RESUMO
We herein report a case of multisystem inflammatory syndrome in adults (MIS-A) complicated with Kikuchi-Fujimoto disease (KFD). A previously healthy 41-year-old man presented with painful swelling of the cervical lymph nodes, fever, diarrhea, conjunctivitis, edema, and hypotension one month after the onset of asymptomatic coronavirus disease 2019. Laboratory investigations revealed an elevation of CRP, and echocardiography indicated diastolic dysfunction. We diagnosed the patient to have MIS-A. Histopathology of the lymph nodes showed necrotizing lymphadenitis. After the initiation of hydrocortisone and diuretics, his symptoms resolved immediately. This case suggested that post-viral immune dysregulation in MIS-A could play a role in the etiology of KFD.
Assuntos
COVID-19 , Doenças do Tecido Conjuntivo , Linfadenite Histiocítica Necrosante , Adulto , COVID-19/complicações , Doenças do Tecido Conjuntivo/complicações , Linfadenite Histiocítica Necrosante/complicações , Linfadenite Histiocítica Necrosante/diagnóstico , Humanos , Linfonodos/patologia , Masculino , Síndrome de Resposta Inflamatória Sistêmica/complicações , Síndrome de Resposta Inflamatória Sistêmica/diagnósticoRESUMO
Advances in our understanding of interstitial lung disease (ILD) pathophysiology and natural history have led to the development of guidelines for the diagnosis and management of several of these complex diseases. The demographics of patients with ILD indicate the disease is not restricted to older adults. Connective tissue disease-associated ILD, familial pulmonary fibrosis, and post-COVID-19 fibrosis may affect women of child-bearing age. Recent trials have excluded pregnant women, thereby limiting the applicability of contemporary therapeutic advances to these patients. This review synthesizes the current knowledge of pregnancy outcomes in those with ILD, with a focus on connective tissue disease-associated ILD, and potential treatment implications for patients with ILD who are pregnant or considering pregnancy. Pregnancy considerations for patients with ILD include the need for preconception counseling and planning to ensure disease stability, medication and vaccination optimization, and multidisciplinary involvement of a patient's pulmonologist, obstetrician, and, when indicated, rheumatologist and genetic counselor. Evidence to date suggests that women with ILD can have safe and healthy pregnancies but that complications may occur in those with severe ILD.
Assuntos
COVID-19 , Doenças do Tecido Conjuntivo , Doenças Pulmonares Intersticiais , Humanos , Feminino , Gravidez , Idoso , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/epidemiologia , Doenças do Tecido Conjuntivo/complicaçõesRESUMO
Hydroxychloroquine has attracted attention in the treatment of COVID-19. Many conflicting findings have been reported regarding the efficacy and safety of this drug, which has been used safely in the rheumatological diseases for years. However, these studies lacked measurement methods that allow accurate assessment of hydroxychloroquine and its metabolite levels. The aim of this study was to measure hydroxychloroquine and its metabolite levels in whole blood samples of patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjogren's syndrome (SS) and scleroderma (Scl) by a robust, simple and accurate validated tandem mass spectrometric method, and to investigate the relationship between these levels with drug-related adverse effects and disease activity scores. The validated LC-MS/MS method was applied to measure blood hydroxychloroquine and its metabolite levels of patients with RA, SLE, SS, Scl. Various haematological and biochemical parameters were measured with Beckman-Coulter AU 5800 and Beckman Coulter LH 780 analyzers, respectively. QTc intervals were calculated with Bazett's formula, and the patients were followed up by clinicians in terms of clinical findings and adverse effects. Hydroxychloroquine levels of patients were similar to previous studies. There was a negative correlation between disease activity scores and hydroxychloroquine levels, while the highest correlation was between QTc interval, creatinine and GFR levels with desethylchloroquine. Bidetylchloroquine had the highest correlation with RBC count and liver function tests. Our findings showed that hydroxychloroquine and its metabolite levels were associated with disease activity scores, renal, hepatic function, QTc prolongation, and hematological parameters.
Assuntos
Antimaláricos/efeitos adversos , Antimaláricos/sangue , COVID-19/complicações , Doenças do Tecido Conjuntivo/complicações , Hidroxicloroquina/efeitos adversos , Hidroxicloroquina/sangue , Adulto , Idoso , Cromatografia Líquida de Alta Pressão , Creatinina/sangue , Eletrocardiografia , Contagem de Eritrócitos , Feminino , Taxa de Filtração Glomerular/efeitos dos fármacos , Humanos , Testes de Função Renal , Testes de Função Hepática , Síndrome do QT Longo/induzido quimicamente , Masculino , Pessoa de Meia-Idade , Espectrometria de Massas em Tandem , Adulto JovemRESUMO
OBJECTIVE: The aim of this study was to evaluate the diagnostic and prognostic value of red blood cell distribution width (RDW) in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). METHODS: We retrospectively reviewed 213 CTD-ILD patients and 97 CTD patients without ILD from February 2017 to February 2020. Hospital and office records were used as data sources. CTD-ILD patients were followed up. RESULTS: Patients with CTD-ILD had significantly higher RDW than those with CTD without ILD (p < 0.001). The area under the receiver operating characteristic curve (AUROC) of RDW for discriminating CTD-ILD from CTD without ILD was 0.64 (95% CI: 0.57-0.70, p < 0.001). The cutoff value of RDW for discriminating CTD-ILD from CTD without ILD was 13.95% with their corresponding specificity (55.9%) and sensitivity (70.1%). Correlation analyses showed that the increased RDW was significantly correlated with decreased DLCO%predicted (r = -0.211, p = 0.002). Cox multiple regression analysis indicated that RDW (HR = 1.495, p < 0.001) was an independent factor in the survival of CTD-ILD. The best cutoff value of RDW to predict the survival of patients with CTD-ILD was 14.05% (AUC = 0.78, 95% CI: 0.72-0.84, p < 0.001). The log-rank test showed a significant difference in survival between the two groups (RDW > 14.05% and RDW < 14.05%). CONCLUSION: RDW was higher in CTD-ILD patients and had a negative correlation with DLCO%predicted. RDW may be an important serum biomarker for severity and prognosis of patients with CTD-ILD.
Assuntos
Biomarcadores/sangue , Doenças do Tecido Conjuntivo/complicações , Índices de Eritrócitos , Doenças Pulmonares Intersticiais/patologia , Estudos de Casos e Controles , Progressão da Doença , Feminino , Seguimentos , Humanos , Doenças Pulmonares Intersticiais/sangue , Doenças Pulmonares Intersticiais/etiologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Curva ROC , Estudos Retrospectivos , Taxa de SobrevidaRESUMO
The COVID-19 pandemic has created many challenges in the management of immune thrombocytopenic purpura (ITP). The recommendation for avoidance of steroids by WHO led to the off-licence use, supported by NHS England, of thrombopoietin mimetics (TPO-RA) for newly diagnosed or relapsed ITP. This is a real-world prospective study which investigated the treatment patterns and outcomes in this setting. Twenty-four hospitals across the UK submitted 343 cases. Corticosteroids remain the mainstay of ITP treatment, but TPO-RAs were more effective. Incidental COVID-19 infection was identified in a significant number of patients (9·5%), while 14 cases were thought to be secondary to COVID-19 vaccination.
Assuntos
COVID-19/epidemiologia , Pandemias , Púrpura Trombocitopênica Idiopática/terapia , Adolescente , Corticosteroides/administração & dosagem , Corticosteroides/efeitos adversos , Corticosteroides/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Doenças Autoimunes/complicações , COVID-19/sangue , Vacinas contra COVID-19/efeitos adversos , Terapia Combinada , Comorbidade , Doenças do Tecido Conjuntivo/complicações , Contraindicações de Medicamentos , Gerenciamento Clínico , Feminino , Hemorragia/epidemiologia , Hemorragia/etiologia , Hospitais de Distrito/estatística & dados numéricos , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Neoplasias/complicações , Uso Off-Label , Transfusão de Plaquetas , Estudos Prospectivos , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Púrpura Trombocitopênica Idiopática/epidemiologia , Púrpura Trombocitopênica Idiopática/etiologia , Centros de Atenção Terciária/estatística & dados numéricos , Tromboembolia/epidemiologia , Tromboembolia/etiologia , Trombopoetina/agonistas , Ácido Tranexâmico/uso terapêutico , Resultado do Tratamento , Reino Unido/epidemiologia , Adulto JovemAssuntos
COVID-19 , Doenças do Tecido Conjuntivo , Doenças Pulmonares Intersticiais , Idoso , COVID-19/complicações , Doenças do Tecido Conjuntivo/complicações , Feminino , Humanos , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/etiologia , Pessoa de Meia-IdadeRESUMO
OBJECTIVES: The impact of inflammatory rheumatic diseases on COVID-19 severity is poorly known. Here, we compare the outcomes of a cohort of patients with rheumatic diseases with a matched control cohort to identify potential risk factors for severe illness. METHODS: In this comparative cohort study, we identified hospital PCR+COVID-19 rheumatic patients with chronic inflammatory arthritis (IA) or connective tissue diseases (CTDs). Non-rheumatic controls were randomly sampled 1:1 and matched by age, sex and PCR date. The main outcome was severe COVID-19, defined as death, invasive ventilation, intensive care unit admission or serious complications. We assessed the association between the outcome and the potential prognostic variables, adjusted by COVID-19 treatment, using logistic regression. RESULTS: The cohorts were composed of 456 rheumatic and non-rheumatic patients, in equal numbers. Mean age was 63 (IQR 53-78) years and male sex 41% in both cohorts. Rheumatic diseases were IA (60%) and CTD (40%). Most patients (74%) had been hospitalised, and the risk of severe COVID-19 was 31.6% in the rheumatic and 28.1% in the non-rheumatic cohort. Ageing, male sex and previous comorbidity (obesity, diabetes, hypertension, cardiovascular or lung disease) increased the risk in the rheumatic cohort by bivariate analysis. In logistic regression analysis, independent factors associated with severe COVID-19 were increased age (OR 4.83; 95% CI 2.78 to 8.36), male sex (1.93; CI 1.21 to 3.07) and having a CTD (OR 1.82; CI 1.00 to 3.30). CONCLUSION: In hospitalised patients with chronic inflammatory rheumatic diseases, having a CTD but not IA nor previous immunosuppressive therapies was associated with severe COVID-19.