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1.
Hematology ; 26(1): 225-239, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33594951

RESUMO

OBJECTIVE: As COVID-19 is a new emerging disease, the hematological/immunological changes that develop in the infected patients remain unknown. This study aims to systematically review the hematologic autoimmune complications in these patients. METHOD: Data from three online databases including Medline (via PubMed), Scopus and Web of Science were searched on 19 December 2020, and after excluding duplicate, irrelevant and inappropriate records, eligible documents were identified. Afterwards, information such as patients' history, presentations, paraclinical data, treatment course and outcome were extracted from the records. RESULTS: A total of 58 documents were considered to be eligible for data extraction which described 94 patients with COVID-19 who developed hematologic autoimmune disorder in their course of infection. Of these patients with COVID-19, the most common hematologic autoimmune disorder was immune thrombocytopenic purpura (55 cases) followed by autoimmune hemolytic anemia (22 cases). Other hematologic autoimmune disorders include antiphospholipid syndrome, thrombotic thrombocytopenic purpura, Evans syndrome and autoimmune neutropenia. CONCLUSION: The current study would help us to always consider an autoimmune etiology for cases with abnormal hematologic finding which further lead to an appropriate treatment of the patients, especially when the symptoms present in about 1-2 weeks after the first manifestation of the infection symptoms. Maybe, at least in this pandemic, it should be recommended to evaluate patients with unexpected and unexplained decrease in their hemoglobulin or platelet count for COVID-19. Another challenging issue is the treatment options. Given the multiorgan involvement and multifaceted nature of the infection, an individualized approach should be taken for each patient.


Assuntos
Doenças Autoimunes/etiologia , Doenças Hematológicas/etiologia , Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/etiologia , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/etiologia , Doenças Autoimunes/sangue , Doenças Hematológicas/sangue , Humanos , Neutropenia/sangue , Neutropenia/etiologia , Púrpura Trombocitopênica Idiopática/sangue , Púrpura Trombocitopênica Idiopática/etiologia , Púrpura Trombocitopênica Trombótica/sangue , Púrpura Trombocitopênica Trombótica/etiologia , Trombocitopenia/sangue , Trombocitopenia/etiologia
2.
Clin Immunol ; 214: 108388, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32200114

RESUMO

Anti-phospholipid antibodies (aPL) and lupus anticoagulant (LAC) represent diagnostic criteria for systemic lupus erythematosus (SLE) and underlie anti-phospholipid syndrome (APS) in patients with and without SLE. 526 healthy controls and 1633 SLE and 1835 primary APS (PAPS) patients were evaluated. LAC was assessed by hexagonal phase phospholipid neutralization assay (HPPNA), diluted Russell viper venom test (dRVVT), and platelet neutralization procedure (PNP). ß2-glycoprotein-I and cardiolipin IgG, IgM, and IgA antibodies (aCL-IgG, aCL-IgM, aCL-IgA) were measured. 222/1633 SLE patients had APS based on the nine-test panel, which afforded the highest sensitivity (74%) and negative predictive value (90%) but lowest specificity (52%). HPPNA was the most sensitive individual test at 52%. The nine-test panel yielded the greatest sensitivity for aPL detection (70%) relative to HPPNA, the most sensitive individual test (36%) in PAPS. Superior sensitivity of a nine-test aPL panel has major implications for preventing potentially fatal thrombotic events in SLE and PAPS.


Assuntos
Síndrome Antifosfolipídica/diagnóstico , Lúpus Eritematoso Sistêmico/complicações , Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/etiologia , Humanos , Inibidor de Coagulação do Lúpus/sangue , Lúpus Eritematoso Sistêmico/sangue , Testes de Função Plaquetária , Valor Preditivo dos Testes , Tempo de Protrombina , Estudos Retrospectivos , Sensibilidade e Especificidade , Trombose/prevenção & controle , beta 2-Glicoproteína I/sangue
3.
Am J Case Rep ; 21: e919037, 2020 Jan 18.
Artigo em Inglês | MEDLINE | ID: mdl-31953377

RESUMO

BACKGROUND Antiphospholipid syndrome (APS) is a rare autoimmune disease characterized by arterial, venous, and small-vessel thrombosis, pregnancy-related morbidity and the presence of antiphospholipid antibodies such as anticardiolipin antibody, and/or anti-beta2-glycoprotein I. In the recent years, APS was observed in patients with solid tumors and the renal cancer, lung carcinoma and breast tumors were the most common tumors linked with APS. CASE REPORT A 53-year-old female presented with pain and pitting edema of left lower extremity that had begun 6 months prior to hospitalization. Deep vein thrombosis (DVT) in the popliteal vein diagnosed by Doppler ultrasonography and the patient was treated with heparin followed by warfarin. Following subdural hematoma, anticoagulant therapy was stopped, and the patient underwent craniotomy. One month later, the patient returned with pain and DVT diagnosed in its right leg. Laboratory tests showed high levels of lupus anticoagulant, IgM and IgG anticardiolipin antibodies. Following a high alkaline phosphatase, diffuse bone marrow involvement was found by whole body bone scan. Looking to find primary tumor, a large infilterable lesion in gastric was seen by endoscopic images, and biopsy histopathology showed a signet ring cell adenocarcinoma. The patient refused chemotherapy and died 6 months after diagnosis. CONCLUSIONS APS is associated with gastric signet ring cell adenocarcinoma.


Assuntos
Síndrome Antifosfolipídica/etiologia , Carcinoma de Células em Anel de Sinete/complicações , Neoplasias Gástricas/complicações , Evolução Fatal , Feminino , Humanos , Pessoa de Meia-Idade
4.
Lupus ; 28(9): 1158-1166, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31299879

RESUMO

INTRODUCTION: Although extensively characterized in the outpatient setting, systemic lupus erythematosus (SLE) in the hospitalization wards is still scarcely portrayed, particularly in the perspective of its evolution over the years. METHODS: Retrospective analysis of SLE patients hospitalized in the Department of Autoimmune Diseases of a university hospital during a 20-year period (1995-2015), describing hospitalization characteristics, causes and predictors of outcome. RESULTS: A total of 814 hospitalizations concerning 339 patients were analysed. The main causes of admission were flare (40.2%), infection (19.2%), diagnostic procedures (18.8%) and thrombotic events (5.4%). Therapy with cyclophosphamide (odds ratio (OR) 1.908, p = 0.047) was associated with admission due to infection, while antimalarials displayed a protective effect (OR 0.649, p = 0.024). Nearly 3.9% of patients required admission to an intensive care unit, with associated antiphospholipid syndrome (OR 7.385, p = 0.04) standing as a predicting factor for this outcome. Readmission at 30 days occurred in 5.8% of patients, with thrombocytopenia (OR 6.007, p = 0.002) and renal involvement (OR 3.362, p = 0.032) featuring as predicting factors. Eight patients died, with antiphospholipid syndrome (OR 26.814, p = 0.02) and thrombocytopenia (OR 31.523, p = 0.01) being associated with mortality. There was no significant variation in patients' demographics or admission causes across the 20-year period, except for a decrease in admissions due to thrombotic and musculoskeletal causes. Recently, an increase in the use of mycophenolate mofetil and lower doses of glucocorticoids were noted. CONCLUSION: While demographics of SLE hospitalizations have not markedly changed over the past 20 years, changes in therapy patterns were observed. Thrombocytopenia, antiphospholipid syndrome and renal involvement featured as predictors of poor outcome.


Assuntos
Síndrome Antifosfolipídica/epidemiologia , Hospitalização/estatística & dados numéricos , Lúpus Eritematoso Sistêmico/terapia , Trombocitopenia/epidemiologia , Adulto , Síndrome Antifosfolipídica/etiologia , Ciclofosfamida/administração & dosagem , Feminino , Humanos , Imunossupressores/administração & dosagem , Lúpus Eritematoso Sistêmico/mortalidade , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Pessoa de Meia-Idade , Readmissão do Paciente/estatística & dados numéricos , Estudos Retrospectivos , Espanha , Trombocitopenia/etiologia
5.
Reumatismo ; 71(2): 92-98, 2019 Jul 09.
Artigo em Inglês | MEDLINE | ID: mdl-31309781

RESUMO

A 62-year-old man with a history of systemic sclerosis was admitted with diffuse alveolar hemorrhage and acute kidney injury without clinical data suggestive of glomerulonephritis. Laboratory tests showed anemia, leukocytosis with neutrophilia, thrombocytopenia, elevated serum creatinine and metabolic acidosis. Antinuclear antibodies were positive at a titer of 1/640 (speckled, 1/160; nucleolar, 1/320) while rheumatoid factor, anti Scl-70, anti-centromere, anti-neutrophil cytoplasmic antibody and anti-glomerular basement membrane antibodies were negative and serum complement levels were within normal range. During the following days, the patient developed multiple organ failure and, eventually, died. Lupus anticoagulant was revealed positive after the patient's death, suggesting a catastrophic antiphospholipid syndrome. Clinical data and autopsy were consistent with this diagnosis.


Assuntos
Síndrome Antifosfolipídica/etiologia , Escleroderma Sistêmico/complicações , Doença Catastrófica , Humanos , Masculino , Pessoa de Meia-Idade
6.
Front Immunol ; 10: 1609, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31354742

RESUMO

Antiphospholipid antibodies (aPLs) comprise a diverse family of autoantibodies targeted against proteins with the affinity toward negatively charged phospholipids or protein-phospholipid complexes. Their clinical significance, including prothrombotic potential of anti-cardiolipin antibodies (aCLs), anti-ß2-glycoprotein I antibodies (aß2-GPIs), and lupus anti-coagulant (LA), is well-established. However, the ontogeny of these pathogenic aPLs remains less clear. While transient appearance of aPLs could be induced by various environmental factors, in genetically predisposed individuals these factors may eventually lead to the development of the antiphospholipid syndrome (APS). Since the first description of APS, it has been found that a wide variety of microbial and viral agents influence aPLs production and contribute to clinical manifestations of APS. Many theories attempted to explain the pathogenic potential of different environmental factors as well as a phenomenon termed molecular mimicry between ß2-GPI molecule and infection-relevant structures. In this review, we summarize and critically assess the pathogenic and non-pathogenic formation of aPLs and its contribution to the development of APS.


Assuntos
Autoimunidade , Meio Ambiente , Exposição Ambiental , Animais , Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/epidemiologia , Síndrome Antifosfolipídica/etiologia , Síndrome Antifosfolipídica/prevenção & controle , Autoanticorpos/imunologia , Infecções Bacterianas/complicações , Infecções Bacterianas/microbiologia , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/complicações , Exposição Ambiental/efeitos adversos , Humanos , Microbiota , Micoses/complicações , Micoses/microbiologia , Vacinas/efeitos adversos , Viroses/complicações , Viroses/virologia
7.
RMD Open ; 5(1): e000924, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31168416

RESUMO

Objective: To perform a systematic literature review (SLR) informing the European Lmmendations for the management of antiphospholipid syndrome (APS) in adults. Methods: A SLR through January 2018 was performed. Research questions were constructed using the Patient, Intervention, Comparator, Outcome (PICO) format. We included data from articles that reported on each relevant intervention. Summary effect estimates were calculated for direct comparison studies that matched the PICO question exactly, and for studies with the relevant intervention and comparator. When meta-analyses were available, we used these estimates. Results: From 7534 retrieved articles (+15 from hand searches), 188 articles were included in the review. In individuals with high-risk antiphospholipid antibody (aPL) profile without prior thrombotic or obstetric APS, two meta-analyses showed a protective effect of low-dose aspirin (LDA) against thrombosis. Two randomised controlled trials (RCTs) and three cohort studies showed no additional benefit of treatment with vitamin K antagonists at target international normalised ratio (INR) 3-4 versus INR 2-3 in patients with venous thrombosis. In patients with arterial thrombosis, two RCTs and two cohort studies showed no difference in risk of recurrent thrombosis between the two target INR groups. One open-label trial showed higher rates of thrombosis recurrences in triple aPL-positive patients treated with rivaroxaban than those treated with warfarin. RCTs and cohort studies showed that combination treatment with LDA and heparin was more effective than LDA alone in several types of obstetric APS. SLR results were limited by the indirect evidence and the heterogeneity of patient groups for some treatments, and only a few high-quality RCTs. Conclusion: Well-designed studies of homogeneous APS patient populations are needed.


Assuntos
Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/terapia , Complicações na Gravidez/diagnóstico , Complicações na Gravidez/terapia , Adulto , Fatores Etários , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/etiologia , Gerenciamento Clínico , Feminino , Humanos , Lúpus Eritematoso Sistêmico/complicações , Avaliação de Resultados em Cuidados de Saúde , Gravidez , Complicações na Gravidez/etiologia , Ensaios Clínicos Controlados Aleatórios como Assunto , Trombose/diagnóstico , Trombose/etiologia , Trombose/prevenção & controle , Trombose/terapia
9.
Biochem Biophys Res Commun ; 512(1): 72-78, 2019 04 23.
Artigo em Inglês | MEDLINE | ID: mdl-30871774

RESUMO

Accelerated atherosclerosis often occurs in patients with antiphospholipid syndrome (APS), and auto-antibodies to ß2 glycoprotein I (anti-ß2GPI) are confirmed as pathogenic antibodies to APS. Our previous studies have demonstrated that the conversion of mouse peritoneal macrophages into foam cells could be enhanced by co-existence of ß2GPI and anti-ß2GPI IgG, but this phenomenon has not been explored in vivo. Here, we present a mouse model to observe the effect of anti-ß2GPI IgG in the development of atherosclerosis. Male ApoE-deficient mice were intraperitoneally injected with anti-ß2GPI IgG (100 µg/mouse) and homologous control IgG (100 µg/mouse) every week for 16 weeks. Plasma lipid composition, magnetic resonance imaging (MRI) and histological staining were used to evaluate vascular inflammation, lumen stenosis and plaque stability. The results showed that the levels of total cholesterol, triglycerol and low-density lipoprotein-cholesterol in plasma were not changed in all mice fed with high-fat diet, but the level of high-density lipoprotein-cholesterol was lower and the atherosclerosis index was significantly increased in HD + anti-ß2GPI group than in other high-fat diet groups. In addition, compared with NR IgG-treated mice, anti-ß2GPI IgG-treated mice showed more lipid deposition in the carotid artery, markedly narrowed arteriolar lumen as well as higher MMP-9 expression, more macrophages and fewer collagen fibers in the aortic arch root. Furthermore, the aortic mRNA levels of TNF-α, IL-1ß, and MCP-1 were significantly increased in anti-ß2GPI IgG-treated mice. Together, these data indicate that anti-ß2GPI IgG increases vascular inflammation, aggravates atherosclerosis and promotes the formation of vulnerable plaque in ApoE-deficient mice.


Assuntos
Anticorpos Antifosfolipídeos/administração & dosagem , Apolipoproteínas E/deficiência , Aterosclerose/etiologia , beta 2-Glicoproteína I/antagonistas & inibidores , Animais , Síndrome Antifosfolipídica/etiologia , Aterosclerose/patologia , Autoimunidade , Dieta Hiperlipídica/efeitos adversos , Modelos Animais de Doenças , Imunoglobulina G/administração & dosagem , Macrófagos/patologia , Masculino , Camundongos , Camundongos Knockout para ApoE , Placa Aterosclerótica/etiologia , Placa Aterosclerótica/patologia
10.
Clin Dermatol ; 37(5): 528-547, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31896408

RESUMO

The color purple can be seen in several types of eruptions including inflammatory dermatoses like lichen planus, infectious dermatoses like ecthyma gangrenosum, neoplasms like Kaposi sarcoma, and vasculitis and vasculopathy. The current review focuses on the clinical appearance, pathophysiology, and treatment of several vasculitides and vasculopathies including capillaritis, cutaneous small-vessel vasculitis, immunoglobulin A (IgA) vasculitis, cryoglobulinemia, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, polyarteritis nodosum, warfarin-induced skin necrosis, heparin-induced thrombocytopenia, purpura fulminans, antiphospholipid antibody syndrome, calciphylaxis, levamisole-induced vasculopathy, and thrombotic thrombocytopenic purpura. Dermatologists play a central role in treating patients with cutaneous vasculitis and vasculopathy and may have the opportunity to facilitate identification of systemic disease by diagnosing cutaneous vasculitis and vasculopathy.


Assuntos
Pele/patologia , Vasculite/etiologia , Anticoagulantes/efeitos adversos , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/tratamento farmacológico , Síndrome Antifosfolipídica/etiologia , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Calciofilaxia/terapia , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/tratamento farmacológico , Síndrome de Churg-Strauss/etiologia , Cor , Crioglobulinemia/diagnóstico , Crioglobulinemia/tratamento farmacológico , Crioglobulinemia/etiologia , Diagnóstico Diferencial , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/etiologia , Granulomatose com Poliangiite/patologia , Humanos , Levamisol/efeitos adversos , Poliangiite Microscópica/diagnóstico , Poliangiite Microscópica/tratamento farmacológico , Poliangiite Microscópica/etiologia , Necrose/induzido quimicamente , Necrose/diagnóstico , Necrose/terapia , Poliarterite Nodosa/diagnóstico , Poliarterite Nodosa/tratamento farmacológico , Poliarterite Nodosa/etiologia , Púrpura Fulminante/diagnóstico , Púrpura Fulminante/etiologia , Púrpura Fulminante/terapia , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/etiologia , Púrpura Trombocitopênica Trombótica/terapia , Vasculite/diagnóstico , Vasculite/terapia , Varfarina/efeitos adversos
11.
Hamostaseologie ; 39(2): 188-194, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30419590

RESUMO

The antiphospholipid syndrome (APS) has occupied haemostaseologists, rheumatologists and obstetricians since its initial description 35 years ago. Its name has been coined because of the antibodies against phospholipids which were the common property of affected patients. In particular, the pathogenesis of APS has been intensively studied after the early discovery that it was possible to induce the clinical manifestations in animals by transfer of antiphospholipid antibodies (aPL). In recent years, it has become clear that aPL are not only structurally heterogeneous but also have different pathogenic properties. This review will focus on the relevance of antigenic specificity of aPL in terms of pathogenesis, diagnosis, and perhaps treatment of APS.


Assuntos
Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/terapia , Animais , Síndrome Antifosfolipídica/etiologia , Síndrome Antifosfolipídica/imunologia , Humanos
12.
Asian Pac J Allergy Immunol ; 37(3): 171-178, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29981567

RESUMO

OBJECTIVES: To describe the characteristics of patients with antiphospholipid syndrome (APS) in an Asian clinical practice setting. METHODS: We conducted a single-center, retrospective study of APS patients attending the rheumatology or hematology clinics, between January 2012 and December 2016. RESULTS: There were 450 patients suspected of having APS referred to our clinics. Seventy-four (16.4%) were diagnosed of APS, 51% of which were definite. Fifty-two (70%) patients were classified as primary APS, 50% of which were definite APS. The most common clinical manifestation was stroke (33%), followed by deep vein thrombosis of the lower extremities (30%) and pulmonary embolism (19%). Hypertension and the presence of at least one established cardiovascular risk factor were independently associated with stroke. Seven (9%) patients had multiorgan thrombosis as their first presentation of APS, 71% of which ultimately suffered from permanent organ damage or died of severe thrombosis, despite not fulfilling the criteria for 'definite' catastrophic APS (CAPS). Late fetal loss was the most prevalent obstetric complication. The majority of patients (79%) tested positive for lupus anticoagulant (LAC), while only 32% tested positive for anti-cardiolipin antibodies. Triple positive profile was documented in 14% of the cohort. Overall, recurrent thrombosis and bleeding complications were recorded in 9% and 28%, respectively. CONCLUSION: APS patients in central Thailand demonstrated high prevalence of stroke, late fetal loss, LAC positivity, and multiorgan thrombosis at first presentation, leading to poor outcomes.


Assuntos
Síndrome Antifosfolipídica/epidemiologia , Síndrome Antifosfolipídica/etiologia , Grupo com Ancestrais do Continente Asiático , Adulto , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Biomarcadores , Suscetibilidade a Doenças/imunologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fenótipo , Estudos Retrospectivos , Trombose/diagnóstico , Trombose/etiologia , Trombose/terapia , Resultado do Tratamento , Adulto Jovem
13.
Front Immunol ; 9: 2413, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30405613

RESUMO

APS is an autoimmune disease in which antiphospholipid antibodies (aPL) cause vascular thrombosis and pregnancy morbidity. In patients with APS, aPL exert pathogenic actions by binding serum beta-2-glycoprotein I (ß2GPI) via its N-terminal domain I (DI). We previously showed that bacterially-expressed recombinant DI inhibits biological actions of IgG derived from serum of patients with APS (APS-IgG). DI is too small (7 kDa) to be a viable therapeutic agent. Addition of polyethylene glycol (PEGylation) to small molecules enhances the serum half-life, reduces proteolytic targeting and can decrease immunogenicity. It is a common method of tailoring pharmacokinetic parameters and has been used in the production of many therapies in the clinic. However, PEGylation of molecules may reduce their biological activity, and the size of the PEG group can alter the balance between activity and half-life extension. Here we achieve production of site-specific PEGylation of recombinant DI (PEG-DI) and describe the activities in vitro and in vivo of three variants with different size PEG groups. All variants were able to inhibit APS-IgG from: binding to whole ß2GPI in ELISA, altering the clotting properties of human plasma and promoting thrombosis and tissue factor expression in mice. These findings provide an important step on the path to developing DI into a first-in-class therapeutic in APS.


Assuntos
Síndrome Antifosfolipídica/etiologia , Síndrome Antifosfolipídica/metabolismo , Coagulação Sanguínea , Imunoglobulina G/imunologia , Imunoglobulina G/metabolismo , Domínios e Motivos de Interação entre Proteínas , beta 2-Glicoproteína I/metabolismo , Adulto , Animais , Anticorpos Antifosfolipídeos/sangue , Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/diagnóstico , Modelos Animais de Doenças , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Masculino , Camundongos , Pessoa de Meia-Idade , Ligação Proteica , Domínios Proteicos , Trombose/sangue , Trombose/etiologia , Trombose/metabolismo , beta 2-Glicoproteína I/química
14.
BMJ Case Rep ; 20182018 Nov 12.
Artigo em Inglês | MEDLINE | ID: mdl-30420561

RESUMO

A 23-year-old woman with history of systemic lupus erythematous presented with dizziness and headache and was admitted for the stroke workup. During her stay, she had sudden painless loss of vision in her right eye consistent with central retinal artery occlusion (CRAO). Ocular massage and paracentesis were attempted without success to resume the flow. She was started on oral high-dose steroids (1 mg/kg) for lupus flare and therapeutic anticoagulation for antiphospholipid syndrome (positive for anticardiolipin and beta-2 microglobulin antibodies). On day 4, she started having painful bluish discoloration of her left index finger and right fifth toe, and on day 5 she had acute onset of left blurry vision with findings consistent with CRAO. She fulfilled the criteria of catastrophic antiphospholipid syndrome and was started on intravenous pulse steroids, plasmapheresis and higher international normalised ratio goal of 3-3.5 with improvement in her left eye vision from 20/200 to 20/20 on near card test by the end of treatment.


Assuntos
Síndrome Antifosfolipídica/etiologia , Lúpus Eritematoso Sistêmico/complicações , Oclusão da Artéria Retiniana/etiologia , Corticosteroides/uso terapêutico , Adulto , Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/terapia , Diagnóstico Diferencial , Feminino , Humanos , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Plasmaferese , Oclusão da Artéria Retiniana/diagnóstico , Oclusão da Artéria Retiniana/terapia , Transtornos da Visão , Adulto Jovem
15.
Expert Rev Clin Immunol ; 14(10): 803-816, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30173578

RESUMO

INTRODUCTION: Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder with systemic manifestations and multiorgan involvement. Although primarily diagnosed, and managed in the outpatient setting, it can occasionally present with life-threatening complications that require rapid assessment and urgent aggressive therapy. Areas covered: In our review, we explore three organ systems that are often affected in SLE, but have the potential to present as medical emergencies; these are the kidney, the central nervous system, and the hematologic system. We take a case-based approach to each clinical scenario, with information given sequentially in order to reflect "real-life" situations where management decisions need to be made with limited information. We review the acute management, pathophysiology, diagnostic approach, and treatment along with a review of the literature, for lupus nephritis presenting as rapidly progressive glomerulonephritis, acute lupus transverse myelitis, and refractory antiphospholipid syndrome. Expert commentary: At the conclusion of each section, we provide an expert commentary regarding each issue, relating to diagnosis, early management, and current evidence behind treatment recommendations.


Assuntos
Síndrome Antifosfolipídica , Lúpus Eritematoso Sistêmico/complicações , Nefrite Lúpica , Vasculite Associada ao Lúpus do Sistema Nervoso Central , Adulto , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/etiologia , Síndrome Antifosfolipídica/terapia , Emergências , Feminino , Humanos , Nefrite Lúpica/diagnóstico , Nefrite Lúpica/terapia , Vasculite Associada ao Lúpus do Sistema Nervoso Central/diagnóstico , Vasculite Associada ao Lúpus do Sistema Nervoso Central/terapia
16.
Adv Respir Med ; 86(3)2018.
Artigo em Inglês | MEDLINE | ID: mdl-29960279

RESUMO

Pulmonary embolism is the most common pulmonary manifestation of primary antiphospholipid syndrome (PAPS). However, PAPS may manifest in the respiratory system also due to non-thrombotic processes. In the following paper we present a case of PAPS-related diffuse alveolar hemorrhage (DAH). Because of sparse literature and a lack of randomized controlled trials, there are currently no recommendations regarding the optimal choice of steroid-sparing agent in treating PAPS-related DAH. In our patient, treatment with cyclophosphamide or mycophenolate mofetil along with low dose prednisone was ineffective, partially because of infectious complications, whereas addition of monthly intravenous immunoglobulin to mycophenolate mofetil and prednisone, appears to control the disease.


Assuntos
Síndrome Antifosfolipídica/complicações , Hemorragia/etiologia , Embolia Pulmonar/complicações , Idoso , Síndrome Antifosfolipídica/etiologia , Humanos , Masculino
17.
Ann Rheum Dis ; 77(11): 1549-1557, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30045853

RESUMO

Systemic lupus erythematosus (SLE), a complex and heterogeneous autoimmune disease, represents a significant challenge for both diagnosis and treatment. Patients with SLE in Latin America face special problems that should be considered when therapeutic guidelines are developed. The objective of the study is to develop clinical practice guidelines for Latin American patients with lupus. Two independent teams (rheumatologists with experience in lupus management and methodologists) had an initial meeting in Panama City, Panama, in April 2016. They selected a list of questions for the clinical problems most commonly seen in Latin American patients with SLE. These were addressed with the best available evidence and summarised in a standardised format following the Grading of Recommendations Assessment, Development and Evaluation approach. All preliminary findings were discussed in a second face-to-face meeting in Washington, DC, in November 2016. As a result, nine organ/system sections are presented with the main findings; an 'overarching' treatment approach was added. Special emphasis was made on regional implementation issues. Best pharmacologic options were examined for musculoskeletal, mucocutaneous, kidney, cardiac, pulmonary, neuropsychiatric, haematological manifestations and the antiphospholipid syndrome. The roles of main therapeutic options (ie, glucocorticoids, antimalarials, immunosuppressant agents, therapeutic plasma exchange, belimumab, rituximab, abatacept, low-dose aspirin and anticoagulants) were summarised in each section. In all cases, benefits and harms, certainty of the evidence, values and preferences, feasibility, acceptability and equity issues were considered to produce a recommendation with special focus on ethnic and socioeconomic aspects. Guidelines for Latin American patients with lupus have been developed and could be used in similar settings.


Assuntos
Síndrome Antifosfolipídica/tratamento farmacológico , Doenças Hematológicas/tratamento farmacológico , Nefropatias/tratamento farmacológico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Síndrome Antifosfolipídica/etiologia , Cardiopatias/tratamento farmacológico , Cardiopatias/etiologia , Doenças Hematológicas/etiologia , Humanos , Nefropatias/etiologia , América Latina , Pneumopatias/tratamento farmacológico , Pneumopatias/etiologia , Lúpus Eritematoso Sistêmico/complicações , Nefrite Lúpica/tratamento farmacológico , Nefrite Lúpica/etiologia , Transtornos Mentais/tratamento farmacológico , Transtornos Mentais/etiologia , Doenças Musculoesqueléticas/tratamento farmacológico , Doenças Musculoesqueléticas/etiologia , Dermatopatias/tratamento farmacológico , Dermatopatias/etiologia , Padrão de Cuidado
18.
Exp Mol Pathol ; 104(2): 151-154, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29551574

RESUMO

Amyloidosis is a disorder characterized by the deposition of insoluble abnormal proteins in the extracellular space. It may occur as a localized lesion or as a systemic disease involving multiple organs and systems. Localized conjunctival amyloidosis is rare and is less frequently associated with systemic involvement. Although amyloidosis itself is a benign lesion involvement of multiple organs and systems is associated with poor prognosis. Diagnosis of amyloidosis is made on biopsy specimens with Congo red staining for the appearance of apple-green birefringence under polarized light microscopy. Liquid chromatography tandem-mass spectrometry (LC-MS/MS) is much more sensitive in diagnosing amyloidosis and can determine the type of amyloid deposit. Here we reported a case of conjunctival amyloidosis in a 52 year-old male patient who was presented with left lower eyelid swelling to our medical center. He has a complicated past medical history of anti-phospholipid antibody syndrome, Buerger's disease (thromboangitis obliterans), and small cell lymphoma (SLL) of the right orbit/eyelid. The patient received radiation to the right orbit to treat SLL with therapy completed one and a half years prior to presentation. Physical examination revealed a firm, raised yellowish colored lesion in the left lower conjunctiva. The conjunctival lesion was biopsied, and tissue sections were examined with Congo red stains and LC-MS/MS analysis. The biopsy showed amyloid deposits without evidence of malignancy, and the type of proteins in the deposit was immunoglobulin light chain (AL) of kappa type. A complete work up was taken for possible systemic involvement of amyloidosis and results were all negative. To our knowledge, this is the first case of localized conjunctival amyloidosis with a history of contralateral orbit/eyelid SLL.


Assuntos
Amiloidose/patologia , Doenças da Túnica Conjuntiva/patologia , Linfoma/patologia , Neoplasias Orbitárias/patologia , Síndrome Antifosfolipídica/etiologia , Biópsia , Humanos , Linfoma/radioterapia , Linfoma não Hodgkin/radioterapia , Masculino , Pessoa de Meia-Idade , Órbita/patologia , Neoplasias Orbitárias/radioterapia , Tromboangiite Obliterante/etiologia
19.
Vnitr Lek ; 64(2): 136-145, 2018.
Artigo em Tcheco | MEDLINE | ID: mdl-29595273

RESUMO

The clinical picture of systemic lupus and antiphospholipid syndrome is remarkably varied and disease manifestations are commonly very heterogeneous. Relatively often both diseases are associated with severe, acute and life threatening manifestations, which places demands on the knowledge of differential diagnostics and experience of the physicians. This article deals with the serious and mostly acute impairment of cardiovascular, respiratory, renal, gastrointestinal, hematopoietic or nervous systems, briefly discusses the acute pregnancy complication and summarizes the basic therapeutic option. It emphasizes the role of both, sometimes inseparable, diseases in differential diagnosis of acute symptoms in internal medicine.Key words: clinical symptoms - diagnostics - live threatening manifestations - lupus erythematosus - systemic antiphospholipid syndrome - therapy.


Assuntos
Síndrome Antifosfolipídica , Lúpus Eritematoso Sistêmico , Síndrome Antifosfolipídica/etiologia , Diagnóstico Diferencial , Feminino , Humanos , Medicina Interna , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Gravidez , Complicações na Gravidez/diagnóstico
20.
Neurocrit Care ; 28(1): 127-132, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-28357636

RESUMO

BACKGROUND: Catastrophic antiphospholipid syndrome (CAPS) is a rare, severe variant of antiphospholipid syndrome with a high mortality rate. We report a unique case of CAPS secondary to Epstein-Barr viral (EBV) infection complicated by pulmonary and intracerebral hemorrhage. A review of the CAPS literature relevant to intensive care practice is used to outline a rational approach to diagnosis and management. METHODS: All data are from a single patient admitted to the Neurosciences Critical Care Unit in Addenbrooke's Hospital, Cambridge, in March 2016. Medline, Web of Science, PubMed, and the Cochrane Library were searched through September 2016 without restrictions for cases of CAPS, management of CAPS in the intensive care unit, and hemorrhage complicating CAPS. The patient gave express written consent to access and publish these data. RESULTS: This is only the second reported case of probable CAPS secondary to EBV infection. Furthermore, pulmonary and intracerebral hemorrhage is rare manifestations of this multisystem prothrombotic state which provided unique challenges to the management. CONCLUSIONS: While rare, CAPS should be considered in any patient presenting with rapidly progressive multiorgan failure, evidence of thrombotic microangiopathy, and antiphospholipid antibodies. A high index of suspicion is required as early, aggressive, multimodal treatment with anticoagulation, and immunosuppression improves outcomes.


Assuntos
Síndrome Antifosfolipídica/etiologia , Hemorragia Cerebral/etiologia , Infecções por Vírus Epstein-Barr/complicações , Adulto , Humanos , Masculino , Adulto Jovem
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