RESUMEN
OBJECTIVES: To compare the survival rate, and the clinical and laboratory characteristics in patients, characterized by the presence of certain anti-neutrophil cytoplasmic auto-antibodies (ANCAs). METHODS: In a retrospective observational study, we analyzed the data of all patients with a positive ANCA test between 1995 and 2005 at our hospital. Based on serology patients were divided in three subgroups (ANCA-Proteinase 3 (PR3), ANCA-Myeloperoxidase (MPO) and atypical ANCA), irrespective of the diagnosis. Patient survival was compared by Kaplan Meier survival analysis. Differences in clinical and laboratory characteristics between the groups of specific ANCAs were determined. RESULTS: Fifty-four ANCA-positive patients were analyzed. Eighteen of these patients were ANCA-PR3-positive, 17 were ANCA-MPO-positive and 19 had a atypical ANCA. A random control group was created of matched ANCA negative patients. Average follow-up time was 52 months. The calculated five year survival rate in respectively the ANCA-PR3- positive group, the ANCA-MPO-positive group, the atypical ANCA group and the ANCA-negative group was 45%, 81%, 90% and 100%. (P = 0.012, Wilcoxon test). A higher mean leukocyte count, a higher mean erythrocyte sedimentation rate and more fever was observed in the ANCA-PR3-positive group compared to the ANCA-MPO-positive group. CONCLUSIONS: A remarkable lower survival rate was observed in ANCA-PR3-positive patients compared to ANCA-MPO-positive patients. We also demonstrated that patients characterized by the presence of a defined ANCA differ in clinical and laboratory characteristics.
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Propylthiouracil (PTU) is a frequently prescribed drug in the treatment of hyperthyroidism. The use of PTU is, however, accompanied by numerous potentially serious side effects including vasculitis. PTU-related vasculitides can present as haematuria, pulmonary haemorrhage, or cutaneous lesion together with aspecific symptoms such as fever, myalgia, arthralgia, and fatigue. Cerebral involvement is seldom observed. We present a 49-year-old female with Graves' disease and asthma, who developed paresis of the proximal extremities, eosinophilia, pulmonary, and cutaneous lesions following treatment with PTU. A cerebral vasculitis consistent with Churg-Strauss syndrome (CSS) was suspected. Although cerebral involvement is seldom observed with PTU treatment, cerebral vasculitis should be considered in patients developing CNS symptoms.
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A 55-year-old man, with no previous history, presented with extreme fatigue and fever and was admitted to hospital. He had progressive renal dysfunction and his serum anti-neutrophil cytoplasmic antibodies (ANCA) were markedly elevated. Renal histology was consistent with ANCA-associated vasculitis. The patient was successfully treated with cyclophosphamide and prednisolone. The classification and management of the ANCA-associated vasculitides are described. The classification was guided by the clinical presentation, serology and results of tissue biopsies. The ANCA inflammation had affected the middle sized and small vessels of especially the upper and lower airways, and the kidneys. The antibodies were directed at proteinase-3 (PR3) or myeloperoxidase (MPO). PR3-ANCA is predominantly found in Wegener's granulomatosis, while MPO-ANCA is related to microscopic polyangiitis. Tissue studies showed granulomatous inflammation of the airways which is typical of Wegener's disease. This type of inflammation is absent in microscopic polyangiitis. The initial treatment schedule consists of prednisone 1 mg/kg daily and oral cyclophosphamide 2 mg/kg daily. In the remission phase, the cyclophosphamide is replaced by azathioprine. It is not yet known how long maintenance treatment should be continued and which parameters have prognostic value.
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Anticuerpos Anticitoplasma de Neutrófilos/sangre , Factores Inmunológicos/uso terapéutico , Enfermedades Renales/diagnóstico , Vasculitis/diagnóstico , Azatioprina/uso terapéutico , Ciclofosfamida/uso terapéutico , Quimioterapia Combinada , Humanos , Enfermedades Renales/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Prednisolona/uso terapéutico , Inducción de Remisión , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Vasculitis/tratamiento farmacológicoRESUMEN
A 41-year-old woman who had suffered from systemic lupus erythematosus (SLE) for 22 years presented with signs of neurological deficits. CT-scanning of the brain revealed hypodense lesions that suggested cerebral infarction due to vasculitis in SLE. However, in spite of intensified immunosuppressive therapy, she showed rapid neurological deterioration. After extensive, additional examinations and tests, the diagnosis was finally changed to progressive multifocal leukoencephalopathy, caused by an opportunistic infection by the JC polyomavirus. Neurological and psychiatric symptoms frequently occur in patients with SLE. The differential diagnosis of these symptoms in SLE is extensive and includes, on the one hand, primary neurological and psychiatric diseases related to direct involvement of the nervous system by SLE, and on the other hand, secondary syndromes arising as a result of complications of the SLE or the immunosuppressive treatment. Opportunistic infections are often an important secondary cause of neurological and psychiatric syndromes in patients with SLE. The clinical symptoms and radiological cerebral signs are non-specific and usually do not suffice to differentiate between the various syndromes. Since each syndrome requires its own specific clinical approach and treatment, extensive diagnostics are mandatory before the diagnosis 'cerebral lupus' can be made and immunosuppressive therapy can be started or intensified.
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Inmunosupresores/efectos adversos , Leucoencefalopatía Multifocal Progresiva/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Adulto , Encéfalo/patología , Diagnóstico Diferencial , Femenino , Humanos , Inmunosupresores/uso terapéutico , Virus JC/aislamiento & purificación , Leucoencefalopatía Multifocal Progresiva/inmunología , Leucoencefalopatía Multifocal Progresiva/patología , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/inmunologíaRESUMEN
OBJECTIVE: To obtain insight in the acute-phase response in SLE. METHODS: The clinical history, SLEDAI, CRP and ferritin concentrations were analysed throughout the disease course of 10 SLE patients. RESULTS: During a mean follow-up of 4.8 years, 10 exacerbations (SLEDAI > or = 11) occurred. Throughout the disease course, CRP and SLEDAI correlated positively in 5 patients, whereas the correlation between SLEDAI and ferritin was positive in 7 patients. However, elevated CRP concentrations together with elevated ferritin levels were only observed during 4 exacerbations. Ferritin concentrations were exceptionately high (> 1500 microg/L) during 4 flare-ups. CRP and ferritin levels remained normal during 5 exacerbations. CONCLUSION: SLE is characterised by highly variable and unusual CRP and ferritin responses that do not always reflect the extent of inflammation in individual patients. Despite severe disease activity, ferritin levels can remain well within the normal range, limiting its clinical usefulness as a marker for disease activity.
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Biomarcadores/análisis , Proteína C-Reactiva/análisis , Ferritinas/sangre , Lupus Eritematoso Sistémico/sangre , Reacción de Fase Aguda , Progresión de la Enfermedad , Estudios de Seguimiento , Humanos , InflamaciónRESUMEN
A 19-year-old woman was admitted because of high fever, rash, arthralgia and sore throat. On physical examination a diffuse skin rash was observed, leaving a facial mask unaffected. C-reactive protein and erythrocyte sedimentation rate were raised (114 mg/l and 26 mm in the first hour, respectively); white blood cell count was normal (6.2 x 10(9)/l) with an increased count of immature forms. An infective, metabolic or haematological cause was excluded. Serum ferritin turned out to be extremely elevated (4318 micrograms/l), so adult-onset Still's disease was diagnosed. The patient fulfilled the criteria of Cush et al. for adult-onset Still's disease. She was first treated with non-steroidal anti-inflammatory drugs (NSAIDs) and, at a later stage in the disease, with corticosteroids. All symptoms disappeared and blood test results normalised.
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Enfermedad de Still del Adulto/diagnóstico , Corticoesteroides/uso terapéutico , Adulto , Antiinflamatorios no Esteroideos/uso terapéutico , Artralgia/etiología , Competencia Clínica , Toma de Decisiones , Diagnóstico Diferencial , Exantema/etiología , Femenino , Ferritinas/sangre , Fiebre/etiología , Humanos , Enfermedad de Still del Adulto/sangre , Enfermedad de Still del Adulto/tratamiento farmacológicoRESUMEN
This cohort study prospectively evaluated the prevalence of the silicone-related symptom complex (SRSC) in relation to antinuclear antibodies (ANA) and magnetic resonance imaging (MRI) of silicone breast implants (SBI) 1 year after implantation. A total of 57 women undergoing mastectomy followed by immediate breast reconstruction (IBR) and SBI between March 1995 and March 1997 at the University Hospital Rotterdam/Daniel den Hoed Cancer Centre, were prospectively evaluated. Just before and 1 year after IBR the sera of these women were tested for the presence of ANA and they were screened for the prevalence of SRSC-related symptoms by questionnaire. All prostheses were evaluated by MRI 1 month and 1 year after IBR. Just before operation 11% of the women had a Sjögren score of more than 2, whereas 30% had such a score 1 year after IBR ( P = 0.01). One year postoperatively women had significantly more RA/Raynaud-related complaints: 21% preoperatively versus 40% 1 year after IBR ( P = 0.03). Within the undefined complaints-related group 19% had a score of 2 or more preoperatively and 33% 1 year after IBR ( P = 0.09). There were no new cases of ANA positivity 1 year after IBR. The linguine sign was seen by MRI in three implants: one 1 month after IBR and two 1 year after IBR. There was no relation to changes in SRSC expression and these MRI findings. In conclusion, 1 year after SBI implantation women had more SRSC-related complaints, especially Sjögren's and RA/Raynaud's. Moreover there was no correlation between elevated SRSC expression and changes in the presence of ANA or changes in MRI of the SBI 1 year after IBR.
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Implantes de Mama/efectos adversos , Enfermedades Reumáticas/etiología , Geles de Silicona/efectos adversos , Adulto , Anticuerpos Antinucleares/análisis , Estudios de Cohortes , Femenino , Humanos , Imagen por Resonancia Magnética , Persona de Mediana Edad , Estudios Prospectivos , Enfermedades Reumáticas/diagnóstico , Enfermedades Reumáticas/inmunología , Enfermedades Reumáticas/fisiopatología , Encuestas y CuestionariosRESUMEN
BACKGROUND: Adult onset of Still's disease is characterized by very high serum ferritin levels, in disproportion with other acute phase proteins (APPs). Because interferon-alpha (IFN-alpha) was observed to cause hyperferritinaemia in three healthy people without increase of other APPs, we hypothesized that IFN-alpha stimulates specifically the synthesis of ferritin. To test this hypothesis, we studied ferritin and other APP levels in patients treated with IFN-alpha. PATIENTS AND METHODS: Fifteen patients treated with IFN-alpha-2b 3-5 times a week, as adjuvant treatment after excision of a high-risk melanoma, were compared with six patients without adjuvant treatment (controls). Serum levels of C-reactive protein (CRP) and secretory phospholipase A2 (sPLA2) were measured using ELISA. Levels of ferritin, alpha1-acid glycoprotein (AAG) and albumin were determined by nephelometry. RESULTS: CRP was decreased significantly after 4 weeks (P < 0.01) in the patients treated with IFN-alpha compared with the nontreated patients, after 6 months of treatment it was still decreased although not significantly. Ferritin increased significantly in the IFN-alpha-treated patients: 187% of pretreatment value after 4 weeks and 217% after 6 months (P < 0.01), while ferritin levels decreased in the nontreated patients. AAG increased significantly in IFN-alpha-treated patients (107, 114%) compared with the control-patients (91, 76%) but differences were less compared with CRP and ferritin. sPLA2 had a variable course, while albumin remained constant within the normal range in both patient groups. CONCLUSIONS: IFN-alpha induced a significant increase in ferritin, with a significant decrease in CRP, little increase in AAG, varying response of sPLA2 and no change in albumin. This finding suggests a specific role for IFN-alpha in the synthesis or secretion of ferritin. This mechanism may also be involved in the marked hyperferritinaemia in adult onset of Still's disease.