RESUMEN
BACKGROUND: Whether patients with autoimmune rheumatic diseases (ARD) have a higher risk for SARS-CoV-2 infection (COVID-19) and how SARS-CoV-2 pandemic impacts on adherence to therapy has not been fully elucidated. We assessed the rate and clinical presentation of COVID-19, and adherence to therapy in a large cohort of patients with ARD followed-up in a tertiary University-Hospital in Northeast Italy. METHODS: Between April 9th and April 25th, 2020, after SARS-CoV-2 infection peak, a telephone survey investigating the impact of COVID-19 on patients with systemic lupus erythematosus (SLE), systemic sclerosis (SSc), rheumatoid arthritis (RA), ANCA-associated vasculitis (AAV), and idiopathic inflammatory myopathies (IIM) was administered. Demographics, disease activity status, therapy, occupational exposure, and adherence to social distancing advise were also collected. RESULTS: 916 patients (397 SLE, 182 AAV, 176 SSc, 111 RA, 50 IIM) completed the survey. 148 patients developed at least one symptom compatible with COVID-19 (cough 96, sore throat 64, fever 64, arthromyalgias 59, diarrhea 26, conjunctivitis 18, ageusia/hyposmia, 18). Among the 916 patients, 65 (7.1%) underwent SARS-CoV-2 nasopharyngeal swab (18 symptomatic and 47 asymptomatic), 2 (0.21%) tested positive, a proportion similar to that observed in the general population of the Veneto region. No deaths occurred. 31 patients (3.4%) withdrew ≥1 medication, mainly immunosuppressants or biologics. Adoption of social distancing was observed by 860 patients (93.9%), including 335 (36.6%) who adopted it before official lockdown. CONCLUSIONS: COVID-19 incidence seems to be similar in our cohort compared to the general population. Adherence to therapy and to social distancing advise was high.
Asunto(s)
Enfermedades Autoinmunes/tratamiento farmacológico , Betacoronavirus , Infecciones por Coronavirus/tratamiento farmacológico , Inmunosupresores/administración & dosificación , Neumonía Viral/tratamiento farmacológico , Enfermedades Reumáticas/tratamiento farmacológico , Adulto , Anciano , Enfermedades Autoinmunes/diagnóstico , Enfermedades Autoinmunes/virología , COVID-19 , Infecciones por Coronavirus/diagnóstico , Infecciones por Coronavirus/patología , Femenino , Humanos , Italia , Masculino , Persona de Mediana Edad , Pandemias , Neumonía Viral/diagnóstico , Neumonía Viral/patología , Enfermedades Reumáticas/diagnóstico , Enfermedades Reumáticas/virología , SARS-CoV-2RESUMEN
The objective was to study both ex vivo and in vitro secretion of pro-inflammatory cytokines in patients affected by Blau syndrome (BS) and carrying p.E383K mutation in the CARD15/NOD2 gene associated with the disease. For ex vivo studies, peripheral blood mononuclear cells (PBMCs), serum from three patients and healthy controls have been collected. PBMCs have been cultured in the presence or absence of inflammatory enhancers, such as lipopolysaccharide (LPS) and muramyl dipeptide (MDP). The levels of interleukin (IL)-1ß, IL-6, IL-8, tumor necrosis factor (TNF)-α and interferon (IFN)-γ were assayed by either immunoassay or array-based system. For in vitro studies, different constructs were created cloning human wild-type and p.E383K-mutated NOD2 cDNA into the expression vector pCMV-Tag2c. HEK293 cell lines were stably transfected, cultured with or without MDP and IL-8 level was assayed in their surnatants. Statistical analysis in both studies was performed using non-parametric tests. Both ex vivo and in vitro studies have not identified a significant increase in secretion of the analyzed proinflammatory cytokines. p.E383K-mutated NOD2 transfected cells express low level of IL-8. The ex vivo basal level results from both serum and PBMCs surnatants present similar levels of IL-1ß, IL-6, TNF-α and IFN-γ in patients and controls. The presence of the stimulant agents (LPS and MDP), either individual or paired, does not lead to significant increases in all cytokines concentrations in patients compared to controls. Taken together, the ex vivo and in vitro data suggest that there is not a primary mediation of IL-1ß and other pro-inflammatory cytokines in BS patients carrying p.E383K.
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Artritis/inmunología , Citocinas/sangre , Sinovitis/inmunología , Uveítis/inmunología , Adulto , Artritis/sangre , Artritis/genética , Biomarcadores/sangre , Estudios de Casos y Controles , Extremidades/patología , Padre , Femenino , Humanos , Técnicas In Vitro , Interferón gamma/sangre , Interleucina-1beta/sangre , Interleucina-6/sangre , Interleucina-8/sangre , Masculino , Persona de Mediana Edad , Mutación , Proteína Adaptadora de Señalización NOD2/genética , Núcleo Familiar , Linaje , Sarcoidosis , Sinovitis/sangre , Sinovitis/genética , Factor de Necrosis Tumoral alfa/sangre , Uveítis/sangre , Uveítis/genéticaRESUMEN
OBJECTIVES: To delineate the molecular mechanisms underlying the process of the diffuse-type giant cell tumours, also called pigmented villonodular synovitis, a rare, aggressive condition of the synovium, the knee synovial tissue expression of colony-stimulating factor-1 gene, as detected by real-time polymerase chain reaction, was compared between patients affected with pigmented villonodular knee synovitis and knee meniscal tears, or persistent gonoarthitis. METHODS: Multiple synovial biopsies of the knee were performed by arthroscopy in five consecutive patients affected by diffuse pigmented villonodular knee synovitis and in 12 patients affected by knee meniscal tears (n. 6) or persistent active gonarthritis (n. 6), recruited from the patients attending the Rheumatology Day Surgery Outpatient Clinic of the University of Padova Hospital. The ethics committee approved the study protocol and the participants signed consent statements after being informed about the content of the study. The diagnosis was made on the basis of a histological examination. The colony-stimulating factor-1 gene expression was assessed by reverse transcription followed by real-time polymerase chain reaction. RESULTS: The detection by RT-PCR of synovial colony-stimulating factor-1 mRNA showed a wide spectrum of expression in the three groups of distinct knee joint disease affected patients, with significantly higher level of colony-stimulating factor-1 mRNA expression in synovial tissue of pigmented villonodular synovitis, in comparison to that of knee meniscal injuries and persistent gonoarthritis patients. CONCLUSIONS: Our findings point out to an important role of colony-stimulating factor-1 in pigmented villonodular knee synovitis disease process and support the idea that colony-stimulating factor-1/colony-stimulating factor-1 receptor interaction may represent a potential therapeutic target of this disease.
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Factor Estimulante de Colonias de Macrófagos/metabolismo , ARN Mensajero/metabolismo , Membrana Sinovial/metabolismo , Sinovitis Pigmentada Vellonodular/metabolismo , Adulto , Artritis/metabolismo , Artritis/patología , Biomarcadores/metabolismo , Biopsia , Femenino , Regulación de la Expresión Génica , Humanos , Masculino , Meniscos Tibiales/metabolismo , Meniscos Tibiales/patología , Persona de Mediana Edad , Membrana Sinovial/patología , Sinovitis Pigmentada Vellonodular/patología , Lesiones de Menisco TibialRESUMEN
OBJECTIVES: To evaluate whether high density lipoproteins (HDL) affect monosodium urate (MSU) crystal-induced inflammation in the murine air pouch model. METHODS: MSU crystals were prepared by Denko's method and sterilized by heating at 180°C for 2 h before each experiment. Human HDL were isolated from peripheral blood of healthy volunteers. MSU crystals (2 mg in 1 ml of PBS) were injected into subcutaneous air pouches in mice in the presence or absence of HDL (0.1 mg). Negative control pouches received 1 ml of PBS. To recover pouch fluid, the pouches were washed with 2 ml of PBS after the animals were sacrificed. The leukocyte count in the lavage fluids was obtained using a hemocytometer and differential leukocyte count was determined by May-Grunwald-Giemsa staining. IL-6, KC, CCL2 and TNF-α levels were measured in exudates by ELISA. RESULTS: MSU crystals increased the number of leukocytes and the neutrophil migration, as well as the concentrations of IL-6, KC and CCL2 in pouch fluids, while the TNF-α levels were not detectable. The treatment with HDL led to a reduction in all inflammatory parameters: the leukocyte count decreased by 73%; the neutrophil density decreased by 35%; the IL-6, KC and CCL2 concentration decreased by 4-, 6- and 5-fold respectively. CONCLUSIONS: This study shows that HDL may limit the inflammatory process by inhibiting leukocyte recruitment and cytokine release. HDL are likely to represent a mechanism of control of crystal-induced inflammation.
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Citocinas/antagonistas & inhibidores , Citocinas/biosíntesis , Modelos Animales de Enfermedad , Inflamación/inmunología , Lipoproteínas HDL/farmacología , Animales , Masculino , Ratones , Ácido Úrico/administración & dosificaciónRESUMEN
The recent outbreak of coronavirus disease (COVID 19), spreading from China all around the world in early 2020, has led scientists to investigate the immuno-mediated mechanisms underlying the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV2) infection. Depending on the amount of cytokines released as the result of the immunological activation induced by SARS-CoV2, three major clinical phenotypes can be identified: "mild",symbolized as a "drizzle" of cytokines, severe as a "storm", and critical as a "hurricane". In patients with mild symptoms, the release of pro-inflammatory cytokines is balanced to obtain a defense response against the virus which is often self-limiting and overcomes without tissue damage. In severe phenotype, resembling a "cytokine-release syndrome", SARS-CoV2 causes the lysis of the immune-mediators leading to a cytokine storm able to induce lung epithelium damage and acute respiratory distress syndrome. In critical patients, the immune response may become uncontrolled, thus the cytokine burst resembles a form of secondary hemophagocytic lymphohistiocytosis which may result in a multi organ failure. In addition to the standard of care, an immune-modulatory therapy tailored to each one of the different phenotypes should be used in order to prevent or reduce the release of cytokines responsible for organ damage and disease progression.
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Lesión Pulmonar Aguda/patología , Infecciones por Coronavirus/patología , Síndrome de Liberación de Citoquinas/patología , Citocinas/sangre , Neumonía Viral/patología , Lesión Pulmonar Aguda/inmunología , Betacoronavirus/inmunología , COVID-19 , Infecciones por Coronavirus/inmunología , Humanos , Linfohistiocitosis Hemofagocítica/patología , Linfopenia/patología , Pandemias , Neumonía Viral/inmunología , Síndrome de Dificultad Respiratoria/inmunología , Síndrome de Dificultad Respiratoria/patología , SARS-CoV-2RESUMEN
OBJECTIVE: To investigate lipid and apolipoprotein (Apo) levels in synovial fluid (SF) and serum of patients with rheumatoid arthritis (RA), psoriatic arthritis (PsA) and osteoarthritis (OA). METHODS: SF of 44 patients (14 RA, 14 PsA, 16 OA) was tested for Apo A-I, HDL-C, total cholesterol (TC), IL-1Beta, TNF-alpha, white blood cell count (WBC) and polymorphonucleate (PMN) percentage. Blood samples, collected simultaneously to the SF, were examined for Apo A-I, HDL-C, TC, TNF-alpha, serum amyloid A (SAA) and C-reactive protein (CRP). Thirty-three healthy donors served as a control group. RESULTS: Serum levels of Apo A-I, HDL-C and TC were higher in OA as compared with RA, PsA and the control group. The patients with inflammatory arthritis had lower serum levels of Apo A-I and HDL-C than did the controls. Apo A-I concentrations were higher in SF of RA patients, while PsA showed the highest concentration of TC, though not reaching statistical significance. A negative correlation was found between serum Apo A-I and synovial WBC (r=-0.48 p=0.002) and IL-1Beta (r=-0.42 p=0.016). There was a strong positive correlation between the Apo A-I SF/serum ratio and synovial WBC (r=0.73 p<0.001), IL-1Beta (r=0.68 p<0.001) and a weak, yet significant, correlation with serum CRP (r=0.49 p=0.002) and SAA (r=0.41 p=0.008). CONCLUSION: Our study confirms that in RA Apo A-I and TC levels are decreased in plasma and increased in SF, thus suggesting infiltration of HDL particles in the inflamed joint with inhibition of the local production of proinflammatory cytokines. On the other hand, it can be hypothesized that the sequestration of Apo A-I in the inflamed tissue may, in part, account for the reduction of circulating HDL and the excess cardiovascular risk in RA and PsA patients.
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Apolipoproteína A-I/metabolismo , Artritis/metabolismo , Colesterol/metabolismo , Articulación de la Rodilla/metabolismo , Líquido Sinovial/metabolismo , Adulto , Anciano , Apolipoproteína A-I/sangre , Artritis/inmunología , Artritis Psoriásica/inmunología , Artritis Psoriásica/metabolismo , Proteína C-Reactiva/metabolismo , Colesterol/sangre , HDL-Colesterol/sangre , Estudios de Cohortes , Femenino , Humanos , Articulación de la Rodilla/inmunología , Masculino , Persona de Mediana Edad , Osteoartritis/inmunología , Osteoartritis/metabolismo , Líquido Sinovial/inmunologíaRESUMEN
MonitorNet is a database established by the Italian Society of Rheumatology (SIR) in January 2007 and funded by the Italian Medicines Agency (AIFA), for the active long-term follow-up of patients with rheumatoid arthritis, psoriatic arthritis and ankylosing spondylitis treated with biologic agents. All hospital Rheumatology Units in Italy were invited to participate in a non-interventional, observational, epidemiological study. The study is conducted in a routine clinical setting (real-world practice) where biologics are prescribed on the basis of current recommendations. In this report we describe the design, methodology, and present preliminary data of the study. At the time of the analysis (April 2009) the database included 3510 patients: 2469 (70.3%) with established RA, 675 (19.2%) with PsA and 366 (10.4%) with AS. The cumulative follow up period was 8,787 patient-years (RA: 8,388, PsA: 157; AS: 242). There were 1,538 adverse events in 938 (26.7%) patients. Infections were recorded in 630 patients, skin-related adverse events in 142 and post-infusion reactions in 90. A total of 30 malignancies were reported. An interim analysis of efficacy was conducted on 2,148 RA patients. Seven hundred and thirty-one patients (35.8%) achieved EULAR remission (defined as DAS28<2.4). When assessed with the more restrictive CDAI and SDAI criteria, the frequency of remission was lower (17.9% and 14.7% respectively). Availability of funding for this study provided an opportunity to organize a collaborative national network of rheumatology clinics to develop a large multicentre observational study.
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Antirreumáticos/uso terapéutico , Productos Biológicos/uso terapéutico , Inmunosupresores/uso terapéutico , Enfermedades Reumáticas/tratamiento farmacológico , Reumatología , Medición de Riesgo , Factor de Necrosis Tumoral alfa/antagonistas & inhibidores , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Antirreumáticos/efectos adversos , Artritis Psoriásica/tratamiento farmacológico , Artritis Reumatoide/tratamiento farmacológico , Productos Biológicos/efectos adversos , Consenso , Quimioterapia Combinada , Femenino , Estudios de Seguimiento , Humanos , Inmunosupresores/efectos adversos , Italia , Masculino , Registros Médicos , Persona de Mediana Edad , Inducción de Remisión , Reproducibilidad de los Resultados , Enfermedades Reumáticas/diagnóstico , Índice de Severidad de la Enfermedad , Sociedades Médicas , Espondilitis Anquilosante/tratamiento farmacológico , Resultado del Tratamiento , Factor de Necrosis Tumoral alfa/efectos adversosRESUMEN
Patients suffering from rheumatoid arthritis have increased risk of infections when compared with general population. The risk depends directly from disease activity and severity. Furthermore, risk increases with aging, immunosuppressive agents and comorbidities such as diabetes, pulmonary and cardiac diseases. In particular corticosteroids, even at low doses, are a major risk factor. Due to disease related risk it is difficult to separate the risk deriving from the use of TNF alpha blockers. Data from clinical trials, meta-analysis and national registers are somewhat contradictory. In patients with rheumatoid arthritis on routine follow-up, treatment with TNF alpha blockers seems to carry an increased risk of infections compared to traditional DMARDs but not associated with increased risk of overall serious infection. Physicians should carefully monitor for signs of infection when using TNF alpha blockers, particularly shortly after treatment initiation.
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Antirreumáticos/efectos adversos , Artritis Reumatoide/microbiología , Infecciones Bacterianas/inmunología , Inmunosupresores/efectos adversos , Factor de Necrosis Tumoral alfa/antagonistas & inhibidores , Antirreumáticos/uso terapéutico , Artritis Reumatoide/tratamiento farmacológico , Artritis Reumatoide/inmunología , Infecciones Bacterianas/inducido químicamente , Quimioterapia Combinada , Medicina Basada en la Evidencia , Humanos , Inmunosupresores/uso terapéutico , Factores de RiesgoRESUMEN
OBJECTIVE: We evaluated both the efficacy and safety of anakinra in daily routine rheumatoid arthritis clinical practice. METHODS: We studied 60 cases, including patients with previous anti-TNFalpha exposure, treated with anakinra (100 mg/daily s.c.) in combination with methotrexate (7.5-10 mg/week i.m.) or leflunomide (20 mg/die) in a two year observational study. Efficacy measures were assessed using the American College of Rheumatology (ACR) response criteria. Safety was evaluated according to a modified World Health Organization adverse reaction term dictionary. RESULTS: At week 14, ACR 20% response criteria have been fulfilled by 53 (91.3%) out of 58 patients, 51 (87.9%) of them achieving also an ACR 50%and 15 (25.8%) an ACR 70%response. Thirteen patients touched 102 weeks of treatment: ACR 20% response was achieved in 92.3%, while ACR 50% and ACR 70% were respectively found in 84.6% and 38.4% of the cases. The mean decrease in HAQ score was 0.38, p<0.001. Of the 16 patients who were previously treated with anti-TNFalpha blockers, 81.2% responded to anakinra. There was no significant difference in the ACR response between groups with and without previous anti-TNFalpha exposure. Seventeen patients (28.3%) stopped anakinra because of side-effects (5%) or failure to respond (23.3%). Only 4 cases of pulmonitis, of which 2 have been hospitalised, and 1 case with tuberculosis (previously treated with infliximab) were observed. CONCLUSIONS: Our clinical experience confirms that anakinra is effective and safe in the treatment of rheumatoid arthritis. Anakinra seems also useful in patients with previous anti-TNFalpha blockers failures. Even though major adverse events were rare, clinicians should be aware of such a possibility.
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Antirreumáticos/uso terapéutico , Artritis Reumatoide/tratamiento farmacológico , Proteína Antagonista del Receptor de Interleucina 1/uso terapéutico , Anciano , Antirreumáticos/administración & dosificación , Antirreumáticos/efectos adversos , Quimioterapia Combinada , Femenino , Humanos , Proteína Antagonista del Receptor de Interleucina 1/administración & dosificación , Proteína Antagonista del Receptor de Interleucina 1/efectos adversos , Isoxazoles/uso terapéutico , Leflunamida , Masculino , Metotrexato/uso terapéutico , Persona de Mediana Edad , Proteínas Recombinantes/uso terapéutico , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Pigmented villonodular synovitis (PVNS) is a rare pre-malignant disease that require aggressive treatment as surgical synovectomy, eventually followed by radiosynovectomy. Nevertheless, the disease often reoccurs after these treatments. To determine the safety and efficacy of intra-articular (IA) TNFalpha blockade with etanercept (ETN), before extended arthroscopic synovectomy, in severe PVNS of the knee, two patients, (a 26-year-old man with B27+ undifferentiated spondylarthropathy and a 32-year-old femal with seronegative oligoarthritis), affected by diffuse knee PVNS (diagnosis made by histological examination), resistant to IA corticosteroid injections and to repeated arthroscopic synovectomy, were submitted, after protocol approval by human research committee and patient's written informed consent to intra-articular etanercept (IA-ETN) treatment with a different dosage schedule: 12.5 mg weekly IA-ETN injection for 4 weeks, followed by extended arthroscopic synovectomy and of 25 mg IA-ETN injection for 4 weeks, respectively. Previous DMARDs treatment was continued in stable appropriate doses. Any adverse events were recorded throughout the study. The following parameters were considered as clinical endpoints: 1) Knee Joint Index (KJI: range 0-14); 2) Thompson index (THI: range 0-9) At the study entry and at the end of follow-up, high frequency ultrasound grey scale synovial thickening (US-ST) was also assessed. No adverse events were observed due to IA-ETN and to arthroscopic synovectomy. Marked improvement of knee disease activity over time and sustained functional recover was obtained. US-ST evaluation before treatment initiation and at the end of follow-up confirmed the regression of knee joint synovial proliferation.
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Antirreumáticos/administración & dosificación , Inmunoglobulina G/administración & dosificación , Articulación de la Rodilla , Receptores del Factor de Necrosis Tumoral/administración & dosificación , Sinovitis Pigmentada Vellonodular/tratamiento farmacológico , Adulto , Etanercept , Femenino , Humanos , Inyecciones Intraarticulares , Masculino , Cuidados Preoperatorios , Sinovectomía , Sinovitis Pigmentada Vellonodular/cirugía , Resultado del TratamientoRESUMEN
A case of an adult patient with vitamin D-resistant osteomalacia or X-linked hypophosphatemic osteomalacia (XLH) with diffuse calcification of entheses is reported. XLH is the most frequent cause of rickets in developed countries. It is characterized by an impaired renal transport of the phosphate and mutation of PFEX (phosphate regulating gene, with homologies to endopeptidase on the X-chromosome). In childhood, the classic clinical presentation includes short stature and bow leg. While at this age the main radiographic features are characterised by rickets, in adult life they are dominated by a generalised calcific enthesopathy. Concerning the pathogenesis of the enthesopathic lesions of XLH, no convincing hypothesis has yet been made. As in our patient, the extension and the severity of enthesopathy seems not related to the severity of the biochemical changes nor to the treatment with calcitriol. The calcified enthesopathy is an integral part of XLH and it is possible that it is found in adult because many years are necessary to produce it.
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Calcinosis/etiología , Osteomalacia/complicaciones , Enfermedades Reumáticas/etiología , Adulto , Resistencia a Medicamentos , Humanos , Masculino , Osteomalacia/diagnóstico , Osteomalacia/tratamiento farmacológico , Vitamina D/uso terapéuticoRESUMEN
OBJECTIVE: The real effects of mud-bath applications on the inflammatory process are still not clarified. We studied these effects on rat adjuvant-induced arthritis. METHODS: Arthritis was induced in 30 rats by subplantar injection of Freund's complete adjuvant (FCA) into the right hind paw. Ten days after FCA injection, the rats were randomized in 3 groups of 10 each: the first one was submitted to a cycle of mud-bath applications, the second one was treated with indomethacin, the third one received only saline per os (control group). The paw volume, measured by plethysmometry, and the serum levels of TNFalpha and IL-1beta were considered as evaluation parameters. RESULTS: FCA injection caused a progressive enhancement of paw volume and a rapid increase of TNFalpha and IL-1beta serum levels. After the randomization, mud-bath applications reduced inflammation and at the end of the treatment the paw volume and the TNFa and IL-1beta serum levels were significantly tapered in comparison to the controls (p < 0.01). CONCLUSION: The results of the study suggest an anti-inflammatory effect of mud-bath applications on adjuvant arthritis in rats. These results could explain the beneficial effects of thermal treatments observed in some inflammatory rheumatic diseases.
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Artritis Experimental/terapia , Peloterapia , Enfermedad Aguda , Animales , Antiinflamatorios no Esteroideos/farmacología , Artritis Experimental/patología , Adyuvante de Freund , Indometacina/farmacología , Interleucina-1/sangre , Masculino , Ratas , Ratas Endogámicas Lew , Factor de Necrosis Tumoral alfa/metabolismoRESUMEN
OBJECTIVE: Ten new cases with primary Sjögren's syndrome (pSS) whose disease began before age 16 are described. Special attention is paid both to the follow-up and treatment of this condition. METHODS: Cases with juvenile pSS were retrospectively identified from our series of 180 pSS patients. Ocular, salivary, and extraglandular manifestations as well as a full laboratory evaluation including HLA-DR typing were retrieved. RESULTS: A disease prevalence of 5.5% (10 cases, 8 female and 2 male) was found in our series. The mean age at onset was 11.0 years, but the disease started at the age of 4 in 2 patients. At onset, parotid swelling was found in 6 cases and extraglandular manifestations in 3. Throughout the follow-up period (mean 48.6 months from the time of diagnosis), the clinical picture was similar to that of pSS in adults, but oral involvement was generally milder. Extraglandular manifestations were always present but never severe. Pertinent laboratory abnormalities (e.g. rheumatoid factor, polyclonal hypergammaglobulinemia, leukopenia, increased ESR, ANA and anti-SSA/SSB antibodies) were found in all patients. Specifically, ANA and anti-SSA were always positive. Moreover, in our cases histocompatibility antigens HLA-DR3 and DR 52 were closely associated with the disease. Clinical outcome was difficult to predict; however, no serious complications have been observed so far. We obtained good results with low-dose steroids and/or hydroxychloroquine, especially with regard to the extraglandular manifestations and laboratory abnormalities. CONCLUSION: We confirm that juvenile pSS is not a rare condition. It closely resembles pSS in adults except for the extremely high prevalence of recurrent parotitis and immunological findings.
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Antirreumáticos/uso terapéutico , Glucocorticoides/uso terapéutico , Hidroxicloroquina/uso terapéutico , Síndrome de Sjögren/epidemiología , Adolescente , Adulto , Edad de Inicio , Niño , Quimioterapia Combinada , Femenino , Estudios de Seguimiento , Prueba de Histocompatibilidad , Humanos , Masculino , Prevalencia , Estudios Retrospectivos , Síndrome de Sjögren/tratamiento farmacológico , Síndrome de Sjögren/etiologíaRESUMEN
Henoch-Schönlein purpura (HSP) is considered to be a small blood vessel systemic vasculitis. Numerous microorganisms have been implicated in triggering HSP. We describe an unusual case of HSP with glomerulonephritis and renal failure requiring haemodialysis in a young adult man who subsequently developed paroxysmal nocturnal haemoglobinuria (PNH) with several haemolytic episodes. Bacterial infections, especially those of the respiratory and urinary tract, might trigger both the diseases.
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Lesión Renal Aguda/diagnóstico , Glomerulonefritis/diagnóstico , Hemoglobinuria Paroxística/diagnóstico , Vasculitis por IgA/diagnóstico , Lesión Renal Aguda/complicaciones , Lesión Renal Aguda/terapia , Adulto , Terapia Combinada , Quimioterapia Combinada , Transfusión de Eritrocitos , Estudios de Seguimiento , Glomerulonefritis/complicaciones , Glomerulonefritis/terapia , Hemoglobinuria Paroxística/complicaciones , Hemoglobinuria Paroxística/terapia , Humanos , Vasculitis por IgA/complicaciones , Vasculitis por IgA/terapia , Masculino , Diálisis Renal , Medición de Riesgo , Resultado del TratamientoRESUMEN
BACKGROUND: We studied the prevalence of fibromyalgia in 3 different groups of patients affected respectively with systemic lupus erythematosus, systemic sclerosis (scleroderma) and primary Sjögren's syndrome. The typical fibromyalgia findings encountered in these diseases were examined. METHODS: We enrolled 250 consecutive outpatients: 100 with systemic lupus erythematosus, 50 with systemic sclerosis, 100 with primary Sjögren's syndrome and 2 control groups (30 healthy subjects and 75 patients with primary fibromyalgia). Fibromyalgia features were evaluated by algometry, VAS for pain, Mc Gill Pain Questionnaire and Fibromyalgia Impact Questionnaire. RESULTS: Fibromyalgia has been found in 1 case (1%) with systemic lupus erythematosus, 1 case with systemic sclerosis (2%), 22 cases (22%) with primary Sjögren's syndrome and in 1 (3.3%) of the healthy controls. The number of tender points was significantly higher (p<0.01) in the patients with Sjögren's syndrome in comparison with the other groups. Fibromyalgic findings were similar in the patients with primary fibromyalgia and Sjögren's syndrome with fibromyalgia, unless for both poor sleep and low algometric thresholds which were more frequently found in primary fibromyalgia (respectively p<0.001 and p=0.05). CONCLUSIONS: Our study suggests that fibromyalgia is relatively frequent in primary Sjögren's syndrome, while in systemic lupus and systemic sclerosis its prevalence is not different from that found in the healthy controls. Typical fibromyalgia findings, except algometric values, were similar between the cases with Sjögren's syndrome plus fibromyalgia and fibromyalgia alone.
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Fibromialgia/etiología , Lupus Eritematoso Sistémico/complicaciones , Esclerodermia Sistémica/complicaciones , Síndrome de Sjögren/complicaciones , Estudios de Casos y Controles , Fibromialgia/epidemiología , Fibromialgia/fisiopatología , Humanos , Dimensión del Dolor , PrevalenciaRESUMEN
We describe the emergence of arthritis following a physical trauma, in a young man with clinical and histopathologic features of primary anetoderma (Jadassohn type) of 13 years' duration. Diagnosis of post-traumatic arthritis in a young patient with genetic predisposition was assumed. Indeed septic arthritis and other possible cause of arthritis were ruled out.
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Articulación del Tobillo , Artritis/etiología , Tejido Elástico , Enfermedades de la Piel/complicaciones , Adulto , Traumatismos de los Dedos/complicaciones , Estudios de Seguimiento , Humanos , Masculino , Síndrome , Factores de Tiempo , Heridas Punzantes/complicacionesRESUMEN
OBJECTIVES: The exact prevalence of erosive osteoarthritis (EOA) is still unknown. Aim of our study was therefore to evaluate the prevalence of osteoarthritis (OA) and EOA of the hand in a representative population from Venetian area. METHODS: All people aged > 40 years of a small town in Venetian area has been considered. A complete clinical evaluation has been performed in all subjects. For those patients with clinical signs or symptoms of OA, radiographic evaluation of the hands has been carried out. RESULTS: The entire population aged >40 years living in Silea entered the study. Signs or symptoms of OA were present in 200 out 640 subjects (31.2%), whereas EOA was diagnosed in 17 (8.5%) all female, mean age 57.5 +/-10.1 years (range 41-74) mean disease duration 5.1 +/-3.8 years (range 1-15). The most frequently involved joint was the second DIP of the right hand. CONCLUSION: The prevalence of EOA in the Venetian area seems to be around 8.5%.
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Mano , Osteoartritis/epidemiología , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Italia/epidemiología , Masculino , Persona de Mediana Edad , PrevalenciaRESUMEN
Different animal studies show that several proinflammatory cytokines are essential for natural resistance to specific infections, particularly versus intracellular organisms. However, uncontrolled overproduction of some proinflammatory cytokines, in diseases such as rheumatoid arthritis, can be just as dangerous to the host as the absence of the same cytokines. Reduction in the production and/or activities of proinflammatory cytokines in rheumatoid arthritis remains a therapeutic objective for many patients. The tumour necrosis factor-alpha (TNF-alpha) blockers infliximab, etanercept and adalimumab and the recombinant interleukin 1 (IL-1) receptor antagonist anakinra are effective in patients with active rheumatoid arthritis. However, there is a growing body of clinical evidence that neutralization of TNF-alpha is associated with an increased risk of opportunistic infections, including mycobacterial diseases. Blockade of IL-1 activity with the IL-1 receptor antagonist (IL-1Ra) appears, at present, to be relatively safe. Postmarketing experience and pharmacovigilance programs are necessary to determine the overall safety profile of the new agents. At this time, treating physicians must weigh carefully the benefits of biologics against their safety, particularly in patients at risk of infection.
Asunto(s)
Artritis Reumatoide/complicaciones , Artritis Reumatoide/tratamiento farmacológico , Citocinas/antagonistas & inhibidores , Infecciones Oportunistas/etiología , Infecciones Oportunistas/prevención & control , Adalimumab , Animales , Anticuerpos Monoclonales/uso terapéutico , Anticuerpos Monoclonales Humanizados , Antirreumáticos/uso terapéutico , Etanercept , Humanos , Inmunoglobulina G/uso terapéutico , Infliximab , Proteína Antagonista del Receptor de Interleucina 1 , Receptores de Interleucina-1/antagonistas & inhibidores , Receptores del Factor de Necrosis Tumoral/uso terapéutico , Sialoglicoproteínas/uso terapéutico , Factor de Necrosis Tumoral alfa/antagonistas & inhibidoresRESUMEN
Most of the salivary glands diseases are characterized only by a few distinct clinical patterns. Medical history and clinical examination are still considered of great relevance. However, in order to obtain a definite diagnosis, imaging techniques are required in most of the cases. Salivary glands ultrasonography (US) is the technique to be used as the first because US can easily differentiate calculosis, inflammatory diseases and tumors. Sonography is also frequently needed to perform needle aspiration or biopsy (FNAC). Sialography should be used essentially for assessing chronic sialoadenitis as well as Sjögren's syndrome. At present, Magnetic Resonance sialography should be preferred because of the greater sensibility in diagnosing inflammatory diseases of the salivary glands. It allows to evaluate both intraglandular oedema and nodules, so that incannulation of the salivary duct is not required. Computer Tomography (CT) and Magnetic Resonance imaging (MR) are useful when neoplasm are suspected, particularly if deep areas of the gland, which cannot be visualized by US, are involved. Sequential scintigraphy is currently employed for assessing the functional status of all the 4 major salivary glands and evaluating the chronic evolution of glandular damage.
RESUMEN
Nasopharyngeal carcinoma has long been reported as the predominant type of cancer associated with dermatomyositis in many several Asian countries, including Hong Kong, Singapore, and Southern-Cina. Dermatomyositis is one of the idiopathic inflammatory myopathies showing characteristic cutaneous manifestations. Reviews from the western literature have demonstrated that certain cancers, such as ovarian and breast carcinoma in women and lung and prostate carcinoma in men, are highly associated with DM relative to the general population. We report the case of a Caucasian Italian patient with nasopharyngeal carcinoma and dermatomyositis. Considering the rarity of nasopharyngeal carcinoma among whites, both the detection and the report of each new case are noteworthy in defining the geographic and ethnic distribution of this tumor.