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3.
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
4.
BMJ Case Rep ; 12(8)2019 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-31420437

RESUMO

A 60-year-old woman was admitted to the hospital with worsening dyspnoea, cough and chest pain. This was on a background of weight loss, decreased appetite, mononeuritis multiplex, chronic eosinophilia and a single episode of a non-blanching rash. Investigations demonstrated a raised troponin and ischaemic changes on ECG, and she was therefore initially treated for a presumed myocardial infarction. However, her symptoms failed to improve with treatment for the acute coronary syndrome. A coronary angiogram revealed no significant flow-limiting disease, and further investigations yielded confirmation of raised eosinophils and a positive perinuclear antineutrophil cytoplasmic antibody test. An echocardiogram demonstrated a pericardial effusion, and subsequent cardiac magnetic resonance features were compatible with myopericarditis. In light of these findings, the patient was diagnosed with eosinophilic granulomatous with polyangiitis and commenced on high-dose intravenous methylprednisolone and cyclophosphamide. She made an excellent recovery and remains in remission on azathioprine and a tapering dose of corticosteroids.


Assuntos
Granuloma Eosinófilo/complicações , Granulomatose com Poliangiite/complicações , Miocardite/etiologia , Pericardite/etiologia , Anti-Inflamatórios/administração & dosagem , Ciclofosfamida/administração & dosagem , Granuloma Eosinófilo/tratamento farmacológico , Feminino , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Imunossupressores/administração & dosagem , Metilprednisolona/administração & dosagem , Pessoa de Meia-Idade , Miocardite/tratamento farmacológico , Pericardite/tratamento farmacológico
5.
BMJ Case Rep ; 12(8)2019 Aug 10.
Artigo em Inglês | MEDLINE | ID: mdl-31401575

RESUMO

Intestinal perforation is a rare and life-threatening complication in granulomatosis with polyangiitis (GPA). A 55-year-old Japanese man who had been undergoing maintenance haemodialysis was diagnosed with GPA via a pathologically proven nasal granuloma and proteinase 3-antineutrophil cytoplasmic antibody-positive status. He was started on 60 mg prednisolone. Haematochezia was observed due to the colon ulcer after treatment initiation. Two doses of intravenous cyclophosphamide were administered every 2 weeks. Despite the treatment, there were two episodes of severe abdominal pain with peritonitis within a week. Perforation of descending colon and severe ischaemia of the ascending colon was observed during each emergency laparotomy, and ileostomy was performed. Vasculitis in the small vessels was confirmed. In conclusion, patient with severe intestinal involvement and who was unresponsive to conventional therapy was treated with surgery followed by rituximab administration, and remission was achieved.


Assuntos
Granulomatose com Poliangiite/tratamento farmacológico , Fatores Imunológicos/uso terapêutico , Perfuração Intestinal/etiologia , Rituximab/uso terapêutico , Colo Descendente/cirurgia , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/diagnóstico , Humanos , Perfuração Intestinal/cirurgia , Laparotomia , Masculino , Pessoa de Meia-Idade , Indução de Remissão
6.
BMC Pulm Med ; 19(1): 154, 2019 Aug 19.
Artigo em Inglês | MEDLINE | ID: mdl-31426769

RESUMO

BACKGROUND: Atypical manifestations, such as elevated serum immunoglobulin-G4 (IgG4) and extra-pulmonary IgG4 positive plasmacyte infiltration, have been described in patients with eosinophilic granulomatosis with polyangiitis (EGPA), such complicated situation might not be readily differentiated from IgG4-related disease. CASE PRESENTATION: Here, we report an interesting and rare case of EGPA in a 41 year-old male with negative anti-neutrophil cytoplasmic antibodies (ANCAs), which showed abundant pulmonary IgG4-positive plasma cells infiltration and markedly elevated serum IgG4 levels without extra-pulmonary lesions of IgG4-related disease. The clinical characteristics hesitated us whether the diagnosis as EGPA overlapping with IgG4-related lung disease should be concluded because of the absence of storiform fibrosis and obliterative phlebitis with lymphoplasmacytic infiltration. The patient's systemic symptoms, pulmonary lesions, blood eosinophils count / percentage, and serum IgG4 levels were significantly improved with immunosuppressive therapy. CONCLUSIONS: We consider that the overlapping pathogenesis exists in the disease course of EGPA and IgG4-related disease, which may lead to interaction during the course of the diseases. And the utilization of diagnostic criteria for IgG4-related lung disease with the careful differentiation is needed in such cases.


Assuntos
Doenças Autoimunes/complicações , Eosinófilos/patologia , Granulomatose com Poliangiite/complicações , Imunoglobulina G/sangue , Pulmão/patologia , Adulto , Diagnóstico Diferencial , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Pulmão/diagnóstico por imagem , Masculino , Metilprednisolona/administração & dosagem , Sinusite/complicações , Tomografia Computadorizada por Raios X
7.
BMJ Case Rep ; 12(7)2019 Jul 08.
Artigo em Inglês | MEDLINE | ID: mdl-31289155

RESUMO

A 22-year-old female patient was admitted to hospital after being referred from the oral medicine clinic where she had been seen for persistent gingivitis and mouth ulcers. She described an insidious history of persistent fevers, dry cough and unexplained weight loss over 4-6 weeks. Imaging showed extensive bilateral pulmonary nodules with mediastinal lymphadenopathy and two lesions in the pancreas. MRI revealed these lesions to be well-defined fluid-filled cysts in the tail of the pancreas, without features of malignancy. Core biopsies taken from her lung nodules demonstrated features of vasculitis with granulomata. This was consistent with her positive immunology for c-antinuclear cytoplasmic antibodies and proteinase-3, which were sent after her fever failed to settle with antibiotic treatment. In keeping with a diagnosis of vasculitis, the patient showed a significant clinical and biochemical response to intravenous methylprednisolone and high-dose daily prednisolone thereafter.


Assuntos
Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/patologia , Linfadenopatia/patologia , Administração Intravenosa , Anticorpos Anticitoplasma de Neutrófilos/sangue , Diagnóstico Diferencial , Feminino , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Granulomatose com Poliangiite/diagnóstico por imagem , Granulomatose com Poliangiite/metabolismo , Humanos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Neoplasias do Mediastino/diagnóstico por imagem , Neoplasias do Mediastino/patologia , Metilprednisolona/administração & dosagem , Metilprednisolona/uso terapêutico , Mieloblastina/sangue , Pâncreas/diagnóstico por imagem , Pâncreas/patologia , Resultado do Tratamento , Adulto Jovem
8.
PLoS One ; 14(7): e0218705, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31291263

RESUMO

AIMS: Cytomegalovirus (CMV) infection under immunosuppression sometimes causes death. This study aimed to elucidate risk factors for CMV infection in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). METHODS: Patients with AAV who underwent remission induction treatment at Okayama University Hospital between 2006 and 2016 were retrospectively analyzed. The primary outcome was the development of CMV infection within 3 months. RESULTS: Of the 111 patients, 13 (11.7%) patients developed CMV infection. Patients with CMV infection were older (p = 0.030) and had a higher body mass index (p = 0.029) in comparison to those without CMV infection. A higher proportion had a severe form (p = 0.001) and granulomatosis with polyangiitis (GPA) (p = 0.001), as well as a higher Birmingham Vasculitis Activity Score (p = 0.018) and C-reactive protein (p = 0.018) levels at baseline. Using logistic regression analysis, severe form and GPA were independent risk factors (odds ratio [OR] = 9.68, 95% confidence interval [CI] = 1.92-60.23, and OR = 7.46, 95% CI = 1.46-47.60, respectively). In addition, patients with CMV infection were more likely than those without infection to be glucocorticoid-related diabetes mellitus (p = 0.025). CONCLUSION: Our study highlights disease severity and subgroups of AAV as risk factors for CMV infection.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Anticorpos Anticitoplasma de Neutrófilos/sangue , Infecções por Citomegalovirus/imunologia , Citomegalovirus/imunologia , Granulomatose com Poliangiite/imunologia , Infecções Oportunistas/imunologia , Idoso , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Índice de Massa Corporal , Proteína C-Reativa/metabolismo , Citomegalovirus/isolamento & purificação , Citomegalovirus/patogenicidade , Infecções por Citomegalovirus/diagnóstico , Infecções por Citomegalovirus/etiologia , Infecções por Citomegalovirus/virologia , Feminino , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/patologia , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/efeitos adversos , Japão , Modelos Logísticos , Masculino , Metilprednisolona/administração & dosagem , Metilprednisolona/efeitos adversos , Pessoa de Meia-Idade , Infecções Oportunistas/diagnóstico , Infecções Oportunistas/etiologia , Infecções Oportunistas/virologia , Indução de Remissão , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença
9.
Intern Med ; 58(21): 3129-3132, 2019 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-31292396

RESUMO

The safety and efficacy of anti-programmed cell death-1 (PD-1) antibodies in patients with granulomatosis with polyangiitis (GPA) still remain unclear. An 83-year-old man with GPA that was well controlled with immunosuppressive therapy was diagnosed with a postoperative recurrence of non-small cell lung cancer (NSCLC). Because the programmed cell death ligand 1 (PD-L1) tumor proportion score was 90%, pembrolizumab was administered. After 10 cycles, immune-related adverse events or GPA flare was not observed, and the patient showed an antitumor response. Anti-PD-1 antibody should therefore be considered a treatment option for PD-L1-high-expressing NSCLC patients with well-controlled GPA.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Antígeno B7-H1/antagonistas & inibidores , Carcinoma Pulmonar de Células não Pequenas/tratamento farmacológico , Granulomatose com Poliangiite/complicações , Neoplasias Pulmonares/tratamento farmacológico , Idoso , Carcinoma Pulmonar de Células não Pequenas/complicações , Carcinoma Pulmonar de Células não Pequenas/diagnóstico por imagem , Carcinoma Pulmonar de Células não Pequenas/secundário , Glucocorticoides/uso terapêutico , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/patologia , Masculino , Recidiva Local de Neoplasia/tratamento farmacológico , Tomografia por Emissão de Pósitrons , Prednisolona/uso terapêutico , Parede Torácica/diagnóstico por imagem , Tomografia Computadorizada por Raios X
10.
Medicina (Kaunas) ; 55(7)2019 Jul 16.
Artigo em Inglês | MEDLINE | ID: mdl-31315254

RESUMO

Granulomatosis with polyangiitis (GPA) is a systemic granulomatous inflammatory disease characterized by small-to-medium vessel vasculitis due to Central Anti-Neutrophil Cytoplasmic Antibody (C-ANCA). GPA commonly involves the lungs and the kidneys. Among the pulmonary manifestations, diffuse alveolar hemorrhage (DHA) is a rare presentation of GPA that can present with hemoptysis leading to acute onset of anemia and hemodynamic instability. An active diagnostic workup including serologic titer of C-ANCA, imaging, intensive care, and aggressive immunosuppression is the key to DAH management. We report a case of DAH secondary to GPA that presented with hemoptysis leading to severe anemia, initially resuscitated symptomatically and started on plasmapheresis with pulse steroids and cyclophosphamide. Timely diagnosis and management led to a remarkable recovery of the pulmonary symptoms and imaging findings of DAH.


Assuntos
Granulomatose com Poliangiite/tratamento farmacológico , Imunossupressores/uso terapêutico , Plasmaferese/métodos , Anticorpos Anticitoplasma de Neutrófilos/análise , Anticorpos Anticitoplasma de Neutrófilos/sangue , Biomarcadores/análise , Biomarcadores/sangue , Serviço Hospitalar de Emergência/organização & administração , Feminino , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/fisiopatologia , Humanos , Pessoa de Meia-Idade , Radiografia/métodos
11.
Arthritis Rheumatol ; 71(11): 1888-1893, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31216123

RESUMO

OBJECTIVE: To assess the frequency of venous thromboembolism (VTE) events in the Rituximab in Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (RAVE) trial and identify novel potential risk factors. METHODS: VTE events in 197 patients enrolled in the RAVE trial were analyzed. Baseline demographic and clinical characteristics were recorded, and univariate and multivariate analyses were performed to identify factors associated with VTE in ANCA-associated vasculitis (AAV). RESULTS: VTE occurred in 16 patients (8.1%) with an overall average time to event of 1.5 months (range 1.0-2.75). In univariate analyses with calculation of hazard ratios (HRs) and 95% confidence intervals (95% CIs), heart involvement (HR 17.408 [95% CI 2.247-134.842]; P = 0.006), positive proteinase 3 (PR3)-ANCA (HR 7.731 [95% CI 1.021-58.545]; P = 0.048), pulmonary hemorrhage (HR 3.889 [95% CI 1.448-10.448]; P = 0.008), and the presence of red blood cell casts (HR 15.617 [95% CI 3.491-69.854]; P < 0.001) were associated with the onset of VTE. In multivariate models adjusted for age and sex, the significant associations between VTE events and heart involvement (HR 21.836 [95% CI 2.566-185.805]; P = 0.005), PR3-ANCA (HR 9.12 [95% CI 1.158-71.839]; P = 0.036), pulmonary hemorrhage (HR 3.91 [95% CI 1.453-10.522]; P = 0.007), and urinary red blood cell casts (HR 16.455 [95% CI 3.607-75.075]; P < 0.001) remained. CONCLUSION: Patients diagnosed as having AAV with pulmonary hemorrhage, positive PR3-ANCA, heart involvement, and the presence of red blood cell casts are at an increased risk to develop VTE. Further studies are needed to confirm and expand these findings and to explore the mechanisms of hypercoagulability in these patients with the aim of informing potential targets for therapeutic intervention.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/epidemiologia , Eritrócitos , Hemorragia/epidemiologia , Pneumopatias/epidemiologia , Embolia Pulmonar/epidemiologia , Urina/citologia , Trombose Venosa/epidemiologia , Adulto , Idoso , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Feminino , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/epidemiologia , Granulomatose com Poliangiite/imunologia , Humanos , Masculino , Poliangiite Microscópica/tratamento farmacológico , Poliangiite Microscópica/epidemiologia , Poliangiite Microscópica/imunologia , Pessoa de Meia-Idade , Mieloblastina/imunologia , Peroxidase/imunologia , Modelos de Riscos Proporcionais , Fatores de Risco , Tromboembolia Venosa/epidemiologia
12.
BMJ Case Rep ; 12(6)2019 Jun 18.
Artigo em Inglês | MEDLINE | ID: mdl-31217209

RESUMO

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss Syndrome, is a systemic autoimmune disease that is usually associated with asthma and eosinophilia. It is a rare condition associated with antineutrophil cytoplasmic antibodies (ANCA). We report a case of a 52-year-old Nigerian woman who presented with bilateral leg swelling with multiple ulcers, background history of allergic rhinitis and chronic sinusitis since adolescence. There were also associated symptoms of peripheral neuropathy, weight loss, peripheral blood eosinophilia and positive perinuclear ANCA and she was previously managed for rheumatoid arthritis and asthma. She fulfilled the American College of Rheumatology criteria for EGPA and was treated with glucocorticoids and methotrexate which led to significant improvement of her symptoms.


Assuntos
Glucocorticoides/uso terapêutico , Granulomatose com Poliangiite/diagnóstico , Imunossupressores/uso terapêutico , Úlcera da Perna/patologia , Metotrexato/uso terapêutico , Anticorpos Anticitoplasma de Neutrófilos , Edema , Feminino , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/fisiopatologia , Humanos , Pessoa de Meia-Idade , Nigéria , Resultado do Tratamento , Perda de Peso
13.
J Immunol Res ; 2019: 1732175, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31198793

RESUMO

The purpose of this paper is to collect and summarize all evidences relating to an association between ANCA-associated vasculitides (AAVs) and hematologic malignancies, in the form of either a paraneoplastic vasculitis or leukemias and lymphomas developing on a preexisting vasculitis. Additionally, the role of cyclophosphamide in vasculitis treatment has been assessed and compared to rituximab. Paraneoplastic AAV seems to be an uncommon presentation of hemopathies. Hematologic malignancy risk in AAV is more likely to be increased by cyclophosphamide, although not yet definitely proven. Furthermore, the pathogenesis of ANCA-associated vasculitis has been reviewed with particular emphasis on the role of proteinase 3 (PR3) in fuelling granulomatosis with polyangiitis (GPA) inflammation. PR3 is a bactericidal protein expressed by neutrophilic granules and on their plasma membrane. Derangements in its expression and function have been linked to leukemias and GPA alike. PR3-derived PR1 peptide is being studied as an immunotherapy target in leukemia and multiple myeloma. This study is aimed at bringing together various evidences from the field of immunological and hematological research, at exposing contradictions, and at revealing novel insights on the association between ANCA-associated vasculitis and hematologic malignancies.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/metabolismo , Ciclofosfamida/uso terapêutico , Granulomatose com Poliangiite/metabolismo , Neoplasias Hematológicas/metabolismo , Neutrófilos/imunologia , Rituximab/uso terapêutico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Grânulos Citoplasmáticos/metabolismo , Granulomatose com Poliangiite/tratamento farmacológico , Neoplasias Hematológicas/tratamento farmacológico , Humanos , Mieloblastina/metabolismo , Peptídeos/metabolismo
15.
BMJ Case Rep ; 12(4)2019 Apr 24.
Artigo em Inglês | MEDLINE | ID: mdl-31023740

RESUMO

Gingival pathology is a daily presentation, however a small number of systemic conditions can manifest similar to a common gingival condition and have fatal results. Dentist referred 56-year-old woman to Oral and Maxillofacial Surgery department with a 2-week medical history of gingival bleeding not responding to local measures. Biopsy showed eosinophilic infiltrate and vasculitis, and blood tests showed positive markers including cytoplasmic antineutrophil cytoplasmatic antibodies. Granulomatosis with polyangiitis is a rare disease affecting the respiratory tract, blood vessels and kidneys. Oral lesions are rarely the primary presenting feature. When left untreated, most cases are fatal within a year of diagnosis. The diagnosis can only be made when certain criteria are found, including granular oral lesions exhibiting an eosinophilic inflammatory infiltrate on biopsy. With 5% of cases showing intraoral lesions as the primary feature, it is essential that dentists have the knowledge of this rare disease to refer and not to treat as a common gingival condition.


Assuntos
Odontólogos/educação , Doenças da Gengiva/diagnóstico , Granulomatose com Poliangiite/patologia , Úlceras Orais/etiologia , Assistência ao Convalescente , Antibacterianos/uso terapêutico , Anticorpos Anticitoplasma de Neutrófilos/metabolismo , Biópsia , Diagnóstico Diferencial , Eosinófilos/patologia , Feminino , Células Gigantes de Corpo Estranho/patologia , Doenças da Gengiva/patologia , Glucocorticoides/uso terapêutico , Granulomatose com Poliangiite/sangue , Granulomatose com Poliangiite/tratamento farmacológico , Hemorragia/diagnóstico , Hemorragia/etiologia , Humanos , Pessoa de Meia-Idade , Úlceras Orais/diagnóstico , Úlceras Orais/tratamento farmacológico , Prednisolona/administração & dosagem , Prednisolona/uso terapêutico , Doenças Raras , Resultado do Tratamento , Combinação Trimetoprima e Sulfametoxazol/administração & dosagem , Combinação Trimetoprima e Sulfametoxazol/uso terapêutico
16.
Tidsskr Nor Laegeforen ; 139(7)2019 Apr 09.
Artigo em Norueguês, Inglês | MEDLINE | ID: mdl-30969044

RESUMO

BACKGROUND: Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, has a predilection for the upper airways, lungs and kidneys. However, any other organ can be affected. Although cutaneous lesions are common, they have only rarely been reported as a primary manifestation of the disease. CASE PRESENTATION: We present a case of a teenage boy with pyoderma gangrenosum-like ulcerations of the neck and face. Anti-neutrophil cytoplasmic antibody with antigen specificity for proteinase 3 (PR3-ANCA) was detected. In the absence of other symptoms and organ manifestations, the ulcerations were still considered to be pyoderma gangrenosum. The ulcers started to heal during treatment with corticosteroids and infliximab. One month later the patient developed sinusitis, and eventually lost vision in his left eye. The diagnosis was changed to GPA and he started treatment with methylprednisolone, rituximab and cyclophosphamide with good response on vision, sinusitis and ulcerations. INTERPRETATION: Recognition of this rare skin presentation of GPA is essential, to prevent delays in diagnosis and treatment that can lead to organ damage.


Assuntos
Face/patologia , Granulomatose com Poliangiite , Pescoço/patologia , Úlcera Cutânea/etiologia , Adolescente , Fármacos Dermatológicos/administração & dosagem , Fármacos Dermatológicos/uso terapêutico , Diagnóstico Diferencial , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/patologia , Humanos , Fatores Imunológicos/administração & dosagem , Fatores Imunológicos/uso terapêutico , Infliximab/administração & dosagem , Infliximab/uso terapêutico , Masculino , Prednisolona/administração & dosagem , Prednisolona/uso terapêutico , Pioderma Gangrenoso/diagnóstico , Rituximab/administração & dosagem , Rituximab/uso terapêutico , Úlcera Cutânea/tratamento farmacológico , Úlcera Cutânea/patologia
17.
J Stroke Cerebrovasc Dis ; 28(8): e107-e109, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31027983

RESUMO

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, a vasculitis affecting small and medium sized vessels usually affects the upper and lower respiratory tract, the kidneys, and the eyes. Neurologic manifestation in central nervous system (CNS) is less frequent than the peripheral and usually is in form of stroke. Few cases of lateral medullary ischemic stroke (Wallenberg syndrome) due to GPA have been reported. A 41 year-old female, presented with acute vertigo, nausea/vomiting, hiccups, dysphagia. In physical examination she had a saddled nose, horner syndrome, soft palate paralysis, crossed hypoesthesia of face, and limbs and hemi-ataxia. Brain magnetic imaging revealed a left lateral medullary infarction and sinusitis confirmed by paranasal Sinus CT scans. Chest CT showed a cavitary mass. Laboratory findings were remarkable for anemia, elevated erythrocyte sedimentation rate, and C-reactive protein. Cytoplasmic antineutrophil cytoplasmic antibody (ANCA)/anti-proteinase 3 was positive. Diagnosis of GPA was established and treatment was started. During 6-month follow-up improvement was satisfying and no relapses occurred. Medullary infarct is reported in few GPA patients, especially at presentation. Definite diagnosis is based on tissue biopsy. Although in context of extra CNS involvement and positive ANCA diagnosis can be made confidently. Treatment of choice in CNS involvement is not clear, corticosteroids and immunosuppressives seem effective. CNS involvement, especially stroke may present GPA or accompany extra CNS symptoms. Prompt diagnosis and treatment is essential.


Assuntos
Granulomatose com Poliangiite/complicações , Síndrome Medular Lateral/etiologia , Adulto , Anticorpos Anticitoplasma de Neutrófilos/sangue , Aspirina/uso terapêutico , Biomarcadores/sangue , Ciclofosfamida/uso terapêutico , Imagem de Difusão por Ressonância Magnética , Feminino , Glucocorticoides/uso terapêutico , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/imunologia , Humanos , Imunossupressores/uso terapêutico , Síndrome Medular Lateral/diagnóstico por imagem , Síndrome Medular Lateral/tratamento farmacológico , Metilprednisolona/uso terapêutico , Mieloblastina/imunologia , Tomografia Computadorizada por Raios X , Resultado do Tratamento
18.
Rheumatol Int ; 39(6): 1083-1090, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30923955

RESUMO

Rituximab, an anti-CD20 monoclonal antibody causing selective B-cell depletion, is used for various systemic inflammatory and autoimmune diseases (SIADs). Long-term safety data on rituximab are limited. The objectives of this study were to evaluate the long-term safety and tolerability of rituximab treatment for SIADs. A retrospective, single-center observational study including all patients ≥ 16 years treated with rituximab for SIADs was performed. The electronic medical records were reviewed, and data concerning indication and duration of rituximab treatment, prior and concurrent immunosuppressive therapy, and adverse events such as infections requiring hospitalization, dysgammaglobulinemia and end organ damage, were collected. A total of 70 patients were included, with a median treatment duration of 54 months, ranging 30-138 months. The most common indications for rituximab treatment were granulomatosis with polyangiitis (22.9%), primary Sjögren's syndrome (20.0%) and systemic lupus erythematosus (14.3%). Infections and persistent dysgammaglobulinemia were the most common adverse events, occurring in 34.3% and 25.7%, respectively. A total of 64 infections were observed in 24 (34.3%) patients, including 1 case of fatal infection. Seventeen patients performed B-cell quantitation during the first 2 years following discontinuation, of which only four (19.0%) demonstrated B-cell reconstitution. End organ damage occurred in two patients, presenting as pyoderma gangrenosum and interstitial pneumonitis. No opportunistic infections were observed. Three patients died during the observational period, of which one was due to lethal infection. This study presents observational data with long treatment duration. It demonstrates that long-term rituximab treatment is relatively well tolerated, and that no cumulative side effects were observed.


Assuntos
Antirreumáticos/efeitos adversos , Doenças Autoimunes/tratamento farmacológico , Disgamaglobulinemia/induzido quimicamente , Inflamação/tratamento farmacológico , Rituximab/administração & dosagem , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Quimioterapia Combinada , Feminino , Granulomatose com Poliangiite/tratamento farmacológico , Hospitalização , Humanos , Imunossupressores/uso terapêutico , Doenças Pulmonares Intersticiais/induzido quimicamente , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Pioderma Gangrenoso/induzido quimicamente , Estudos Retrospectivos , Rituximab/uso terapêutico , Síndrome de Sjogren/tratamento farmacológico , Fatores de Tempo , Adulto Jovem
19.
BMJ Case Rep ; 12(2)2019 Feb 28.
Artigo em Inglês | MEDLINE | ID: mdl-30824462

RESUMO

A 62-year-old man presented with excruciating joint pains, back stiffness and numbness of his hands and feet. Over the past 18 months, he had experienced similar episodes for which the diagnoses of bilateral carpal tunnel syndrome and lateral epicondylitis had been made. Physical examination revealed polyarticular arthritis affecting the shoulders, wrists and right knee. Palpable purpura overlying the calves and ankles was present. Laboratory tests showed markedly elevated erythrocyte sedimentation rate and C-reactive protein in the setting of negative blood and urine cultures. Rheumatoid factor and antinuclear antibodies were negative. Chest CT demonstrated bilateral pulmonary infiltrates. A punch biopsy of the rash showed leukocytoclastic vasculitis. Anti-proteinase-3 titers returned strongly positive. A diagnosis of granulomatosis with polyangiitis was made. Treatment with high-dose steroids, followed by rituximab resulted in normalisation of inflammatory markers with subsequent resolution of joint pains, rash and pulmonary infiltrates and improvement of neuropathic symptoms.


Assuntos
Síndrome do Túnel Carpal , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/patologia , Cotovelo de Tenista , Anti-Inflamatórios/uso terapêutico , Artralgia/etiologia , Biópsia , Diagnóstico Diferencial , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Fatores Imunológicos/uso terapêutico , Pulmão/diagnóstico por imagem , Masculino , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Rituximab/uso terapêutico , Tomografia Computadorizada por Raios X
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