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1.
J Clin Immunol ; 42(7): 1553-1563, 2022 10.
Artículo en Inglés | MEDLINE | ID: mdl-35789314

RESUMEN

PURPOSE: About 20-30% of patients with common variable immunodeficiency (CVID) develop granulomatous-lymphocytic interstitial lung disease (GLILD) as one of several non-infectious complications to their immunodeficiency. The purpose of this study was to identify biomarkers that could distinguish GLILD from other non-infectious complications in CVID. METHODS: We analyzed serum biomarkers related to inflammation, pulmonary epithelium injury, fibrogenesis, and extracellular matrix (ECM) remodeling, and compared three subgroups of CVID: GLILD patients (n = 16), patients with other non-infectious complications (n = 37), and patients with infections only (n = 20). RESULTS: We found that GLILD patients had higher levels of sCD25, sTIM-3, IFN-γ, and TNF, reflecting T cell activation and exhaustion, compared to both CVID patients with other inflammatory complications and CVID with infections only. GLILD patients also had higher levels of SP-D and CC16, proteins related to pulmonary epithelium injury, as well as the ECM remodeling marker MMP-7, than patients with other non-infectious complications. CONCLUSION: GLILD patients have elevated serum markers of T cell activation and exhaustion, pulmonary epithelium injury, and ECM remodeling, pointing to potentially important pathways in GLILD pathogenesis, novel targets for therapy, and promising biomarkers for clinical evaluation of these patients.


Asunto(s)
Inmunodeficiencia Variable Común , Enfermedades Pulmonares Intersticiales , Humanos , Inmunodeficiencia Variable Común/complicaciones , Inmunodeficiencia Variable Común/diagnóstico , Inmunodeficiencia Variable Común/patología , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/etiología , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Biomarcadores , Linfocitos T/patología
2.
Rheumatol Int ; 42(7): 1213-1220, 2022 07.
Artículo en Inglés | MEDLINE | ID: mdl-34984516

RESUMEN

This study aimed at exploring the association between detectable cardiac and pulmonary involvement in long-term juvenile dermatomyositis (JDM) and to assess if patients with cardiac and pulmonary involvement differ with regard to clinical characteristics. 57 JDM patients were examined mean 17.3 (10.5) years after disease onset; this included clinical examination, myositis specific/associated autoantibodies (immunoblot), echocardiography, pulmonary function tests and high-resolution computed tomography. Cardiac involvement was defined as diastolic and/or systolic left ventricular dysfunction and pulmonary involvement as low diffusing capacity for carbon monoxide, low total lung capacity and/or high-resolution computed tomography abnormalities. Patients were stratified into the following four groups: (i) no organ involvement, (ii) pulmonary only, (iii) cardiac only, and (iv) co-existing pulmonary and cardiac involvement. Mean age was 25.7 (12.4) years and 37% were males. One patient had coronary artery disease, seven had a history of pericarditis, seven had hypertension and three had known interstitial lung disease prior to follow-up. There was no association between cardiac (10/57;18%) and pulmonary (41/57;72%) involvement (p = 0.83). After stratifying by organ involvement, 21% of patients had no organ involvement; 61% had pulmonary involvement only; 7% had cardiac involvement only and 11% had co-existing pulmonary or cardiac involvement. Patients with co-existing pulmonary or cardiac involvement had higher disease burden than the remaining patients. Patients with either cardiac or pulmonary involvement only, differed in clinical and autoantibody characteristics. We found no increased risk of developing concomitant cardiac/pulmonary involvement in JDM. Our results shed light upon possible different underlying mechanisms behind pulmonary and cardiac involvement in JDM.


Asunto(s)
Dermatomiositis , Enfermedades Pulmonares Intersticiales , Adulto , Autoanticuerpos , Estudios Transversales , Dermatomiositis/complicaciones , Femenino , Corazón , Humanos , Enfermedades Pulmonares Intersticiales/etiología , Masculino , Pruebas de Función Respiratoria/efectos adversos
3.
Clin Immunol ; 175: 69-74, 2017 02.
Artículo en Inglés | MEDLINE | ID: mdl-27919819

RESUMEN

Patients with common variable immunodeficiency (CVID) constitute a clinically and immunologically heterogeneous group characterized by B-cell dysfunction with hypogammaglobulinemia and defective immunoglobulin class switch of unknown etiology. Current classification systems are insufficient to achieve precise disease management. Characterization of signaling pathways essential for B-cell differentiation and class switch could provide new means to stratify patients. We evaluated constitutive and induced signaling by phospho-specific flow cytometry in 26 CVID patients and 18 healthy blood donors. Strong responses were induced both in CVID and healthy donor B cells upon activation. In contrast, constitutive phosphorylation levels of STAT3,-5,-6, Erk, PLC-γ and Syk were significantly increased in CVID B cells only. Hierarchical clustering revealed a subgroup of CVID patients with elevated constitutive phosphorylation of Syk and PLC-γ. All these patients had non-infectious complications, indicating that a distinct phosphorylation pattern of kinases in B cells identifies a clinically important subgroup of CVID patients.


Asunto(s)
Subgrupos de Linfocitos B/inmunología , Inmunodeficiencia Variable Común/inmunología , Fosforilación/inmunología , Fosfotransferasas/inmunología , Adulto , Anciano , Femenino , Humanos , Cambio de Clase de Inmunoglobulina/inmunología , Activación de Linfocitos/inmunología , Masculino , Persona de Mediana Edad , Receptores de Antígenos de Linfocitos B/inmunología , Transducción de Señal/inmunología , Adulto Joven
4.
J Autoimmun ; 81: 110-119, 2017 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-28476239

RESUMEN

Common variable immunodeficiency (CVID) is defined by hypogammaglobulinemia and B-cell dysfunction, with significant clinical and immunological heterogeneity. Severe non-infectious complications, such as autoimmunity, granulomatous disease and splenomegaly, constitute a major cause of morbidity in CVID patients. T cells are generally regarded important for development of these clinical features. However, while T-cell abnormalities have been found in CVID patients, functional characteristics of T cells corresponding to well-defined clinical subtypes have not been identified. As common γ-chain cytokines play important roles in survival and differentiation of T cells, characterization of their signaling pathways could reveal functional differences of clinical relevance. We characterized CVID T cells functionally by studies of cytokine-induced signaling, and correlated the findings to defined clinical subtypes. Peripheral blood T cells from 29 CVID patients and 19 healthy donors were analyzed for i) phenotype, ii) cytokine-induced (interleukin (IL)-2, IL-4, IL-7 and IL-21) phosphorylation of signal transducer and activator of transcription (STAT) 3, STAT5 and STAT6, and iii) T-helper (Th)1/Th2 polarization. Expression of IL-4 receptor and downstream signaling molecules was measured. A subgroup of CVID patients (n = 7) was identified by impaired IL-4-induced p-STAT6 in naive and memory CD4 and CD8 T cells. This corresponded to patients with the largest accumulation of severe (non-infectious) complications. The signaling defect persisted over years and was not due to constitutively activated p-STAT6. The CD4 T cells were strongly Th1-skewed, but IL-4 signaling was impaired independently of Th status. However, IL-4Rα and Janus kinase (JAK) 1 mRNA levels were significantly lower than in normal donors, providing a likely mechanism for the defective IL-4-induced p-STAT6 and Th1-bias. In conclusion, we identified a subgroup of CVID patients with defective IL-4 signaling in T cells, with severe clinical features of inflammation and autoimmunity.


Asunto(s)
Inmunodeficiencia Variable Común/inmunología , Inmunodeficiencia Variable Común/metabolismo , Interleucina-4/metabolismo , Transducción de Señal , Linfocitos T/inmunología , Linfocitos T/metabolismo , Adulto , Biomarcadores , Inmunodeficiencia Variable Común/complicaciones , Inmunodeficiencia Variable Común/diagnóstico , Femenino , Expresión Génica , Humanos , Inmunofenotipificación , Janus Quinasa 1/metabolismo , Masculino , Persona de Mediana Edad , Receptor de Muerte Celular Programada 1/genética , Receptor de Muerte Celular Programada 1/metabolismo , Factor de Transcripción STAT6/metabolismo , Índice de Severidad de la Enfermedad , Células TH1/inmunología , Células TH1/metabolismo , Células Th2/inmunología , Células Th2/metabolismo
5.
Liver Int ; 37(3): 458-465, 2017 03.
Artículo en Inglés | MEDLINE | ID: mdl-27558072

RESUMEN

BACKGROUND & AIMS: The strongest genetic risk factors in primary sclerosing cholangitis (PSC) are encoded in the HLA complex. Antineutrophil cytoplasmic antibodies (ANCA) have been reported in up to 94% of PSC patients, but their clinical significance and immunogenetic basis are ill defined. We aimed to characterize clinical and genetic associations of ANCA in PSC. METHODS: Antineutrophil cytoplasmic antibodies were analysed with indirect immunofluorescence in 241 Norwegian PSC patients. HLA-B and HLA-DRB1 genotyping was performed in the patients and in 368 healthy controls. Data on perinuclear ANCA (pANCA) and HLA-DRB1 were available from 274 ulcerative colitis (UC) patients without known liver disease. RESULTS: Antineutrophil cytoplasmic antibodies were found in 193 (80%) of the PSC patients, with pANCA in 169 (70%). ANCA-positive patients were younger than ANCA negative at diagnosis of PSC and had a lower frequency of biliary cancer (9% vs 19%, P=.047). There were no differences between PSC patients with and without inflammatory bowel disease. Genetically, the strong PSC risk factors HLA-B*08 (frequency in healthy 13%) and DRB1*03 (14%) were more prevalent in ANCA-positive than -negative patients (43% vs 25%, P=.0012 and 43% vs 25%, P=.0015 respectively). The results were similar when restricting the analysis to pANCA-positive patients. In UC patients without liver disease, HLA-DRB1*03 was more prevalent in pANCA-positive compared with -negative patients (P=.03). CONCLUSIONS: Antineutrophil cytoplasmic antibodies identified PSC patients with particular clinical and genetic characteristics, suggesting that ANCA may mark a clinically relevant pathogenetic subgroup in the PSC-UC disease spectrum.


Asunto(s)
Anticuerpos Anticitoplasma de Neutrófilos/sangre , Colangitis Esclerosante/sangre , Colangitis Esclerosante/genética , Antígeno HLA-B27/genética , Cadenas HLA-DRB1/genética , Adolescente , Adulto , Anciano , Biomarcadores , Estudios de Casos y Controles , Estudios Transversales , Bases de Datos Factuales , Femenino , Técnica del Anticuerpo Fluorescente Indirecta , Genotipo , Humanos , Enfermedades Inflamatorias del Intestino/genética , Modelos Logísticos , Masculino , Persona de Mediana Edad , Noruega , Adulto Joven
6.
Clin Med Res ; 12(1-2): 27-32, 2014 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-24415744

RESUMEN

BACKGROUND: Liver transplantation regularly requires transfusion of red blood cells (RBCs), plasma, and platelets. Compared to fresh frozen plasma (FFP) from single blood donors, solvent/detergent-treated plasma (SD-plasma) pooled from several hundred blood donors has advantages with respect to pathogen reduction, standardized content of plasma proteins, and significantly reduced risk of transfusion related lung injury and allergic/immunologic adverse reactions. However, SD-plasma has been suspected to increase the incidence of hyperfibrinolysis and thromboembolic events. STUDY DESIGN AND METHODS: We investigated the transfusion practices, hyperfibrinolysis parameters, and thrombosis outcomes in 195 consecutive adult primary liver transplants in our center using SD-plasma (Octaplas) as the exclusive source of plasma. RESULTS: Perioperatively, median (interquartile range) 4 (1 to 9) RBC-units, 10 (4 to 18) plasma-bags, and 0 (0 to 2) platelet-units were transfused. Hyperfibrinolysis defined as LY30 ≤ 7.5% was detected in 12/138 thrombelastography-monitored patients (9%). These patients received significantly more RBCs, plasma, and platelets than did patients without hyperfibrinolysis. Thrombotic graft complications were observed in three patients (2%). Pulmonary embolism was not observed in any patient. CONCLUSION: SD-plasma is a safe plasma product for liver transplant recipients, and the incidences of hyperfibrinolysis and thromboembolic events are not significantly different from those seen in centers using FFP.


Asunto(s)
Transfusión de Componentes Sanguíneos/métodos , Fibrinólisis , Trastornos Hemorrágicos/epidemiología , Trasplante de Hígado/métodos , Plasma , Complicaciones Posoperatorias/epidemiología , Tromboembolia/epidemiología , Adulto , Anciano , Detergentes , Humanos , Persona de Mediana Edad , Noruega , Solventes , Tromboelastografía , Adulto Joven
8.
Tidsskr Nor Laegeforen ; 138(6)2018 03 20.
Artículo en Noruego | MEDLINE | ID: mdl-29557140
9.
Nat Biotechnol ; 40(4): 488-498, 2022 04.
Artículo en Inglés | MEDLINE | ID: mdl-34873326

RESUMEN

Unlike chimeric antigen receptors, T-cell receptors (TCRs) can recognize intracellular targets presented on human leukocyte antigen (HLA) molecules. Here we demonstrate that T cells expressing TCRs specific for peptides from the intracellular lymphoid-specific enzyme terminal deoxynucleotidyl transferase (TdT), presented in the context of HLA-A*02:01, specifically eliminate primary acute lymphoblastic leukemia (ALL) cells of T- and B-cell origin in vitro and in three mouse models of disseminated B-ALL. By contrast, the treatment spares normal peripheral T- and B-cell repertoires and normal myeloid cells in vitro, and in vivo in humanized mice. TdT is an attractive cancer target as it is highly and homogeneously expressed in 80-94% of B- and T-ALLs, but only transiently expressed during normal lymphoid differentiation, limiting on-target toxicity of TdT-specific T cells. TCR-modified T cells targeting TdT may be a promising immunotherapy for B-ALL and T-ALL that preserves normal lymphocytes.


Asunto(s)
ADN Nucleotidilexotransferasa , Linfocitos T , Animales , Células Madre Hematopoyéticas , Linfocitos , Ratones , Receptores de Antígenos de Linfocitos T/genética
10.
N Engl J Med ; 355(12): 1199-209, 2006 Sep 21.
Artículo en Inglés | MEDLINE | ID: mdl-16990383

RESUMEN

BACKGROUND: Previous studies have shown improvement in left ventricular function after intracoronary injection of autologous cells derived from bone marrow (BMC) in the acute phase of myocardial infarction. We designed a randomized, controlled trial to further investigate the effects of this treatment. METHODS: Patients with acute ST-elevation myocardial infarction of the anterior wall treated with percutaneous coronary intervention were randomly assigned to the group that underwent intracoronary injection of autologous mononuclear BMC or to the control group, in which neither aspiration nor sham injection was performed. Left ventricular function was assessed with the use of electrocardiogram-gated single-photon-emission computed tomography (SPECT) and echocardiography at baseline and magnetic resonance imaging (MRI) 2 to 3 weeks after the infarction. These procedures were repeated 6 months after the infarction. End points were changes in the left ventricular ejection fraction (LVEF), end-diastolic volume, and infarct size. RESULTS: Of the 50 patients assigned to treatment with mononuclear BMC, 47 underwent intracoronary injection of the cells at a median of 6 days after myocardial infarction. There were 50 patients in the control group. The mean (+/-SD) change in LVEF, measured with the use of SPECT, between baseline and 6 months after infarction for all patients was 7.6+/-10.4 percentage points. The effect of BMC treatment on the change in LVEF was an increase of 0.6 percentage point (95% confidence interval [CI], -3.4 to 4.6; P=0.77) on SPECT, an increase of 0.6 percentage point (95% CI, -2.6 to 3.8; P=0.70) on echocardiography, and a decrease of 3.0 percentage points (95% CI, 0.1 to -6.1; P=0.054) on MRI. The two groups did not differ significantly in changes in left ventricular end-diastolic volume or infarct size and had similar rates of adverse events. CONCLUSIONS: With the methods used, we found no effects of intracoronary injection of autologous mononuclear BMC on global left ventricular function.


Asunto(s)
Trasplante de Médula Ósea , Infarto del Miocardio/terapia , Trasplante de Médula Ósea/efectos adversos , Trasplante de Médula Ósea/métodos , Vasos Coronarios , Técnicas de Diagnóstico Cardiovascular , Femenino , Humanos , Inyecciones , Masculino , Persona de Mediana Edad , Infarto del Miocardio/fisiopatología , Volumen Sistólico , Trasplante Autólogo , Insuficiencia del Tratamiento , Función Ventricular Izquierda
11.
Scand J Clin Lab Invest ; 69(1): 79-84, 2009.
Artículo en Inglés | MEDLINE | ID: mdl-18836945

RESUMEN

OBJECTIVE: Phenotypical changes in the human bone marrow (BM) due to age and stress have not so far been properly addressed in the literature. In the present study, we compared CD34(+) BM cells between older and young volunteers. The influence of stress on CD34(+) cell phenotype in older patients was investigated in an age-matched group with acute myocardial infarction (AMI). Cytokines thought to influence BM CD34(+) cell homeostasis were also analysed. MATERIAL AND METHODS: BM mononuclear cells of 10 older volunteers and of 7 young volunteers (18-25 years), as well as 22 AMI patients, were analysed by flow cytometry for the following markers: CD34, CD38, CD117 (c-kit) and CD133. Blood samples were analysed for CRP, IL-6, MCP-1, IL-8, MMP-9, TIMP-1 and TNFalpha by ELISA methods. RESULTS: Significantly higher numbers of CD34(+) CD38(-) cells (both absolute and relative) were observed in older volunteers than in young volunteers and AMI patients. Higher numbers of immature progenitors, namely CD34(+)CD38(-) cells and CD34(+)CD38(-)CD117(+)CD133(+) cells, were observed among older volunteers compared to the other groups. However, the relative number of CD34(+) cells lacking CD38 expression or expressing CD133 was higher in the old volunteers and AMI patients. None of the circulating factors investigated correlated with any of the cell population yields. CONCLUSION: In this study, we found that the absolute and relative numbers of BM CD34(+)CD38(-) progenitor cells increase with age. The increment is attenuated in patients with AMI.


Asunto(s)
Envejecimiento/patología , Antígenos CD34/metabolismo , Células de la Médula Ósea/patología , Estrés Fisiológico , ADP-Ribosil Ciclasa 1/metabolismo , Proteínas de Fase Aguda/análisis , Adolescente , Adulto , Distribución por Edad , Anciano , Proteína C-Reactiva/análisis , Recuento de Células , Citocinas/sangre , Femenino , Citometría de Flujo , Humanos , Masculino , Persona de Mediana Edad , Infarto del Miocardio/sangre , Infarto del Miocardio/patología , Fenotipo
12.
Tidsskr Nor Laegeforen ; 129(16): 1631-4, 2009 Aug 27.
Artículo en Noruego | MEDLINE | ID: mdl-19721478

RESUMEN

BACKGROUND: Myositis-specific antibodies (MSA) are autoantibodies that are almost exclusively detected in idiopathic inflammatory myopathies (IIM). This article provides an overview of these autoantibodies and how they can be used clinically to identify subgroups of IIM. MATERIAL AND METHODS: The article is based on a non-systematic literature review and our own experience. RESULTS: MSA can be detected in up to 50 % of patients with IIM. Patients with anti-synthetase antibodies have a constellation of clinical findings termed "the anti-synthetase syndrome", in which interstitial lung disease dominates the clinical picture. Anti-Mi2 antibodies is another myositis-specific antibody. Patients with anti-Mi2 antibodies often have classical dermatomyositis, while the anti-SRP antibody identifies patients with severe myopathy, poor response to treatment with corticosteroids and histological findings of muscle cell necrosis - often lacking inflammatory infiltrates. The newly detected anti-CADMp140 appears to be associated with amyopathic or hypomyopathic dermatomyositis, previously called dermatomyositis sine myositis. Anti-p155 antibodies are most often found in patients who also have cancer. INTERPRETATION: Myositis-specific antibodies may be useful for identification of clinical subgroups of IIM and can thereby affect the choice of medical treatment.


Asunto(s)
Autoanticuerpos/análisis , Miositis/inmunología , Aminoacil-ARNt Sintetasas/inmunología , Antiinflamatorios/uso terapéutico , Dermatomiositis/inmunología , Progresión de la Enfermedad , Humanos , Inmunosupresores/uso terapéutico , Péptidos y Proteínas de Señalización Intercelular , Miositis/clasificación , Miositis/tratamiento farmacológico , Proteínas Nucleares/inmunología , Péptidos/inmunología , Partícula de Reconocimiento de Señal/inmunología
13.
J Rheumatol ; 44(4): 459-465, 2017 04.
Artículo en Inglés | MEDLINE | ID: mdl-28089974

RESUMEN

OBJECTIVE: Extensive skin disease and renal crisis are hallmarks of anti-RNA polymerase III (RNAP)-positive systemic sclerosis (SSc), while lung and heart involvement data are conflicting. Here, the aims were to perform time-course analyses of interstitial lung disease (ILD) and pulmonary hypertension (PH) in the RNAP subset of a prospective unselected SSc cohort and to use the other autoantibody subsets as comparators. METHODS: The study cohort included 279 patients with SSc from the observational Oslo University Hospital cohort with complete data on (1) SSc-related autoantibodies, (2) paired, serial analyses of lung function and fibrosis by computed tomography, and (3) PH verified by right heart catheterization. RESULTS: RNAP was positive in 33 patients (12%), 79% of which had diffuse cutaneous SSc. Pulmonary findings were heterogeneous; 49% had no signs of fibrosis while 18% had > 20% fibrosis at followup. Forced vital capacity at followup was < 80% in 39% of the RNAP subset, comparable to the antitopoisomerase subset (ATA; 47%), but higher than anticentromere (ACA; 13%). Accumulated frequency of PH in the RNAP subset (12%) was lower than in ACA (18%). At 93% and 78%, the 5- and 10-year survival rates in RNAP were comparable to the ATA and ACA subsets. CONCLUSION: In this cohort, the RNAP subset was marked by cardiopulmonary heterogeneity, ranging from mild ILD to development of severe ILD in 18%, and PH development in 12%. These data indicate that cardiopulmonary risk stratification early in the disease course is particularly important in RNAP-positive SSc.


Asunto(s)
Autoanticuerpos/inmunología , Enfermedades Cardiovasculares/complicaciones , Enfermedades Pulmonares/complicaciones , ARN Polimerasa III/inmunología , Esclerodermia Sistémica/complicaciones , Adulto , Anciano , Enfermedades Cardiovasculares/diagnóstico por imagen , Enfermedades Cardiovasculares/inmunología , Enfermedades Cardiovasculares/mortalidad , Estudios de Cohortes , Progresión de la Enfermedad , Femenino , Humanos , Enfermedades Pulmonares/diagnóstico por imagen , Enfermedades Pulmonares/inmunología , Enfermedades Pulmonares/mortalidad , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Prospectivos , Esclerodermia Sistémica/diagnóstico por imagen , Esclerodermia Sistémica/inmunología , Esclerodermia Sistémica/mortalidad , Índice de Severidad de la Enfermedad , Tasa de Supervivencia , Tomografía Computarizada por Rayos X
14.
Arthritis Res Ther ; 19(1): 284, 2017 12 21.
Artículo en Inglés | MEDLINE | ID: mdl-29268795

RESUMEN

BACKGROUND: The phenotypic stability of mixed connective tissue disease (MCTD) is not clear, and knowledge about disease activity and remission is scarce. We aimed to establish the occurrence of evolution from MCTD to another defined rheumatic condition, and the prevalence and durability of remission after long-term observation. METHODS: In this large population-based prospective observational MCTD cohort study (N = 118), disease conversion was defined by the development of new auto-antibodies and clinical features compliant with another well-defined rheumatic condition. Remission was defined by a combination of systemic lupus erythematosus disease activity index 2000 (SLEDAI-2 K) of 0 and European League Against Rheumatism scleroderma trials and research (EUSTAR) activity index <2.5. Predictors of phenotypic stability and disease remission were assessed by logistic regression. RESULTS: Among 118 patients, 14 (12%) developed another well-defined rheumatic condition other than MCTD after mean disease duration of 17 (SD 9) years. Puffy hands predicted a stable MCTD phenotype in univariable regression analysis (OR 7, CI 2-27, P = .010). Disease activity defined by SLEDAI-2 K, decreased gradually across the observation period and > 90% of patients had EUSTAR activity index <2.5. There were 13% patients in remission throughout the whole mean observation period of 7 (SD 2) years. The strongest predictor of remission was percentage of predicted higher forced vital capacity. CONCLUSIONS: Our results strengthen the view of MCTD as a relatively stable disease entity. Long-term remission in MCTD is not frequent; however, the low SLEDAI-2 K and EUSTAR scores during the observation period suggests that the disease runs a milder course than systemic lupus erythematosus and systemic sclerosis.


Asunto(s)
Enfermedad Mixta del Tejido Conjuntivo/patología , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Enfermedad Mixta del Tejido Conjuntivo/epidemiología , Estudios Prospectivos , Enfermedades Reumáticas/epidemiología
15.
Sci Rep ; 7: 39710, 2017 01 05.
Artículo en Inglés | MEDLINE | ID: mdl-28054583

RESUMEN

Common variable immunodeficiency (CVID) is a heterogeneous group of diseases. Our aim was to define sub-groups of CVID patients with similar phenotypes and clinical characteristics. Using eight-color flow cytometry, we analyzed both B- and T-cell phenotypes in a cohort of 88 CVID patients and 48 healthy donors. A hierarchical clustering of probability binning "bins" yielded a separate cluster of 22 CVID patients with an abnormal phenotype. We showed coordinated proportional changes in naïve CD4+ T-cells (decreased), intermediate CD27- CD28+ CD4+ T-cells (increased) and CD21low B-cells (increased) that were stable for over three years. Moreover, the lymphocytes' immunophenotype in this patient cluster exhibited features of profound immunosenescence and chronic activation. Thrombocytopenia was only found in this cluster (36% of cases, manifested as Immune Thrombocytopenia (ITP) or Evans syndrome). Clinical complications more frequently found in these patients include lung fibrosis (in 59% of cases) and bronchiectasis (55%). The degree of severity of these symptoms corresponded to more deviation from normal levels with respect to CD21low B-cells, naïve CD4+ and CD27− CD28+ CD4+ T-cells. Next-generation sequencing did not reveal any common genetic background. We delineate a subgroup of CVID patients with activated and immunosenescent immunophenotype of lymphocytes and distinct set of clinical complications without common genetic background.


Asunto(s)
Linfocitos B/inmunología , Linfocitos T CD4-Positivos/inmunología , Inmunodeficiencia Variable Común/inmunología , Pulmón/patología , Púrpura Trombocitopénica Idiopática/inmunología , Adolescente , Adulto , Anciano , Separación Celular , Estudios de Cohortes , Femenino , Fibrosis , Citometría de Flujo , Humanos , Inmunosenescencia , Activación de Linfocitos , Masculino , Persona de Mediana Edad , Fenotipo , Adulto Joven
16.
Tidsskr Nor Laegeforen ; 123(17): 2439-42, 2003 Sep 11.
Artículo en Noruego | MEDLINE | ID: mdl-14594145

RESUMEN

BACKGROUND: This is the first survey of therapeutic hemapheresis in Norway. The purpose was to collect data from all Norwegian dialysis and blood units performing hemapheresis treatment and stem cell collection for comparison with Swedish data. MATERIALS AND METHODS: Questionnaires were sent to all regional and central hospitals in Norway including two specialized centres for stem cell apheresis. The questions included the number of hemapheresis procedures, patients, and indications. RESULTS: All units responded to the questionnaire. There were 17 dialysis units; seven blood units and two special units who performed 2,141 procedures. 12 units did not carry out any hemapheresis treatment at all. There were five stem cell collections per 100,000 inhabitants and 42.5 therapeutic apheresis procedures per 100,000 inhabitants. INTERPRETATION: Hemapheresis treatment is performed in all Norwegian health regions. The indications are quite similar to those reported from Sweden except for stem cell collections and erythrocytaphereses. The dominance of the dialysis units in the total number of procedures performed and the prominent use of filtration techniques are special features of the Norwegian practice of apheresis.


Asunto(s)
Eliminación de Componentes Sanguíneos , Eliminación de Componentes Sanguíneos/efectos adversos , Eliminación de Componentes Sanguíneos/métodos , Eliminación de Componentes Sanguíneos/estadística & datos numéricos , Citaféresis/métodos , Citaféresis/estadística & datos numéricos , Humanos , Noruega , Intercambio Plasmático/efectos adversos , Intercambio Plasmático/métodos , Intercambio Plasmático/estadística & datos numéricos , Plasmaféresis/efectos adversos , Plasmaféresis/métodos , Plasmaféresis/estadística & datos numéricos , Pautas de la Práctica en Medicina , Encuestas y Cuestionarios , Suecia
19.
Arthritis Care Res (Hoboken) ; 62(8): 1103-11, 2010 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-20506141

RESUMEN

OBJECTIVE: To compare muscle strength, physical health, and HLA-DRB1 allele carriage frequencies in patients with longstanding juvenile dermatomyositis (DM) with that of controls, and to determine the presence of and risk factors for muscle weakness and magnetic resonance imaging (MRI)-detected muscle damage in juvenile DM patients. METHODS: Fifty-nine patients with juvenile DM examined a median of 16.8 years (range 2.0-38.1 years) after disease onset were compared with 59 age- and sex-matched controls. Muscle strength/endurance was measured by manual muscle testing (MMT) and the Childhood Myositis Assessment Scale (CMAS); health status was measured by the Short Form 36. HLA-DRB1 alleles were determined by sequencing in patients and 898 healthy controls. In patients, disease activity/damage was measured by the Disease Activity Score (DAS), Myositis Damage Index (MDI), Health Assessment Questionnaire/Childhood Health Assessment Questionnaire, and MRI scans of the thigh muscles. Early disease characteristics were obtained by chart review. RESULTS: Patients had lower muscle strength/endurance (P < 0.001 for both) and physical health (P = 0.014) and increased HLA-DRB1*0301 (P = 0.01) and DRB1*1401 (P = 0.003) compared with controls. In patients, persistent muscle weakness was found in 42% with MMT (score <78) and in 31% with the CMAS (score <48), whereas MRI-detected muscle damage was found in 52%. Muscle weakness and MRI-detected muscle damage were predicted by MDI muscle damage and a high DAS 1 year postdiagnosis. CONCLUSION: A median of 16.8 years after disease onset, juvenile DM patients were weaker than the controls; muscle weakness/reduced endurance was found in 31-42% of patients and MRI-detected muscular damage was found in 52% of patients. The outcomes were predicted by high disease activity and muscle damage present 1 year postdiagnosis.


Asunto(s)
Dermatomiositis/diagnóstico , Estado de Salud , Miositis/diagnóstico , Estudios de Casos y Controles , Niño , Preescolar , Estudios de Cohortes , Susceptibilidad a Enfermedades , Femenino , Antígenos HLA-DR/genética , Cadenas HLA-DRB1 , Humanos , Masculino , Actividad Motora , Fuerza Muscular , Debilidad Muscular/complicaciones , Músculo Esquelético/patología , Músculo Esquelético/fisiopatología , Factores de Riesgo , Piel/patología , Resultado del Tratamiento
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