continued to have steroid-resistant proteinuria, but stable renal function after a 12-year follow up period. She had 2 pregnancies during this period and was delivered at term using caesarian section. She received heparin during the pregnancies. Later she developed hypertension easily controlled by atenolol. This case provides evidence that primary APLS can be associated with proliferative GN due to immune deposits and not only TMA as previously reported, and in the complete absence of SLE. Performing more renal biopsies in this group of patients may disclose a greater prevalence of proliferative GN and may help in devising a rationale for treatment

Cardiac involvement in primary antiphospholipid syndrome and anticardiolipin positive systemic lupus erythematosus

Since the recognition of antiphospholipid syndrome [APS], many cardiac manifestations have been reported in association with the anti-phospholipid [aPL] antibodies. The APS syndrome can be either primary or secondary to an underlying condition, most commonly systemic lupus erythematosus [SLE]. Echocardiographic studies have disclosed heart valve abnormalities in about a third of patients with APS. The aPL antibodies have been suggested to be a pathogenetic factor in the cardiac abnormalities. To evaluate prospectively the prevalence of cardiac abnormalities in patients with SLE and primary antiphospholipid syndrome [PAPS], and correlate these data with serum level of anticardiolipin [aCL] antibodies. Sixty three patients with SLE [62 females and 1 male] were enrolled and divided into two groups according to the presence [Group III, n=35] or absence of aCL [Group II, n=28]. Ten patients with PAPS [7 females and 3 males] were recruited [Group IV, n=10]. In addition, 23 healthy age and sex matched controls, were included [Group I] The serum levels of IgG and IgM aCL antibodies were measured for all patients and controls by a st and ardized ELISA test. All patients and controls also, underwent st and ard two-dimensional and Doppler echocardiographic examination within a week of serum testing. The aCL IgG antibodies were positive in 30 of 63 [47.6%] patients with SLE, in all 10 [100%] patients with PAPS, and in 1 of 23 [4.5%] control individuals. The aCL IgM antibodies were positive in 12 of 63 [19%] patients with SLE, in 4 of 10 [40%] patients with PAPS, and in none of the control individuals. Both IgG and IgM aCL antibodies were positive in 7 of 63 [11%] patients with SLE and in 4 of 10 [40%] patients with PAPS. Echocardiographic findings showed normal heart in all control subjects [group I], and in 16 [57%] of SLE patients with absence of elevated aCL levels [group II], 10 [28.5%] of SLE patients with elevated aCL levels [group III] and 3 [30%] of patients with PAPS [group IV]. Valvular lesions were detected in 7 patients [25%] in group II, 15 [43%] in group III and 7 [70%] in group IV. Pericardial effusion was SLE: systemic lupus erythematosus, PAPS:primary antiphospholipid syndrome, aPL:anti-phospholipid antibodies, aCL:anticardiolipin antibodies. detected in 3 patients [11%] in group II, 10 [28.5%] in group III, and 1 [10%] in group IV. Myocardial dysfunction was detected in 1 patient [3.5%] in group II, 7 [20%] in group III and 2 [20%] in group IV Left ventricular hypertrophy was detected in 2 patients [7%] in group II, 5 [14%] in group III and 1 [10%] in group IV. Pulmonary hypertension was detected in none [0%] of patients in group II, 4 [11.5%] in group III and 2 [20%] in group IV Diastolic dysfunction was detected in 12 patients [43%] in group II, 14 [40%] in group III and 4 [40%] in group IV. Valvular lesions, myocardial dysfunction and pulmonary hypertension in patients with PAPS and SLE are associated with elevated aCL antibodies. There was no significant difference in the frequency of cardiac involvement between patients with increased aCL antibodies in SLE and those with PAPS. Thus, aCL may play an important role in the pathogenesis of valvular lesions as well as myocardial abnormalities

Síndrome anticuerpo antifosfolípido: cómo llegamos a su diagnóstico? / Anti-phosppholipid antibody syndrome: how do we arrive to its diagnosis?

Nuestro objetivo es presentar un razonamiento diagnóstico para la detección del síndrome anticuerpo antifosfolípido, teniendo en cuenta las variadas manifestaciones cutáneas del mismo. La sintomatología clínica, la biopsia de piel, el análisis hematológico e inmunológico incluyendo anticuerpos anticardiolipinas y el estudio de la coagulación constituyen la metodología para llegar al diagnóstico de certeza del síndrome. Las manifestaciones clínicas más relevantes son trombosis arteriales y venosas en cualquier segmento del árbol vascular y abortos recurrentes. Además puede presentar cefaleas e hipertensión arterial. Hacemos hincapié en el estudio de coagulación, sus pruebas de selección (APTT-dRVVT-TCK) y la detección de anticuerpos anticardiolipinas por enzimoinmunoanálisis

Sindrome antifosfolipidico catastrofico. Comunicacion de dos formas de presentación / Catastrophic antiphopholipidid syndrome. Communication of two forms of presentation

El síndrome antifosfolipídico (SAFL) se caracteriza por la presencia de abortos espontáneos, trombosis arteriales y venosas, trombocitopenia y el hallazgo de anticuerpos antifosfolipídicos en sangre. En una minoría de los casos tiene un curso rápidamente fatal. Presentamos dos pacientes jóvenes con lupus eritematoso sistémico (LES) y anticuerpos antifosfolipídicos, que desarrollaron un cuadro agudo que rápidamente les provocó la muerte. Si bien el cuadro clínico y de laboratorio fue distinto - en una paciente había predominantemente microtrombosis con anemia hemolítica microangiopática, similar a la púrpura trombótica trombocitopénica (PTT), mientras que en la otra se evidenció trombosis de vasos pequeños y medianos, sin hemólisis-, en la autopsia de ambas pacientes se encontraron trombosis en múltiples órganos, configurando el cuadro de SAFL catastrófico. No hay casos descriptos de este síndrome desencadenado por neumonía por Pneumocistis carinii como se observó en una de nuestras pacientes.

Síndrome antifosfolípido / Antiphospholipid syndrome

El síndrome antifosfolípido es un proceso patológico cuya manifestación principal es la trombosis. Existen numerosas teorías, todas respaldadas por datos, acerca de la coagulación patológica; sin embargo todavía no se conoce el mecanismo exacto de la trombosis responsable de als manifestaciones clínicas del síndrome. Actualmente se eestá prestando mucha atención a una proteína plasmática, la & 2 glucoproteina 1, la cual sería el epítope reconocido por el anticuerpo antifosfolípido. Las pruebas analíticas para diagnosticar síndrome antifosfolípido incluyen: anticuerpos anticardiolipina, anticoagulante lúpico; las cuales pueden ser difíciles de realizar e interpretar. El tratamiento de elección para la mayor parte de las manifestaciones del síndrome, es la anticoagulación.

Flank ulcer in a patient with primary antiphospholipid syndrome

A 32-year-old woman had a recurrent shallow ulcer on the flank. A biopsy specimen showed thromboses in the dermal vessels and she was found to have circulating antiphospholipid antibody with no associated systemic disease. A clean ulcer developed on the flank of a patient with primary antiphospholipid syndrome is considered to be a rarely encountered/unusual presentation of this syndrome.

Sindrome antifosfolipide e gestacao / Antiphospholipid syndrome and pregnancy

Os anticorpos antifosfolipides sao um grupo de auto-anticorpos contra fosfolipidios de carga negativa. Sua presenca, em combinacao com perda gestacional de repeticao, trombose arterial ou venosa ou trombocitopenia define a chamada sindrome antifosfolipide. Nas pacientes portadoras dessa sindrome o risco de perda gestacional e bastante elevado, e mesmo as gestacoes viaveis sao de alto risco para a ocorrencia de complicacoes como pre-eclampsia, crescimento intra-uterino retardado, sofrimento fetal anteparto e parto prematuro. O mecanismo fisiopatologico envolvido permanece desconhecido. Varios tratamentos, como a aspirina, a prednisona, a heparina e a imunoglobulina humana intravenosa, tem sido propostos, na tentativa de evitar a alta incidencia de complicacoes maternas e perinatais associadas a sindrome

Vasculitis en el síndrome antifosfolípido

La asociación de vasculitis con enfermedades del tejido conectivo es bien conocida y con frecuencia producen eventos oclusivos vasculares; informamos los casos de un paciente con lupus eritematoso sistémico y otro con artritis reumatoidea, con vasculitis reumatoidea y síndrome antifosfolípido secundario, que desarrollaron oclusión arterial en miembros inferiores y que requirieron amputación en quienes se documentó la presencia de vasculitis, vasculopatía y trombo organizado. La pérdida dramática de tejido es rara para vasculitis aislada o síndrome antifosfolípido primario. Sugerimo en pacientes con estas enfermedades que cursen con compromiso vascular rápidamete progresivo sospechar la asociación de vasculitis y trombosis e iniciar manejo agresivo con inmunosupresores, antiagregantes plaquetarios y vasodilatadores

Infertilidad y síndrome antifosfolípido primario: reporte de un caso / Infertility and primary antiphospholipid syndrome: case report

El síndrome antifosfolípido primario cursa con pérdidas repetidas del embarazo siendo su patogenia controversial. La combinación de prednisona y aspirina parece ser una buena alternativa para la terapia del proceso. Se reporta el caso de una paciente de 35 años con siete pérdidas recurrentes del embarazo que en su siguiente gestación se le diagnosticó el síndrome antifosfolípido primario primario. La enfermedad fue investigada por técnicas de evaluación para el anticoagulante lúpico y la detección de anticuerpos anticardiolipina por enzimoinmunoensayo. Se trató con prednisona y aspirina (30 y 100 mg diarios respectivamente). La gestación transcurrió normalmente hasta la semana 35 cuando se interrumpió quirúrgicamente por compromiso del bienestar fetal. Se obtuvo recién nacida sana. En nuestro caso la evolución materna fue satisfactoria y conseguimos la supervivencia fetal con el esquema de tratamiento citado. Pensamos que la combinación de un inmunosupresor y un antiagregante plaquetario es un buen esquema terapéutico para la obtención de productos vivos en pacientes portadoras de la enfermedad y pérdidas gestacionales recurrentes

Hepatic veno-occlusive disease as a presentation of antiphospholipid antibody syndrome in a young kuwaiti woman

A young Kuwaiti woman presented with severe jaundice, constitutional symptoms and deranged liver function tests suggestive of hepatitis. Investigations, including a liver biopsy, showed the presence of hepatic veno-occlusive disease [HVOD] associated with antiphospholipid antibody syndrome [APS]. Clinical and laboratory abnormalities improved rapidly with anticoagulation treatment with warfarin. During the follow-up of over 1 year she remained completely normal without recurrence of symptoms. HVOD is a known but rare presentation of APS. Recognition and treatment of this condition at an early stage can be rewarding