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2.
Medicine (Baltimore) ; 98(48): e18178, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31770269

RESUMO

RATIONALE: Occasionally, tubulointerstitial lesions can be found in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). However, significantly isolated tubulointerstitial nephritis (TIN) with germinal centers is rare. PATIENT CONCERNS: A 17-year-old Chinese Han patient showed rapidly progressive glomerulonephritis, anuria, and serum creatinine of 19.4 mg/dL. DIAGNOSIS: He had positive ANCA targeting myeloperoxidase (55.0 RU/mL). The renal biopsy showed crescent formation in 100% of glomeruli. Of special note, the glomerular crescents were surrounded by granulomatous inflammation, extensive tubular destruction or disappearance, and massive interstitial infiltration. A diagnosis of AAV was thus made with the involved organ restricted to the kidney. INTERVENTIONS: The patient underwent 7 rounds of plasmapheresis, 3 pulses of methylprednisolone therapy (500 mg per pulse), and oral prednisolone (50 mg/d). Rituximab (500 mg) was used after the plasma exchange treatment. OUTCOMES: ANCA was negative, while anti-modified C-reactive protein (anti-mCRP) antibodies remained positive. The patient was dependent on hemodialysis. We found anti-mCRP antibody in the serum of the patient, with the major epitope on amino acids 35 to 47 of mCRP. LESSONS: We proposed that the anti-mCRP antibody might play an important role in this case of acute TIN in AAV.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Proteína C-Reativa/imunologia , Glucocorticoides/administração & dosagem , Nefrite Intersticial , Troca Plasmática/métodos , Plasmaferese/métodos , Rituximab/administração & dosagem , Adolescente , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/fisiopatologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/terapia , Anticorpos Anticitoplasma de Neutrófilos/sangue , Progressão da Doença , Centro Germinativo/patologia , Humanos , Fatores Imunológicos/administração & dosagem , Testes de Função Renal/métodos , Glomérulos Renais/patologia , Glomérulos Renais/fisiopatologia , Masculino , Nefrite Intersticial/diagnóstico , Nefrite Intersticial/imunologia , Nefrite Intersticial/fisiopatologia , Nefrite Intersticial/terapia , Resultado do Tratamento
3.
Wiad Lek ; 72(9 cz 1): 1691-1696, 2019.
Artigo em Polonês | MEDLINE | ID: mdl-31586985

RESUMO

Antineutrophil cytoplasmic antibodies (ANCA) play a key role in the pathogenesis of ANCA-associated vasculitides (AAV). These diseases are rare, occur in every age, but most commonly in adults and elder patients. Among them there are: granulomathosis with poyangiitis (GPA), microscopic poyangiitis (MPA) and eosinophilic granulomathosis with polyangiitis (EGPA). In the article we try to analyse the course of AAV in eldery patients, according to accessible literature. Among AAV patients, those with MPA diagnosis are elder than GPA and EGPA patients. Elder AAV patients present more frequently severe kidney and lung involvement. Elder patients are more at risk to develop complications in the course of disease, but also treatment-related, including severe infections. In elder patients immunosupresive agents dosage, therethore, should be tapered and adjusted to the renal function.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/fisiopatologia , Idoso , Anticorpos Anticitoplasma de Neutrófilos/sangue , Granulomatose com Poliangiite , Humanos , Poliangiite Microscópica
4.
Biosens Bioelectron ; 142: 111548, 2019 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-31400729

RESUMO

Antimyeloperoxidase (anti-MPO) is regarded as one of the most important circulating autoantibodies for anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAVs). Hence, it is crucial for highly sensitive detection of anti-MPO to monitor efficacy of AAVs in clinical diagnosis. Herein, a highly sensitive electrochemiluminescence (ECL) immunosensor for anti-MPO detection was constructed by combining reduced graphene oxide-supported PtCo nanocubes hybrids (PtCo@rGO) with hybridization chain reaction (HCR) as signal amplification. Multiple ECL luminophores (Dox-ABEI) prepared by cross-linking of N-(aminobutyl)-N-(ethylisoluminol) (ABEI) and doxorubicin (Dox) were intercalated into dsDNA products of HCR, achieving the effective immobilization of ECL luminophores to obtain strong ECL emission. Benefiting from the efficient catalytic activity of PtCo@rGO toward H2O2, the massive the superoxide radical (O2●-) were generated to further react with ABEI for ECL emission. Thus, the designed ECL immunoassay for anti-MPO detection exhibited excellent sensitivity of a concentration variation from 50 fg/mL to 1 ng/mL and a detection limit of 15.68 fg/mL. Importantly, this work proposed an enzyme-free ECL immunoassay with high sensitivity, excellent specificity for protein detection in clinical diagnosis.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/imunologia , Técnicas Biossensoriais/métodos , Grafite/química , Nanopartículas Metálicas/química , Peroxidase/imunologia , Anticorpos Anticitoplasma de Neutrófilos/análise , Anticorpos Anticitoplasma de Neutrófilos/sangue , Anticorpos Imobilizados/química , Anticorpos Imobilizados/imunologia , Cobalto/química , DNA/química , Humanos , Imunoensaio/métodos , Limite de Detecção , Medições Luminescentes/métodos , Luminol/química , Hibridização de Ácido Nucleico/métodos , Oxirredução , Platina/química
6.
BMJ Case Rep ; 12(7)2019 Jul 10.
Artigo em Inglês | MEDLINE | ID: mdl-31296634

RESUMO

Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic small-vessel vasculitic disease that can present with positive MPO-P-ANCA (myeloperoxidase-perinuclear-anti-neutrophil cytoplasmic antibody). It is a rare condition that is difficult to diagnose. We present the case of a 64-year-old man with late-onset adult asthma and treated nasopharyngeal carcinoma who initially presented to us with proximal myopathy. Thereafter, he developed a constellation of fleeting symptoms which included rhinosinusitis, mononeuritis multiplex, skin vasculitis and arthritis. Blood investigations showed that he had eosinophilia, and skin biopsy demonstrated dermal vasculitis with eosinophils. He was found to be MPO-C-ANCA positive, and although initially thought to have granulomatosis with polyangiitis, the diagnosis was later revised to EGPA. This case highlights the diagnostic challenges with atypical presentations of EGPA and also presents a rare case of positive MPO-C-ANCA that has never been described in EGPA before.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/sangue , Asma/complicações , Síndrome de Churg-Strauss/complicações , Síndrome de Churg-Strauss/diagnóstico , Granulomatose com Poliangiite , Peroxidase/sangue , Síndrome de Churg-Strauss/sangue , Diagnóstico Diferencial , Humanos , Masculino , Pessoa de Meia-Idade
7.
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
8.
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
9.
BMC Pulm Med ; 19(1): 122, 2019 Jul 08.
Artigo em Inglês | MEDLINE | ID: mdl-31286925

RESUMO

BACKGROUND: Granulomatosis with polyangiitis (GPA) is characterised by the main violation of the upper and lower respiratory tract and kidney. GPA is considered a systemic vasculitis of medium-sized and small blood vessels where aortic involvement is extremely rare. CASE PRESENTATION: A 28-year-old male was admitted to the hospital due to 4 h of chest pain. Computed tomography scan of the aorta showed a thickened aortic wall, pulmonary lesions, bilateral pleural effusion and pericardial effusion. The aortic dissection should be considered. An emergency operation was performed on the patient. Surgical biopsies obtained from the aortic wall showed destructive changes, visible necrosis, granulation tissue hyperplasia and a large number of acute and chronic inflammatory cells. Nearly a year later, the patient was re-examined for significant pulmonary lesions. His laboratory studies were significantly positive for anti-neutrophilic antibody directed against proteinase 3. Finally, the diagnosis of GPA was obviously established. CONCLUSIONS: Although GPA rarely involves the aorta, we did not ignore the fact that GPA may involve large blood vessels. In addition, GPA should be included in the systemic vasculitis that can give rise to aortitis and even aortic dissection.


Assuntos
Aneurisma Dissecante/diagnóstico por imagem , Aortite/diagnóstico por imagem , Granulomatose com Poliangiite/diagnóstico , Pulmão/patologia , Adulto , Anticorpos Anticitoplasma de Neutrófilos/sangue , Dor no Peito/etiologia , Ecocardiografia , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/fisiopatologia , Humanos , Masculino , Mieloblastina/imunologia , Tomografia Computadorizada por Raios X
10.
Int Arch Allergy Immunol ; 180(1): 64-71, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31189169

RESUMO

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis associated with asthma, anti-neutrophil cytoplasmic antibodies (ANCA) positivity, and tissue eosinophilia. OBJECTIVE: To describe the presenting clinical features, significant biochemical alterations, and also potential pathogenic factors in adult patients diagnosed in our Center over a period of >20 years. METHOD: A retrospective study of EGPA patients diagnosed from 1994 to 2019 at ASST Grande Ospedale Metropolitano Niguarda Ca' Granda, Milan (Italy), which was performed according to the 1990 American College of Rheumatology criteria and Chapel Hill Consensus Conference definition. A dataset was compiled, registering demographic and clinical features, biochemical analysis at onset, and also the therapies received 3 months prior to EGPA diagnose. Statistical analyses were subsequently conducted dividing patients in 2 groups based on ANCA positivity and comparing them. RESULTS: Two groups were clearly identified by ANCA serology and specific organ involvement in accordance with literature reports; however, our data underline for the first time the association between anti-leukotriene receptor antagonists (LTRAs) and ANCA positivity. The group of previously treated patients presents an OR of 6.42 to be ANCA positive. This finding could be attributed to an imbalanced stimulation of leukotriene receptors, inducing both mast cells activation and an increased neutrophil extracellular traps release from neutrophils. CONCLUSION: Despite the limitations of this retrospective study, the association between LTRAs and ANCA antibodies elucidates the mechanism by which innate immunity is directly involved in tolerance breakdown and autoantibodies production. Validation of our results with targeted studies could clarify the differences between ANCA-positive and ANCA-negative patients with important consequences on the use of some drug classes in the treatment of EGPA and asthmatic subjects.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/imunologia , Autoanticorpos/imunologia , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/imunologia , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/imunologia , Leucotrienos/imunologia , Adulto , Anticorpos Anticitoplasma de Neutrófilos/sangue , Autoanticorpos/sangue , Biomarcadores , Síndrome de Churg-Strauss/sangue , Feminino , Granulomatose com Poliangiite/sangue , Humanos , Itália , Masculino , Pessoa de Meia-Idade , Fenótipo , Estudos Retrospectivos , Avaliação de Sintomas
11.
Biomed Res Int ; 2019: 7508240, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31236412

RESUMO

We investigated whether serum aminoacyl-tRNA synthetase-interacting multifunctional protein-1 (AIMP1) could predict severe cases of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) based on the Birmingham vasculitis activity score (BVAS). Sixty-one patients with AAV were selected for inclusion from our prospective AAV cohort. AAV-specific indices and clinical manifestations were assessed, and laboratory tests were performed on the day of blood sampling. Patients with severe AAV were defined as those with a BVAS higher than the lower limit of the highest tertile of BVAS (BVAS ≥ 12). We measured serum AIMP1 levels of the stored serum samples. A total of 20 (32.8%) and 41 (67.2%) patients were classified as having severe and nonsevere AAV according to the cut-off of BVAS ≥ 12. Patients with severe AAV showed higher frequencies of general and renal manifestations, along with ANCA positivity, and exhibited a higher mean neutrophil count, erythrocyte sedimentation rate, and C-reactive protein levels, but lower mean haemoglobin and serum albumin levels than those with nonsevere AAV. The mean serum AIMP1 level in patients with severe AAV was significantly higher than that of patients with nonsevere AIMP1 (351.1 vs. 98.4 pg/mL, p = 0.006). Multivariate logistic regression analysis including variables showing significance in univariate analyses revealed that only serum AIMP1 exhibited a significant association with severe AAV (odds ratio 1.004, p = 0.031). When we set the optimal cut-off of serum AIMP1 for severe AAV to 50.28 pg/mL, patients with severe AAV more frequently had AIMP1 levels above the cut-off than those with nonsevere AAV (80.0% vs. 31.7%, relative risk 8.615, p < 0.001). The results from our study suggest that serum AIMP1 can be used to estimate the cross-sectional severe AAV population based on the BVAS.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/sangue , Anticorpos Anticitoplasma de Neutrófilos/sangue , Biomarcadores/sangue , Citocinas/sangue , Proteínas de Neoplasias/sangue , Proteínas de Ligação a RNA/sangue , Adulto , Idoso , Aminoacil-tRNA Sintetases/genética , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/genética , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Anticorpos Anticitoplasma de Neutrófilos/genética , Sedimentação Sanguínea , Estudos Transversais , Feminino , Humanos , Rim/metabolismo , Rim/patologia , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Estudos Prospectivos , Índice de Gravidade de Doença
12.
J Clin Lab Anal ; 33(6): e22908, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31106488

RESUMO

BACKGROUND: Antineutrophil cytoplasmic autoantibodies against neutrophil granule bactericidal/permeability-increasing protein (BPI-ANCA) has been found in many inflammatory diseases, such as COPD, which can reduce the killing effect of BPI on Gram-negative bacteria. This study was aimed to assess the clinical significance of BPI-ANCA detecting in COPD patients with Pseudomonas aeruginosa (P aeruginosa) colonization. METHODS: A total of 216 COPD patients with lung P aeruginosa colonization, 244 patients with P aeruginosa infection from June 2015 to June 2018, and 100 healthy individuals were included. Serum BPI-ANCA, tumor necrosis factor (TNF)-α, and interleukin (IL)-6 and IL-1ß levels were detected by ELISA, and the lung function of the patients was measured at stable clinical stages. Patients with COPD were grouped according to BPI-ANCA detection and GOLD criteria, and serum TNF-α, IL-6, and IL-1ß levels and indices reflecting lung function were compared and analyzed between groups. RESULTS: Positive rate of BPI-ANCA in COPD patients with P aeruginosa colonization was 48.15%; and compared with BPI-ANCA(-) group, FEV1 %pred and FEV1 /FVC(%) in BPI-ANCA(+) patients were significantly decreased, while TNF-α, IL-6, and IL-1ß levels were elevated. There were 31.73% and 36.54% BPI-ANCA(+) patients with severe and very severe airflow limitation, respectively, which was significantly higher than that in the BPI-ANCA(-) group. FEV1 %pred and FEV1 /FVC(%) were negatively correlated with TNF-α, IL-6, IL-1ß, and NEU%. C-reactive protein (CRP) was negatively correlated with FEV1 %pred, yet not significantly correlated with FEV1 /FVC(%). CONCLUSION: BPI-ANCA positivity is associated with inflammatory status in COPD patients with pulmonary P aeruginosa colonization and can be used as a potential biomarker assessing disease severity.


Assuntos
Peptídeos Catiônicos Antimicrobianos/sangue , Infecções por Pseudomonas/sangue , Doença Pulmonar Obstrutiva Crônica/microbiologia , Idoso , Anticorpos Anticitoplasma de Neutrófilos/sangue , Proteínas Sanguíneas , Estudos de Casos e Controles , Feminino , Humanos , Interleucina-1beta/sangue , Interleucina-6/sangue , Masculino , Pessoa de Meia-Idade , Doença Pulmonar Obstrutiva Crônica/sangue , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Testes de Função Respiratória , Estudos Retrospectivos , Fator de Necrose Tumoral alfa/sangue
13.
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
15.
Med. clín (Ed. impr.) ; 152(8): 310-316, abr. 2019. tab
Artigo em Espanhol | IBECS | ID: ibc-183610

RESUMO

Existe una gran variedad de marcadores útiles tanto en el diagnóstico como en el seguimiento de la enfermedad inflamatoria intestinal. Clásicamente se han utilizado los serológicos, ampliamente distribuidos y accesibles, pero en los últimos años han cobrado importancia los fecales, en especial la calprotectina fecal, por haber demostrado mayor precisión a la hora tanto de establecer la sospecha de la enfermedad como de predecir la curación mucosa o la persistencia de actividad inflamatoria. La calprotectina fecal muestra buena capacidad para predecir estudios endoscópicos patológicos, pero tiene una especificidad limitada ya que puede alterarse en otros cuadros digestivos con síntomas similares. La calprotectina fecal presenta mayor precisión cuando se asocia a otros parámetros, en especial a la proteína C reactiva, y a escalas clínicas de actividad inflamatoria. Finalmente, hay múltiples marcadores de nueva generación, serológicos y fecales, de los que hay escasa evidencia, aunque algunos han mostrado resultados prometedores en diferentes estudios


There are many useful biomarkers for initial diagnosis and the management of inflammatory bowel disease. Serologic biomarkers have been traditionally used because they are widely disposable, but recently faecal biomarkers, especially faecal calprotectin, have acquired great importance as they have shown to be more precise when establishing suspicion of the disease and also as predictors of mucosal healing or persistence of inflammatory activity. Faecal calprotectin is a good tool for predicting abnormal endoscopic studies, but has limited specificity because its levels can be altered in many digestive diseases presenting with similar symptoms. The precision of faecal calprotectin is higher when associated with other altered parameters, especially with C-reactive protein, or with clinical scores of inflammatory activity. Finally, there are many new generation serologic and faecal biomarkers. Despite there not being much evidence about these yet, some of them have shown promising results in different studies


Assuntos
Humanos , Doença de Crohn/diagnóstico , Colite Ulcerativa/diagnóstico , Fezes/química , Proteína C-Reativa/análise , Piruvato Quinase/sangue , Anticorpos Anticitoplasma de Neutrófilos/sangue , Proteína S100A12/análise , Lactoferrina/análise , Sangue Oculto , Expressão Gênica , Biomarcadores/análise
16.
J Clin Rheumatol ; 25(5): 217-223, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30896460

RESUMO

INTRODUCTION: The value of antineutrophil cytoplasmic antibody (ANCA) measurements among patients with an established diagnosis of ANCA-associated vasculitis (AAV) to assess disease activity or predict relapse remains controversial, but recent evidence suggests a possible role for rituximab-treated patients. PATIENTS AND METHODS: All patients with active vasculitis and positive proteinase 3 (PR3)-ANCA who were starting a 2-year treatment course of rituximab for induction of remission at Addenbrooke's Hospital between January 2011 and January 2016 were included in this study. Common department practice consists of 6 g of rituximab given over 2 years, concomitant corticosteroids (0.5-1.0 mg/kg) with rapid taper over 3 months, and cessation of oral maintenance immunosuppressive agents at time of first rituximab dose. Clinical and laboratory data were collected retrospectively using electronic patient records. RESULTS: Fifty-seven patients with current PR3-ANCA positivity were included in the analysis. Median follow-up was 59 months. PR3-ANCA negativity was achieved in 25 patients (44%) with a median time of 14 months. Clinical remission was achieved in 53 patients (93%) with a median time of 3 months. Among the 53 patients who achieved remission during follow-up, 24 (45%) relapsed with a median time to relapse of 36 months from remission. Both PR3-ANCA-negative status and 50% reduction in PR3-ANCA from baseline (as time-varying covariates) were significantly associated with a longer time to relapse (PR3-ANCA-negative status: hazards ratio, 0.08 [95% confidence interval, 0.01-0.63, p = 0.016]; 50% reduction in PR3-ANCA: hazards ratio, 0.25 [95% confidence interval, 0.18-0.99, p = 0.046]). CONCLUSIONS: Achieving and maintaining PR3-ANCA negativity after rituximab was associated with longer-lasting remission.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Anticorpos Anticitoplasma de Neutrófilos/sangue , Fatores Imunológicos/uso terapêutico , Mieloblastina/sangue , Rituximab/uso terapêutico , Biomarcadores/sangue , Ensaio de Imunoadsorção Enzimática , Feminino , Glucocorticoides/uso terapêutico , Humanos , Masculino , Pessoa de Meia-Idade , Indução de Remissão
17.
Ann Otol Rhinol Laryngol ; 128(8): 708-714, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30924348

RESUMO

OBJECTIVES: The aim of this study was to review the histologic diagnostic yield of airway biopsies with a suspected granulomatosis with polyangiitis (GPA) diagnosis at a single center devoted to respiratory diseases using previously published criteria. A secondary aim was to apply the algorithm proposed by the European Medicines Agency to determine whether more biopsies were confidently identified as having GPA diagnoses. METHODS: From a total of 132 airway biopsies (2005-2015), 50 were randomly selected for second review by an expert pathologist, and previously published criteria were applied. Thereafter, antineutrophil cytoplasm autoantibody testing results and the European Medicines Agency algorithm were applied. RESULTS: Repeat review and application of the published criteria resulted in an increase from 16 to 25 diagnoses of GPA. This increased to 35 of 50 when antineutrophil cytoplasm autoantibody results and the European Medicines Agency algorithm were applied. Interobserver correlation was 57.5% among pathologists (κ = 0.19), which was likely due to missing clinical information and inadequate tissue samples. Patients with generalized disease were 2.6 times more likely to obtain diagnostic GPA airway biopsy results than those with limited disease (airway only). CONCLUSIONS: An increase in the diagnostic yield of this malady could be attained by following an algorithm that incorporates carefully retrieved clinical, endoscopic, and serologic data, coupled with systematic histopathologic sample review.


Assuntos
Granulomatose com Poliangiite/diagnóstico , Sistema Respiratório/patologia , Adolescente , Adulto , Idoso , Algoritmos , Anticorpos Anticitoplasma de Neutrófilos/sangue , Biópsia , Feminino , Granulomatose com Poliangiite/sangue , Granulomatose com Poliangiite/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Avaliação de Sintomas , Adulto Jovem
19.
BMJ Case Rep ; 12(3)2019 Mar 14.
Artigo em Inglês | MEDLINE | ID: mdl-30872335

RESUMO

Granulomatosis with polyangiitis (GPA) was diagnosed in a patient with a 16-month history of IgG4-related lung disease that spontaneously became asymptomatic. Cytoplasmic antineutrophil cytoplasmic antibody (ANCA) was positive at the time of diagnosis of IgG4-related disease (IgG4-RD), but there was no vasculitis or kidney disease. Sixteen months later he developed rapidly progressive glomerulonephritis that responded to cyclophosphamide treatment. While undergoing treatment for GPA, he was found to have a lung mass identified as small cell lung cancer. This mass was present at the time of the IgG4-RD diagnosis. GPA can be confused with IgG4-RD histologically and they rarely coexist. ANCA antibodies are primarily IgG4 subclass. IgG4-RD has been associated with cancer and may improve prognosis. We speculate that this patient may have had small cell lung cancer that incited an IgG4 predominant immune response with coexistent ANCA antibodies that eventually resulted in GPA. Immunosuppressive treatment of GPA likely accelerated the progression of the lung cancer.


Assuntos
Granulomatose com Poliangiite/patologia , Doença Relacionada a Imunoglobulina G4/patologia , Neoplasias Pulmonares/patologia , Pulmão/patologia , Carcinoma de Pequenas Células do Pulmão/patologia , Anticorpos Anticitoplasma de Neutrófilos/sangue , Ciclofosfamida/administração & dosagem , Ciclofosfamida/uso terapêutico , Evolução Fatal , Glomerulonefrite/complicações , Granulomatose com Poliangiite/complicações , Humanos , Imunoglobulina G/sangue , Doença Relacionada a Imunoglobulina G4/imunologia , Imunossupressores/uso terapêutico , Pulmão/diagnóstico por imagem , Pulmão/cirurgia , Neoplasias Pulmonares/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Carcinoma de Pequenas Células do Pulmão/complicações , Carcinoma de Pequenas Células do Pulmão/tratamento farmacológico , Cirurgia Torácica Vídeoassistida/métodos
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