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1.
Medicine (Baltimore) ; 99(5): e19036, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32000451

RESUMO

RATIONALE: Monoclonal gammopathy of undetermined significance (MGUS) is a plasma cell proliferative disorder that consistently precedes multiple myeloma. Peripheral neuropathy in patients with IgG-MGUS tends to vary in clinical phenotype. We report a rare case of a patient with IgG-MGUS who had nonsystemic vasculitic neuropathy (NSVN). PATIENT CONCERNS: A 56-year-old Japanese woman presented with progressive sensory ataxia with episodic paresthesia. Her clinical and laboratory values were compatible with IgG-MGUS. A nerve conduction study suggested possible chronic inflammatory demyelinating polyneuropathy. However, intravenous immunoglobulin therapy was not effective. A sural nerve biopsy specimen revealed mildly reduced myelinated fiber density and myelin ovoid formation, with epineural arterioles infiltrated by inflammatory cells. DIAGNOSES: We accordingly diagnosed her condition as NSVN. INTERVENTIONS: She was accordingly started on oral prednisolone (40 mg/d) at 3 months after the onset of her neurological symptoms. OUTCOMES: At 1 year after the oral prednisolone treatment was begun, the patient's neurological symptoms showed no worsening. LESSONS: These findings indicate NSVN as a possible cause of peripheral neuropathy in patients with IgG-MGUS. Cumulatively, our findings highlight the need for a nerve biopsy for peripheral neuropathy in patients with IgG-MGUS as a possible cause of NSVN. The early diagnosis of NSVN is expected to be beneficial for such patients.


Assuntos
Gamopatia Monoclonal de Significância Indeterminada/diagnóstico , Doenças do Sistema Nervoso Periférico/diagnóstico , Vasculite/diagnóstico , Idoso , Biópsia , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imunoglobulina G , Gamopatia Monoclonal de Significância Indeterminada/tratamento farmacológico , Doenças do Sistema Nervoso Periférico/tratamento farmacológico , Prednisolona/uso terapêutico , Vasculite/tratamento farmacológico
2.
Postgrad Med ; 131(7): 546-549, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31483199

RESUMO

Objectives: To identify the cognitive and functional deficits in a well-characterized group of patients with vasculitis of the nervous system. Methods: Sixty-seven patients diagnosed with Central Nervous System (CNS) or Peripheral nervous System (PNS) vasculitis over a 14-year period were retrospectively identified. Data on clinical presentation, laboratory, radiographic and tissue biopsy investigations, and treatment were collated. Cognitive, functional and quality of life evaluation assessments were performed in 31 patients who agreed to participate and included Addenbrooke's Cognitive Examination-revised (ACE-R), Nottingham Extended Activities of Daily Living (NEADL) and EQ-5D-3L quality of life questionnaires. Results: CNS vasculitis patients exhibited cognitive impairment, with a mean ACE-R score of 74/100 (standard deviation (SD) 16). NEADL and EQ-5D-3L scores were in the impaired range at 41/66 (SD 21) and 57/81 (SD 22), respectively. Patients with just PNS vasculitis exhibited fewer cognitive deficits with ACE-R and NEADL scores of 87 (SD 8) and 46 (SD 16) respectively. EQ-5D-3L score was in the impaired range of 65 (SD 22). Conclusions: Vasculitis of the nervous system and, in particular, CNS vasculitis causes cognitive impairment and deficits in functional ability. Such patients should be targeted for cognitive rehabilitation.


Assuntos
Disfunção Cognitiva/psicologia , Doenças do Sistema Nervoso Periférico/psicologia , Vasculite do Sistema Nervoso Central/psicologia , Atividades Cotidianas , Adulto , Idoso , Idoso de 80 Anos ou mais , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/fisiopatologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/psicologia , Disfunção Cognitiva/etiologia , Disfunção Cognitiva/fisiopatologia , Estudos de Coortes , Estudos Transversais , Feminino , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/tratamento farmacológico , Arterite de Células Gigantes/fisiopatologia , Arterite de Células Gigantes/psicologia , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/fisiopatologia , Granulomatose com Poliangiite/psicologia , Nível de Saúde , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso Periférico/complicações , Doenças do Sistema Nervoso Periférico/tratamento farmacológico , Doenças do Sistema Nervoso Periférico/fisiopatologia , Qualidade de Vida , Estudos Retrospectivos , Vasculite/complicações , Vasculite/tratamento farmacológico , Vasculite/fisiopatologia , Vasculite/psicologia , Vasculite do Sistema Nervoso Central/complicações , Vasculite do Sistema Nervoso Central/tratamento farmacológico , Vasculite do Sistema Nervoso Central/fisiopatologia , Adulto Jovem
3.
Actas dermo-sifiliogr. (Ed. impr.) ; 110(7): 590-596, sept. 2019. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-185530

RESUMO

La vasculitis eosinofílica recurrente cutánea primaria es una enfermedad rara, caracterizada por placas purpúricas, eritematosas y pruriginosas asociadas a edema. Comparamos y analizamos las características clínicas, dermatoscópicas e histológicas de la enfermedad en 4 pacientes de nuestro hospital y en 13 casos de pacientes publicados previamente. Se incluyeron 17 pacientes, con una mediana de edad de 56 años, y una mediana de duración de la enfermedad de 6 meses. En la mayoría de los casos las lesiones fueron generalizadas (47%) o localizadas en los miembros inferiores (41%). Las características histológicas más frecuentes fueron: color purpúrico (71%), edema local (65%), vasculitis necrosante (94%) e infiltración eosinofílica (100%). La mayoría de los pacientes (82,4%) recibió esteroides orales (mediana de la dosis de 30 mg). La vasculitis eosinofílica recurrente cutánea primaria podría ser una entidad clínica infradiagnosticada. El análisis de los pacientes nos permitió proponer algunos criterios diagnósticos para su definición. Recomendamos una estrategia terapéutica con esteroides de alta potencia y dapsona, pudiéndose considerar como el tratamiento de primera línea


Primary recurrent cutaneous eosinophilic vasculitis is a rare condition characterized by pruritic, erythematous, purpuric plaques associated to edema. We compared and analyze the clinical, dermoscopic and histological features of the disease in 4 patients from our hospital and 13 patients published in the literature. Seventeen patients were included, with a median age of 56 yo, a median duration of disease of 6 months. Lesions were most frequently generalized (47%) or localized in lower limbs (41%). The most frequent features were purpuric color (71%), local edema (65%), necrotizing vasculitis (94%) and eosinophil infiltration (100%) in histology. Most of the patients (82.4%) were given oral steroids with a median dose of 30 mg. Primary recurrent cutaneous eosinophilic vasculitis might be an infra-diagnosed condition. The analysis of the patients allowed us to propose diagnostic criteria for the definition of this disease. We suggest a therapeutic strategy with high-potency steroids and dapsone, which might be considered as first-line treatment


Assuntos
Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Vasculite/tratamento farmacológico , Dermatopatias Vasculares/tratamento farmacológico , Eosinofilia/complicações , Recidiva , Dermoscopia/instrumentação , Vasculite/diagnóstico , Dapsona/uso terapêutico , Corticosteroides/uso terapêutico , Dermatopatias Vasculares/fisiopatologia , Prednisona/uso terapêutico , Diagnóstico Diferencial
4.
Cochrane Database Syst Rev ; 8: CD012448, 2019 08 16.
Artigo em Inglês | MEDLINE | ID: mdl-31425625

RESUMO

BACKGROUND: Kawasaki disease (KD) is an acute inflammatory vasculitis (inflammation of the blood vessels) that mainly affects children between six months and five years of age. The vasculitis primarily impacts medium-sized blood vessels, especially in the coronary arteries. In most children, intravenous immunoglobulin (IVIG) and aspirin therapy rapidly reduce inflammatory markers, fever, and other clinical symptoms. However, approximately 15% to 20% of children receiving the initial IVIG infusion show persistent or recurrent fever and are classified as IVIG-resistant. Tumor necrosis factor-alpha (TNF-α) is an inflammatory cytokine that plays an important role in host defence against infections and in immune responses. Several studies have established that blocking TNF-α is critical for obtaining anti-inflammatory effects in children with KD, thus, there is a need to identify benefits and risks of TNF-α blockers for the treatment of KD. OBJECTIVES: To evaluate the efficacy and safety of using TNF-α blockers (i.e. infliximab and etanercept) to treat children with Kawasaki disease. SEARCH METHODS: The Cochrane Vascular Information Specialist searched the Cochrane Vascular Specialised Register, CENTRAL, MEDLINE, Embase and CINAHL databases, the World Health Organization International Clinical Trials Registry Platform and ClinicalTrials.gov trials register to 19 September 2018. We also undertook reference checking of grey literature. SELECTION CRITERIA: We included randomised controlled trials (RCTs) that compared TNF-α blockers (i.e. infliximab and etanercept) to placebo or other drugs (including retreatment with IVIG) in children with KD, reported in abstract or full-text. DATA COLLECTION AND ANALYSIS: Two review authors independently applied the study selection criteria, assessed risk of bias and extracted data. When necessary, we contacted study authors for additional information. We used GRADE to assess the certainty of the evidence. MAIN RESULTS: We included five trials from 14 reports, with a total of 494 participants. All included trials were individual RCTs that examined the effect of TNF-α blockers for KD.Five trials (with 494 participants) reported the incidence of treatment resistance. TNF-α blockers reduced the incidence of treatment resistance (TNF-α blocker intervention group 30/237, control group 58/257; risk ratio (RR) 0.57, 95% confidence interval (CI) 0.38 to 0.86; low-certainty evidence).Four trials reported the incidence of coronary artery abnormalities (CAAs). Three trials (with 270 participants) contributed data to the meta-analysis, since we could not get the data needed for the analysis from the fourth trial. There was no clear difference between groups in the incidence of CAAs (TNF-α blocker intervention group 8/125, control group 9/145; RR 1.18, 95% CI 0.45 to 3.12; low-certainty evidence).Three trials with 250 participants reported the adverse effect 'infusion reactions' after treatment initiation. The TNF-α blocker intervention decreased infusion reactions (TNF-α blocker intervention group 0/126, control group 15/124; RR 0.06, 95% CI 0.01 to 0.45; low-certainty evidence).Two trials with 227 participants reported the adverse effect 'infections' after treatment initiation. There was no clear difference between groups (TNF-α blocker intervention group 7/114, control group 10/113; RR 0.68, 95% CI 0.33 to 1.37; low-certainty evidence).One trial (with 31 participants) reported the adverse effect 'cutaneous reactions' (rash and contact dermatitis). There was no clear difference between the groups for incidence of rash (TNF-α blocker intervention group 2/16, control group 0/15; RR 4.71, 95% CI 0.24 to 90.69; very low-certainty evidence) or for incidence of contact dermatitis (TNF-α blocker intervention group 1/16, control group 3/15; RR 0.31, 95% CI 0.04 to 2.68; very low-certainty evidence).No trials reported other adverse effects such as injection site reactions, neutropenia, infections, demyelinating disease, heart failure, malignancy, and induction of autoimmunity. AUTHORS' CONCLUSIONS: We found a limited number of RCTs examining the effect of TNF-α blockers for KD. In summary, low-certainty evidence indicates that TNF-α blockers have beneficial effects on treatment resistance and the adverse effect 'infusion reaction' after treatment initiation for KD when compared with no treatment or additional treatment with IVIG. Further research will add to the evidence base. Due to the small number of underpowered trials contributing to the analyses, the results presented should be treated with caution. Further large high quality trials with timing and type of TNF-α blockers used are needed to determine the effects of TNF-α blockers for KD.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Pré-Escolar , Humanos , Fatores Imunológicos , Lactente , Ensaios Clínicos Controlados Aleatórios como Assunto , Vasculite/tratamento farmacológico
5.
Medicine (Baltimore) ; 98(28): e16382, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31305439

RESUMO

RATIONALE: Type I monoclonal cryoglobulinemia is usually associated with lymphoproliferative disorders, such as monoclonal gammopathy of undetermined significance (MGUS), myeloma, chronic lymphocytic leukemia (CLL) and lymphoplasmocytic lymphoma (LPL). Clinical symptoms related to Type I cryoglobulin (CG) isotype often include skin, neurological and renal manifestations. PATIENT CONCERNS: A 42-year-old woman who initially presented urticaria, palpable purpura in both her upper extremities and legs, eosinophilia and Raynaud phenomenon. Skin biopsy revealed eosinophil infiltration. Monoclonal immunoglobulin (Ig) G-κprotein was detected and CG was also positive. DIAGNOSES: The patient was finally diagnosed as MGUS related Type I CG. INTERVENTIONS: Cyclophosphamide-dexamethasone-thalidomide (CDT) therapy was initiated. OUTCOMES: The treatment relieved the skin symptoms efficiently. LESSONS: To our knowledge, this is a rare case of Type I cryoglobulinemic vasulitis with eosinophilia complicated by MGUS, and the effective treatment of cyclophosphamide combined with thalidomide and prednisone may provide a new therapeutic option for cryoglobulinemic vasulitis.


Assuntos
Crioglobulinemia/complicações , Eosinofilia/complicações , Gamopatia Monoclonal de Significância Indeterminada/complicações , Vasculite/complicações , Adulto , Crioglobulinemia/diagnóstico , Crioglobulinemia/tratamento farmacológico , Diagnóstico Diferencial , Eosinofilia/diagnóstico , Eosinofilia/tratamento farmacológico , Feminino , Humanos , Gamopatia Monoclonal de Significância Indeterminada/diagnóstico , Gamopatia Monoclonal de Significância Indeterminada/tratamento farmacológico , Vasculite/diagnóstico , Vasculite/tratamento farmacológico
6.
BMJ Case Rep ; 12(7)2019 Jul 16.
Artigo em Inglês | MEDLINE | ID: mdl-31315843

RESUMO

We report the case of a 56-year-old man who presented to the emergency department with a 3-day onset of left limb weakness and feeling intoxicated with poor balance. Stroke hospitalisations in the USA decreased from 2000 to 2010, however the number of hospitalised patients with ischaemic stroke and HIV infection has increased significantly. Herein, we discuss the management of this unique case to highlight the importance of a broad differential diagnosis when approaching HIV/AIDS patients presenting with acute or subacute neurological focalisation. Given that HIV vasculopathy is a diagnosis of exclusion, it requires a thoughtful elimination of all possible aetiologies.


Assuntos
Infarto Encefálico/diagnóstico por imagem , Infarto Cerebral/etiologia , Infecções por HIV/complicações , Vasculite/virologia , Administração Intravenosa , Antivirais/administração & dosagem , Antivirais/uso terapêutico , Infarto Encefálico/patologia , Infarto Cerebral/diagnóstico por imagem , Infarto Cerebral/terapia , Angiografia por Tomografia Computadorizada , Diagnóstico Diferencial , Infecções por HIV/imunologia , Herpesvirus Humano 3/genética , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Acidente Vascular Cerebral/etiologia , Resultado do Tratamento , Infecção pelo Vírus da Varicela-Zoster/líquido cefalorraquidiano , Vasculite/líquido cefalorraquidiano , Vasculite/diagnóstico , Vasculite/tratamento farmacológico
7.
Pediatrics ; 144(2)2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31320468

RESUMO

Behçet disease (BD) is a multisystemic autoinflammatory disorder characterized by recurrent mucocutaneous, ocular, musculoskeletal, gastrointestinal, central nervous system, and vascular manifestations. Pulmonary arterial involvement (PAI) of BD is probably the most severe form of vasculitis, at least in children. PAI has a high mortality, morbidity, and recurrence rate. There are limited data regarding treatment and outcomes of pediatric patients with BD with PAI. Herein, we report 2 pediatric patients with BD presented with hemoptysis and support our data with a systematic review. These patients were given immunosuppressive therapy, which covered pulse methylprednisolone followed by oral prednisolone, intravenous cyclophosphamide every 3 weeks for a total of 6 cycles, and interferon-α2a concomitantly. These are the first reported cases in the literature successfully treated with this treatment modality in a complication with 50% mortality. These patients have been followed up for a period of at least 4 years without any vascular recurrence. Pediatricians should be aware that patients with BD may not present with full diagnostic criteria. They should consider BD in a child with PAI to avoid diagnostic delay and start life-saving accurate immunosuppressive treatment.


Assuntos
Síndrome de Behçet/diagnóstico por imagem , Síndrome de Behçet/tratamento farmacológico , Imunossupressores/administração & dosagem , Pneumopatias/diagnóstico por imagem , Pneumopatias/tratamento farmacológico , Adolescente , Ciclofosfamida/administração & dosagem , Quimioterapia Combinada , Humanos , Masculino , Metilprednisolona/administração & dosagem , Vasculite/diagnóstico por imagem , Vasculite/tratamento farmacológico
8.
BMC Surg ; 19(1): 73, 2019 Jul 02.
Artigo em Inglês | MEDLINE | ID: mdl-31266494

RESUMO

BACKGROUND: Diagnosis and management of acute abdomen secondary to systematic lupus erythematosus (SLE) has always been a clinical challenge. CASE PRESENTATION: A 21-year-old lady, with BMI 17.7, presented to our department with acute abdomen. Laparoscopy was carried out to exclude surgical emergency when conservative regimen failed. The patient revealed a history of purpuric changes and lupus test was positive for SLE. CONCLUSION: Based on our experience, early laparoscopy to alleviate acute abdomen has shown to improve the prognosis of the patient.


Assuntos
Abdome Agudo/diagnóstico , Abdome Agudo/cirurgia , Ascite/etiologia , Lúpus Eritematoso Sistêmico/complicações , Mesentério/irrigação sanguínea , Vasculite/cirurgia , Abdome Agudo/tratamento farmacológico , Abdome Agudo/etiologia , Administração Intravenosa , Administração Oral , Antirreumáticos/administração & dosagem , Ascite/diagnóstico por imagem , Ascite/tratamento farmacológico , Ascite/cirurgia , Feminino , Glucocorticoides/administração & dosagem , Humanos , Hidroxicloroquina/administração & dosagem , Laparoscopia , Mesentério/diagnóstico por imagem , Metilprednisolona/administração & dosagem , Prognóstico , Vasculite/diagnóstico por imagem , Vasculite/tratamento farmacológico , Vasculite/etiologia , Adulto Jovem
10.
Orphanet J Rare Dis ; 14(1): 110, 2019 05 16.
Artigo em Inglês | MEDLINE | ID: mdl-31096996

RESUMO

Linear scleroderma "en coup de sabre" (LSES) variant is a cephalic subtype of localized scleroderma that can be associated with extracutaneous stigmata, such as epilepsy, dementia syndromes, as well as focal central nervous system neurologic deficits. While the pathophysiology of cutaneous linear scleroderma includes endothelial cell injury and up regulation of pro-fibrogenic pathways, the basis of LSES-associated neurologic complications is largely unknown. We report a patient with a history of LSES who developed intractable epilepsy and cognitive decline. Magnetic resonance imaging (MRI) of the brain exhibited numerous persistently enhancing brain lesions. Due to progressive neurologic deterioration over a period of 7 years, despite interventional therapy, a brain biopsy was performed. Neuropathologic analysis exhibited acute and chronic cortical ischemia associated with a small vessel lymphocytic vasculitis. Direct immunofluorescent studies showed C5b-9 and IgG deposition on endothelium while indirect immunofluorescent studies demonstrated reactivity of the patient's serum with the microvasculature of the patient's own brain tissue and generic human umbilical vein endothelial cells indicative of anti-endothelial cell antibodies. Therapy focusing on damaged endothelium was implemented. The interleukin-6 (IL-6) receptor inhibitor tocilizumab was used and the patient improved dramatically, likely reflecting the drug's effect on the replenishment of endothelial progenitor cells.


Assuntos
Esclerodermia Localizada/tratamento farmacológico , Esclerodermia Localizada/patologia , Adulto , Anticorpos Monoclonais Humanizados/uso terapêutico , Cognição/efeitos dos fármacos , Progressão da Doença , Epilepsia/diagnóstico por imagem , Epilepsia/tratamento farmacológico , Epilepsia/patologia , Feminino , Técnica Indireta de Fluorescência para Anticorpo , Humanos , Imuno-Histoquímica , Imagem por Ressonância Magnética , Poliendocrinopatias Autoimunes/diagnóstico por imagem , Poliendocrinopatias Autoimunes/tratamento farmacológico , Poliendocrinopatias Autoimunes/patologia , Receptores de Interleucina-6/antagonistas & inibidores , Esclerodermia Localizada/diagnóstico por imagem , Vasculite/diagnóstico por imagem , Vasculite/tratamento farmacológico , Vasculite/patologia
13.
Medicine (Baltimore) ; 98(17): e15392, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31027135

RESUMO

INTRODUCTION: The Revised International Chapel Hill Consensus Conference 2012 subdivides vasculitides based on combinations of features that separate different forms of vasculitis into definable categories. Hypereosinophilic vasculitis with sparing of the respiratory tract and renal system is a rare presentation that is yet to be described in the Revised International Chapel Hill Consensus Conference 2012 report that addresses nomenclature of vasculitides. This is a condition that involves a vascular injury due to either a primary eosinophilic vasculitis or an underlying connective tissue disease and it predisposes patients to a prothrombotic state. PATIENT CONCERNS: A 39-year-old patient presented with left hand digital ischemia, preceded by Raynaud phenomenon, and vasculitic rash. For 3 months, he was having digital ischemia affecting the left 2nd and 3rd digits in the form of pallor and gangrenous discoloration with a preceding history of a pinpoint pruritic rash affecting his lower limbs that extended to involve the trunk and upper limbs over a short period of time and responded to only a tapering dose of oral steroids. Examination revealed a delayed capillary refill in all left-hand digits and a weak left radial pulse but no bruit. The rest of his peripheral vascular examination was unremarkable. DIAGNOSIS: Investigations revealed an absolute eosinophilic count of 4.34 K/µL and erythrocyte sedimentation rate of 44 mm/h. A magnetic resonance angiogram showed a beaded appearance of the left ulnar artery distally and the radial artery branches in the left hand and subsequently was diagnosed with hypereosinophilic vasculitis. INTERVENTIONS: He was started on oral prednisone of 1 mg/kg daily orally tapering done as well as azathioprine for maintenance. OUTCOMES: Two weeks postdischarge, the patient was seen in the outpatient department where his ischemic symptoms improved, and his skin rash healed. Noticed improvement in his splinter hemorrhages was also detected. He continued to do well on 2 years follow-up CONCLUSION:: This case reflects the importance of frequent reevaluation for vasculitic diseases criteria and nomenclature. Hypereosinophilic vasculitis with absent respiratory and renal involvement is a rare presentation with scarce evidence to guide treatment.


Assuntos
Síndrome Hipereosinofílica/diagnóstico , Vasculite/diagnóstico , Adulto , Diagnóstico Diferencial , Humanos , Síndrome Hipereosinofílica/tratamento farmacológico , Masculino , Vasculite/tratamento farmacológico
14.
BMJ Case Rep ; 12(4)2019 Apr 08.
Artigo em Inglês | MEDLINE | ID: mdl-30967450

RESUMO

A 29-year-old female patient presented with chest pain, breathlessness and syncope on the background of constitutional symptoms, oral ulceration and a rash. Multiple investigations were performed, including a CT pulmonary angiogram (CTPA) that was initially felt to show imaging features consistent with a diagnosis of chronic thromboembolic disease (CTED). The patient was referred to a tertiary pulmonary hypertension centre and the possibility of pulmonary vasculitis was raised. Subsequent positron emission tomography (PET)-CT revealed imaging features supporting this diagnosis. The patient was treated with intravenous cyclophosphamide infusions, following which her symptoms improved. A repeat PET-CT 6 months after treatment showed resolution in pulmonary artery and mediastinal uptake, but persistence of pulmonary artery occlusions on a repeat CTPA. A final diagnosis of pulmonary vasculitis secondary to Behçet's disease was made. This case report aims to raise awareness of the imaging features of CTED and its mimics.


Assuntos
Hipertensão Pulmonar/diagnóstico , Artéria Pulmonar/diagnóstico por imagem , Vasculite/diagnóstico , Administração Intravenosa , Adulto , Antirreumáticos/administração & dosagem , Síndrome de Behçet/complicações , Ciclofosfamida/administração & dosagem , Diagnóstico Diferencial , Ecocardiografia , Feminino , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/tratamento farmacológico , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Artéria Pulmonar/fisiopatologia , Tromboembolia/complicações , Tromboembolia/diagnóstico , Vasculite/complicações , Vasculite/tratamento farmacológico
16.
Mult Scler Relat Disord ; 30: 244-246, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30849680

RESUMO

BACKGROUND: Multiple Sclerosis (MS) has been associated with several immune-mediated diseases but the mechanisms that explain such associations, as well as their implications in clinical practice and treatment are rarely discussed. CASE PRESENTATION: We report the case of a patient with a history of MS since she was 27 years old, followed by a diagnosis of Hypocomplementemic Urticarial Vasculitis (HUV) seven years later. Several disease-modifying treatments for MS and HUV were used but with limited benefit in both diseases and significant MS progression. Activity of both diseases was later stabilized with Rituximab. We discuss the hypotheses of a central nervous system involvement in urticarial vasculitis or an association between MS and HUV, and examine the challenges in their management. CONCLUSIONS: In the presence of concurrent immune-mediated diseases, the diagnosis of MS can be challenging. This clinical presentation posed significant difficulties in disease management, influencing therapeutic options and their effectiveness/adverse effects profile. The best approach in MS patients with concurrent autoimmune diseases remains to be established and more reports are needed to help clarify this subject.


Assuntos
Esclerose Múltipla/complicações , Urticária/patologia , Vasculite/complicações , Adulto , Feminino , Humanos , Fatores Imunológicos/uso terapêutico , Esclerose Múltipla/tratamento farmacológico , Rituximab/uso terapêutico , Vasculite/tratamento farmacológico
17.
Redox Biol ; 22: 101143, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30897521

RESUMO

First described as essential to the phagocytic activity of leukocytes, Nox2-derived ROS have emerged as mediators of a range of cellular and tissue responses across species from salubrious to deleterious consequences. Knowledge of their role in inflammation is limited, however. We postulated that TNFα-induced endothelial reactive oxygen species (ROS) generation and pro-inflammatory signaling would be ameliorated by targeting Nox2. Herein, we in silico-modelled two first-in-class Nox2 inhibitors developed in our laboratory, explored their cellular mechanism of action and tested their efficacy in in vitro and mouse in vivo models of inflammation. Our data show that these inhibitors (CPP11G and CPP11H) disrupted canonical Nox2 organizing factor, p47phox, translocation to Nox2 in the plasma membrane; and abolished ROS production, markedly attenuated stress-responsive MAPK signaling and downstream AP-1 and NFκB nuclear translocation in human cells. Consequently, cell adhesion molecule expression and monocyte adherence were significantly inhibited by both inhibitors. In vivo, TNFα-induced ROS and inflammation were ameliorated by targeted Nox2 inhibition, which, in turn, improved hind-limb blood flow. These studies identify a proximal role for Nox2 in propagated inflammatory signaling and support therapeutic value of Nox2 inhibitors in inflammatory disease.


Assuntos
Células Endoteliais/efeitos dos fármacos , Células Endoteliais/metabolismo , Inibidores Enzimáticos/farmacologia , Membro Posterior/irrigação sanguínea , Membro Posterior/metabolismo , NADPH Oxidase 2/antagonistas & inibidores , Fluxo Sanguíneo Regional/efeitos dos fármacos , Vasculite/metabolismo , Animais , Biomarcadores , Adesão Celular , Linhagem Celular , Inibidores Enzimáticos/química , Humanos , Camundongos , Modelos Moleculares , Conformação Molecular , Monócitos/efeitos dos fármacos , Monócitos/metabolismo , NADPH Oxidase 2/química , NADPH Oxidase 2/metabolismo , NF-kappa B/metabolismo , Ligação Proteica , Domínios e Motivos de Interação entre Proteínas , Espécies Reativas de Oxigênio/metabolismo , Transdução de Sinais , Relação Estrutura-Atividade , Fator de Transcrição AP-1/metabolismo , Vasculite/tratamento farmacológico , Vasculite/etiologia , Vasculite/patologia
20.
BMJ Case Rep ; 12(1)2019 Jan 29.
Artigo em Inglês | MEDLINE | ID: mdl-30700467

RESUMO

Anticoagulants have recently been recognised as a cause of acute kidney injury (AKI). We describe the case of a 75-year-old man with IgA vasculitis and atrial fibrillation treated with rivaroxaban, who presented with macroscopic haematuria and an acute decline in renal function. Two months before referral, he noted palpable purpuric lesions and was diagnosed with IgA vasculitis based on skin biopsy findings; the skin lesion disappeared following treatment with a steroid external preparation. Renal biopsy revealed glomerular haemorrhage and red blood cell casts. Although rivaroxaban was withdrawn, his kidney function worsened and he was started on haemodialysis. His renal function did not recover. To the best of our knowledge, this is the first case of direct oral anticoagulant (DOAC)-related AKI in systemic vasculitis. During DOAC therapy, close monitoring of a patient's urinalysis results and their renal function may be required for patients with systemic vasculitis to avoid AKI.


Assuntos
Lesão Renal Aguda/induzido quimicamente , Inibidores do Fator Xa/efeitos adversos , Rivaroxabana/efeitos adversos , Vasculite/tratamento farmacológico , Lesão Renal Aguda/terapia , Idoso , Diagnóstico Diferencial , Humanos , Imunoglobulina A , Masculino , Diálise Renal
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