RESUMEN
INTRODUCTION: Spontaneous adrenal hemorrhages (AH) are a rare condition with no consensus about their management. METHODS: Patients were identified using the Medicalization of the Information System Program database, imaging software and a call for observations to internists, intensivists and obsetricians working at our institution. Adult patients whose medical records were complete and whose diagnosis was confirmed by medical imaging were included. RESULTS: From 2000 to 2007, 20 patients were identified, including 15 were women. The clinical onset of AH was non-specific. In five cases, AH occurred during pregnancy; four of them were unilateral and right sided. The etiology of the other fifteen (bilateral adrenal hemorrhage in 11) were as follows: antiphospholipid syndrome (n=8), heparin-induced thrombocytopenia (n=4), essential thrombocythemia (n=3), spontaneous AH due to oral anticoagulants (n=1), complication of a surgical act (n=3), and sepsis (n=3). In seven cases, two causes were concomitant. The diagnosis of AH was often confirmed by abdominal CT. An anticoagulant treatment was initiated in 16 cases. Ten of the eleven patients presenting with bilateral adrenal hematomas were treated using a long-term substitute opotherapy. One patient died because of a catastrophic antiphospholipid syndrome. CONCLUSION: The clinical onset of HS is heterogeneous and non-specific. The confirmatory diagnosis is often based on abdominal CT. The search for an underlying acquired thrombophilia is essential and we found in this study etiological data comparable to the main series in the literature. Adrenal insufficiency is most of the time definitive in cases of bilateral involvement.
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Enfermedades de las Glándulas Suprarrenales , Síndrome Antifosfolípido , Enfermedades de las Glándulas Suprarrenales/diagnóstico , Enfermedades de las Glándulas Suprarrenales/epidemiología , Enfermedades de las Glándulas Suprarrenales/terapia , Adulto , Anticoagulantes/uso terapéutico , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/diagnóstico , Síndrome Antifosfolípido/epidemiología , Femenino , Hematoma/diagnóstico , Hematoma/epidemiología , Hematoma/etiología , Hemorragia , Humanos , Embarazo , Estudios RetrospectivosRESUMEN
The association between thymoma and autoimmunity is well known. Besides myasthenia gravis, which is found in 15 to 20% of patients with thymoma, other autoimmune diseases have been reported: erythroblastopenia, systemic lupus erythematosus, inflammatory myopathies, thyroid disorders, Isaac's syndrome or Good's syndrome. More anecdotally, Morvan's syndrome, limbic encephalitis, other autoimmune cytopenias, autoimmune hepatitis, and bullous skin diseases (pemphigus, lichen) have been reported. Autoimmune diseases occur most often before thymectomy, but they can be discovered at the time of surgery or later. Two situations require the systematic investigation of a thymoma: the occurrence of myasthenia gravis or autoimmune erythroblastopenia. Nevertheless, the late onset of systemic lupus erythematosus or the association of several autoimmune manifestations should lead to look for a thymoma. Neither the characteristics of the patients nor the pathological data can predict the occurrence of an autoimmune disease after thymectomy. Thus, thymectomy usefulness in the course of the autoimmune disease, except myasthenia gravis, has not been demonstrated. This seems to indicate the preponderant role of self-reactive T lymphocytes distributed in the peripheral immune system prior to surgery. Given the high infectious morbidity in patients with thymoma, immunoglobulin replacement therapy should be considered in patients with hypogammaglobulinemia who receive immunosuppressive therapy, even in the absence of prior infection.
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Enfermedades Autoinmunes/etiología , Timoma/complicaciones , Neoplasias del Timo/complicaciones , Enfermedades Autoinmunes/clasificación , Enfermedades Autoinmunes/epidemiología , Humanos , Incidencia , Factores de Riesgo , Timoma/epidemiología , Timoma/inmunología , Neoplasias del Timo/epidemiología , Neoplasias del Timo/inmunologíaRESUMEN
Interleukin-1 is a major cytokine of innate immunity and inflammation. It exerts various systemic effects during the inflammatory response, such as fever induction, thrombopoiesis and granulopoiesis, or leukocyte recruitment. Its involvement has been demonstrated in many inflammatory-mediated diseases, such as diabetes or gout. Moreover, interleukin-1 plays a pivotal role in some autoinflammatory diseases, such as cryopyrinopathies or familial Mediterranean fever. In these diseases, a constitutional defect of the inflammasome, a protein complex responsible for the activation of interleukin-1, explains the hypersecretion of interleukin-1. Other autoinflammatory diseases have a more complex pathophysiology involving deregulation of the interleukin-1 pathway, upstream or downstream of the inflammasome, or through more complex mechanisms. In this review, we are detailing the synthesis, the activation, the signalling, and the regulation of interleukin-1. We then describe the autoinflammatory diseases or related-diseases where the pathological role of interleukin-1 has been demonstrated.
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Enfermedades Autoinflamatorias Hereditarias/metabolismo , Inflamasomas/metabolismo , Interleucina-1/metabolismo , Predisposición Genética a la Enfermedad , Enfermedades Autoinflamatorias Hereditarias/genética , Humanos , MutaciónRESUMEN
INTRODUCTION: Several case reports have reported the benefit of intravenous immunoglobulin therapy in many autoimmune diseases, including systemic lupus erythematosus. CASE REPORTS: Here, we report on two cases of lupus myocarditis treated with high dose of intravenous immunoglobulin. The first patient was a 42-year-old woman who presented with lupus myocarditis that was resistant to corticosteroids and cyclophosphamide, and who was finally successfully treated with a single dose of 2 g/kg of intravenous immunoglobulin. The patient displayed clinical improvement a few days later. The second case - a 43-year-old woman was diagnosed with lupus myocarditis and immunosuppressive drugs were contraindicated because of the context of a recent infective endocarditis. She was treated with repeated dose of 2 g/kg of intravenous immunoglobulin. Clinical improvement was observed and the left ventricular ejection fraction increased from 20 % to 60 % within a few days. We also report 9 similar observations identified from a literature review. CONCLUSION: The use of intravenous immunoglobulin in lupus myocarditis is not officially recognized but could be considered as an alternative when conventional therapies have failed or are contraindicated.
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Inmunoglobulinas Intravenosas/uso terapéutico , Lupus Eritematoso Sistémico/terapia , Miocarditis/terapia , Uso Fuera de lo Indicado , Adulto , Femenino , Humanos , Lupus Eritematoso Sistémico/complicaciones , Miocarditis/etiología , Resultado del TratamientoRESUMEN
Opportunistic infections (OI) are uncommon in sarcoidosis (1 to 10%) and mostly occur in patients with previously diagnosed disease or can rarely be the presenting manifestation. The most common OIs are, in descending order: aspergillosis, cryptococcosis, and mycobacterial infections. Treatment with corticosteroids is the most frequent risk factor for OI occurrence during sarcoidosis but immunosuppressive drugs and therapy with anti-TNFα are also risk factors. Overall, clinical presentation, treatment, and outcome are identical to that occur in other conditions complicated with the occurrence of OIs. However, some atypical presentations of OIs can mimic sarcoidosis exacerbation and misdiagnosis may lead clinicians to increase immunosuppression, causing worsening of the OI. The meticulous collection of patient's history along with factors differentiating OI from sarcoidosis exacerbation is key factor to optimally manage these patients.
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Infecciones Oportunistas/etiología , Sarcoidosis/complicaciones , Aspergilosis/etiología , Criptococosis/etiología , Humanos , Huésped Inmunocomprometido , Infecciones por Mycobacterium/etiología , Micosis/etiología , Infecciones Oportunistas/inmunología , Infecciones Oportunistas/terapia , Enfermedades Raras/etiología , Factores de Riesgo , Sarcoidosis/inmunología , Sarcoidosis/terapia , Virosis/etiologíaRESUMEN
INTRODUCTION: Gemcitabine-induced thrombotic microangiopathy is a rare event whose management is not yet consensual. The use of eculizumab could be of interest. CASE REPORT: A 68-year-old woman was treated by gemcitabine as adjuvant chemotherapy of a pancreatic adenocarcinoma. Two months later, the patient presented with mechanical hemolytic anemia, thrombocytopenia and high blood pressure that led to the diagnosis of thrombotic microangiopathy. Gemcitabine was stopped. Plasma exchange therapy was introduced since hematological and renal parameters had worsened. As clinical efficacy was insufficient, eculizumab was introduced at a dose of 900 mg per week 4 times, then 1200 mg every 2 weeks. Symptoms along with hematological and nephrological analysis were back to physiological standards after 7 intravenous injections. CONCLUSION: Eculizumab seems to be an effective treatment against gemcitabine-induced thrombotic microangiopathy in case of severe hematological and renal injuries associated with a lack of response to plasma exchange therapy.
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Anticuerpos Monoclonales Humanizados/uso terapéutico , Desoxicitidina/análogos & derivados , Microangiopatías Trombóticas/inducido químicamente , Microangiopatías Trombóticas/tratamiento farmacológico , Adenocarcinoma/tratamiento farmacológico , Anciano , Desoxicitidina/efectos adversos , Femenino , Humanos , Neoplasias Pancreáticas/tratamiento farmacológico , Resultado del Tratamiento , GemcitabinaRESUMEN
OBJECTIVES: To describe the clinical features, treatment, and outcome of autoimmune diseases (AD) in a cohort of patients with thymoma. DESIGN: Pathological records from three university hospitals, between 2005 and 2011, were reviewed to identify patients with thymoma. Patients with thymoma and AD were compared with patients with thymoma without AD. RESULTS: 47/85 (55%) cases of thymoma had AD, including myasthenia gravis (MG) (n=33), Hashimoto's thyroiditis (n=4), Isaac's syndrome (n=3), Morvan syndrome (n=2), pure red cell aplasia (n=2), systemic lupus (n=2), lichen planus (n=2), and one case of each following conditions: aplastic anemia, autoimmune hemolytic anemia, Good's syndrome, pemphigus, autoimmune hepatitis, Graves' disease, limbic encephalitis, and inflammatory myopathy. Six patients (7%) presented at least 2 ADs. The median duration of follow-up after surgery was 60 months (40-78 months). In 32 patients, the diagnosis of AD preceded the diagnosis of thymoma, in 9 patients, thymoma was diagnosed at the same time as the AD and 7 patients had been operated on when they developed an AD. We found a significative difference on the Masaoka stage between the MG patients and the patients who present another AD (p=0.028). No risk factor for developing an AD after thymectomy was identified. CONCLUSIONS: We describe here the long-term follow-up of a large series of AD related to thymoma. Our results confirm previous data concerning AD occurrence in patients with thymoma and suggest that preexisting autoimmunity is not a risk factor for developing autoimmune manifestations after thymectomy.
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Timoma/etiología , Neoplasias del Timo/etiología , Autoinmunidad , Humanos , Factores de Riesgo , TimectomíaAsunto(s)
Dolor Abdominal/diagnóstico , Astenia/diagnóstico , Enfermedad de Castleman/diagnóstico , Hepatopatías/diagnóstico , Pérdida de Peso , Dolor Abdominal/complicaciones , Reacción de Fase Aguda/diagnóstico , Astenia/complicaciones , Enfermedad de Castleman/complicaciones , Diagnóstico Diferencial , Femenino , Humanos , Hepatopatías/complicaciones , Persona de Mediana Edad , Pérdida de Peso/fisiologíaRESUMEN
AIM: To assess the clinical features and outcome of lymphoma when associated with sarcoidosis and to determine whether this association gives lymphoma a better prognosis. DESIGN: Multicentre retrospective cohort study. METHODS: Retrospective chart review. RESULTS: Twenty-one patients were included (9 males, 12 females). Median age at sarcoidosis diagnosis was 48 years (range: 24-68 years). In 14 cases, lymphoma occurred within a previously known sarcoidosis. Five patients received a concomitant diagnosis of sarcoidosis and lymphoma, whereas lymphoma preceded sarcoidosis in two patients. Three patients were diagnosed with Hodgkin's lymphoma and 18 patients with non-Hodgkin's lymphoma (diffuse large B-cell lymphoma (DLBCL) (n = 11), follicular lymphoma (n = 2), chronic lymphocytic leukemia/small lymphocytic lymphoma (n = 2), anaplastic large cell lymphoma ALK + (n = 1), angioimmunoblastic T-cell lymphoma (n = 1) and T-cell prolymphocytic leukemia (n = 1)). Thirteen patients were alive and in complete remission. Median age at the time of diagnosis of sarcoidosis was lower in patients with concomitant lymphoma compared with patients with sarcoidosis preceding lymphoma (34 years vs. 51 years, P = 0.01). Patients presenting with DLBCL associated with sarcoidosis were compared with DLBCL without sarcoidosis. No statistical difference was found in the risk of death or progression between the two groups (P = 0.685). CONCLUSIONS: We report here the largest series of lymphoma associated sarcoidosis patients. As opposed to previous studies, we observed a predominance of patients with DLBCL. Our study confirms the concept of the sarcoidosis-lymphoma syndrome. Large B-cell lymphoma does not have a better prognosis when associated with sarcoidosis.
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Linfoma/complicaciones , Sarcoidosis/complicaciones , Adolescente , Adulto , Edad de Inicio , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Niño , Preescolar , Supervivencia sin Enfermedad , Femenino , Humanos , Lactante , Linfoma/diagnóstico , Linfoma/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Imagen Multimodal , Tomografía de Emisión de Positrones , Estudios Retrospectivos , Sarcoidosis/diagnóstico , Sarcoidosis/tratamiento farmacológico , Esteroides/uso terapéutico , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Adulto JovenRESUMEN
Adult-onset Still's disease is a rare and difficult to diagnose multisystemic disorder considered as a multigenic autoinflammatory syndrome. Its immunopathogenesis seems to be at the crossroads between inflammasomopathies and hemophagocytic lymphohistiocytosis, the most severe manifestation of the disease. According to recent insights in the pathophysiology and thanks to cohort studies and therapeutic trials, two phenotypes of adult-onset Still's disease may be distinguished: a systemic pattern, initially highly symptomatic and with a higher risk to exhibit life-threatening complications such as reactive hemophagocytic lymphohistiocytosis, where interleukin-1 blockade seems to be very effective, a chronic articular pattern, more indolent with arthritis in the foreground and less severe systemic manifestations, which would threat functional outcome and where interleukin-6 blockade seems to be more effective. This review focuses on these data.
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Enfermedad de Still del Adulto/clasificación , Enfermedad de Still del Adulto/etiología , Enfermedad de Still del Adulto/terapia , Adulto , Progresión de la Enfermedad , Humanos , Factores de Riesgo , Enfermedad de Still del Adulto/diagnósticoRESUMEN
BACKGROUND: Transverse myelitis is a rare complication of systemic lupus erythematosus (SLE). This retrospective multicentre study identifies the prognostic factors in a relatively large patient series. PATIENTS AND METHODS: Twenty patients fulfilled the SLE criteria of the ACR classification and the Transverse Myelitis Consortium Working Group. A severe neurological flare was defined as muscle strength grade <3/5 in more than half the muscle groups at the motor neurological level. Inability to run or another significant ambulation-unrelated disability was considered as 'unfavourable neurological outcome'. RESULTS: Myelitis was the first SLE symptom in 12 patients; in the eight others, it occurred 8.6 years (median delay) after SLE onset. Eleven patients presented severe neurological impairments. The treatment included corticosteroids in all patients associated with intravenous cyclophosphamide in 11 and/or hydroxychloroquine in 14. Unfavourable outcomes were observed in 53% of the patients at six months and in 28% at end of follow-up (median: 5.9 years). An initial severe neurological impairment and no cyclophosphamide use were associated with unfavourable neurological outcomes at six months and at end of follow-up, respectively. CONCLUSION: Transverse myelitis may reveal SLE or occur more than 10 years after SLE diagnosis. The initial severity of the neurological flare (with paraplegia) is the main prognostic marker. The study provides arguments for cyclophosphamide use.
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Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/tratamiento farmacológico , Mielitis Transversa/tratamiento farmacológico , Mielitis Transversa/etiología , Enfermedad Aguda , Corticoesteroides/uso terapéutico , Adulto , Anciano , Antiinflamatorios/uso terapéutico , Antirreumáticos/uso terapéutico , Ciclofosfamida/uso terapéutico , Femenino , Estudios de Seguimiento , Humanos , Hidroxicloroquina/uso terapéutico , Inmunosupresores/uso terapéutico , Masculino , Persona de Mediana Edad , Limitación de la Movilidad , Mielitis Transversa/diagnóstico , Pronóstico , Estudios Retrospectivos , Índice de Severidad de la EnfermedadRESUMEN
PURPOSE: Drug-induced immune haemolytic anemia occurs in one case per million and can be fatal. Our aim was to describe the main characteristics and the type of drug involved. METHODS: Cases were retrospectively identified using spontaneous notifications collected by our pharmacovigilance centre and the results of immuno-hematological investigations performed by the laboratory of French blood establishment of Lyon between 2000 and 2012. Inclusion criteria were: an immune (positive direct antiglobulin test), hemolytic, anemia (haemoglobin <100 g/L), with at least a plausible causal relationship with drug exposure according to the French method of imputability or the presence of drug-dependent antibodies, and exclusion of other causes of hemolysis. RESULTS: Ten cases (5 men and 5 women, median age 54.4 years) were identified. Causal drugs were ambroxol, beta-interferon, cefotetan, ceftriaxone, loratadine, oxacillin, oxaliplatine, piperacillin-tazobactam, pristinamycin, and quinine. The median time to onset of anemia after starting the culprit drug was 6 days (2 hours to 16 days). The median nadir of hemoglobin was 57.9 g/L (range: 34-78). The direct antiglobulin test was positive in 8 patients: IgG only (n=4), IgG and complement (n=3), and IgA (n=1). Drug-induced immune haemolytic anemia was considered as definite in 5 cases with positive drug-induced antibodies, probable in 4 cases negative for the detection of drug-induced antibodies but with plausible or likely causal relationship with drug exposure, and probable with an autoimmune mechanism in 1 case. CONCLUSION: The diagnosis of DIIHA is often difficult because of the similarities with autoimmune haemolytic anemia and the inconstant sensitivity of immunologic tests that sometimes required repetitive assessment.
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Anemia Hemolítica/inducido químicamente , Anemia Hemolítica/inmunología , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos , Anticuerpos/sangre , Femenino , Humanos , Inmunoglobulina A/inmunología , Inmunoglobulina G/inmunología , Masculino , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
PURPOSE: Parvovirus B19 causes erythema infectiosum in children, transient aplastic anemia in patients with hemoglobinopathies, pur red cell aplasia in immunocompromised persons and hydrops fetalis in pregnancy. The spectrum of clinical and biological manifestations in immunocompetent adult continues to grow up. METHODS: We report on a case series of 26 patients with primary parvovirus B19 infection in immunocompetent adults. This is a retrospective study over the period 2000 to 2010 in two departments of internal medecine. The diagnostic was clinical, serological or molecular. RESULTS: There was a female predominance (sex-ratio 3.33/1). Median patient age at diagnostic was 38.8 years (range: 18-68). The predominant symptoms were fever (65%), peripheral and symmetrical polyarthralgia (62%) and skin rash (58%). Two patients had neurological manifestations (sixth cranial nerve palsy, distal paresthesia) and one patient had myocarditis. Abnormal laboratory values included increased acute phase reactants (73%), thrombocytopenia (43%), lymphopenia (38%) and elevated liver enzymes (37%). Antinuclear (19%), anti-DNA (28%) and anti-phospholipids antibodies (14%), and hypocomplementemia (32%) were observed. False reaction with anti-CMV and anti-EBV IgM positivity was documented in 27% of cases. Two patients had persistent parvovirus B19 infection. CONCLUSION: The diversity of the clinical manifestations of parvovirus B19 infection may be misleading for the clinician. However, the diagnosis should be suspected in immunocompetent adults to limit the risk of transmission to the patients who could develop a severe infection such as pregnant women or immunocompromised patients.
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Infecciones por Parvoviridae/diagnóstico , Infecciones por Parvoviridae/epidemiología , Infecciones por Parvoviridae/terapia , Parvovirus B19 Humano , Adolescente , Adulto , Anciano , Autoinmunidad , Femenino , Humanos , Inmunocompetencia , Masculino , Persona de Mediana Edad , Infecciones por Parvoviridae/inmunología , Parvovirus B19 Humano/patogenicidad , Estudios Retrospectivos , Adulto JovenRESUMEN
PURPOSE: Infectious aortic aneurysms are rare conditions, being responsible of 2% of aortic aneurysms. Most published results are surgical case series concerning infected abdominal aorta. In this retrospective study, we assessed clinical features and outcome of patients presenting infectious thoracic aortic aneurysms. PATIENTS AND METHODS: Diagnosis was based upon a combination of imaging evidence for thoracic aorta aneurysm and evidence for an infective aetiology including a culture of a causative pathogen, or a favourable outcome with anti-infective therapy. Retrospective case series. RESULTS: Six men and one woman were included, with a mean age of 66 years. All the patient presented at least one cardiovascular risk factor or atherosclerosis localisation. Fever (71%) and chest pain (42%) were the most common clinical presenting manifestations. The causative pathogens were: Staphylococcus aureus (N=1), Salmonella enteritidis (N=3) and Candida albicans (N=1). The contrast-enhanced computed-tomography disclosed an aneurysm whose diameter reached more than 50 mm (N=5), that increased rapidly in size (N=5), or presented an inflammatory aspect of the aortic wall (N=4). Management was both medical and interventional: surgery (N=3) or endoluminal repair (N=4). Outcome was favourable in six patients; one patient died from aneurysm-related complications. CONCLUSION: Clinical manifestations revealing an infectious thoracic aneurysm are variable. Diagnosis should be considered in patients presenting a rapidly-growing aneurysm, especially in the presence of elevated acute phase reactants. Endoluminal repair constitutes a treatment option. The role of FDG-PET for diagnosis and follow-up remains to be defined.
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Aneurisma Infectado/microbiología , Aneurisma Infectado/cirugía , Aneurisma de la Aorta Torácica/diagnóstico , Aneurisma de la Aorta Torácica/cirugía , Anciano , Anciano de 80 o más Años , Aneurisma Infectado/diagnóstico , Aneurisma Infectado/mortalidad , Antibacterianos/uso terapéutico , Aneurisma de la Aorta Torácica/mortalidad , Prótesis Vascular , Candidiasis/complicaciones , Candidiasis/tratamiento farmacológico , Dolor en el Pecho/etiología , Femenino , Fiebre/etiología , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Infecciones por Salmonella/complicaciones , Infecciones por Salmonella/tratamiento farmacológico , Infecciones Estafilocócicas/complicaciones , Infecciones Estafilocócicas/tratamiento farmacológicoRESUMEN
Little is known about the interactions between adult-onset Still's disease (AOSD) and pregnancy. In an attempt to clarify the link between these 2 conditions, we retrospectively analyzed patients registered as suffering from AOSD seen in our university hospital. A total of 57 patients, among them 30 women, were diagnosed. Ten pregnancies in 8 women were identified. Three cases manifested AOSD in their first trimester, all treated with prednisone. Premature births and flares occurred in 2 patients. One patient developed a monocyclic AOSD during her second pregnancy's postpartum. In the 4 other cases, AOSD was known and quiescent before pregnancy. One patient had 2 pregnancies without any flare or complication. One patient experienced her first pregnancy while under treatment and presented a late flare 8 months after delivery. The third patient developed exacerbation in the first trimester of her second pregnancy which was treated with IgIV alone. The last one presented her first pregnancy 7 years after diagnosis. A prednisone-treated systemic flare occurred during the first trimester without later complication. Based on our own experience and the analysis of only two series of the literature, including, respectively, 4 and 5 patients, we suggest that two settings could be distinguished. First, AOSD can occur during pregnancy and can be responsible for obstetrical complications. Then, in patients with known AOSD, the second trimester and postpartum appear to be periods exposing to disease recurrence. Thus, we recommend a close multidisciplinary monitoring by a rheumatologist and an obstetrician prior to, during and after pregnancies.
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Complicaciones del Embarazo/fisiopatología , Enfermedad de Still del Adulto/fisiopatología , Adulto , Estudios de Cohortes , Progresión de la Enfermedad , Femenino , Glucocorticoides/uso terapéutico , Humanos , Prednisona/uso terapéutico , Embarazo , Complicaciones del Embarazo/tratamiento farmacológico , Resultado del Embarazo , Nacimiento Prematuro , Estudios Retrospectivos , Enfermedad de Still del Adulto/tratamiento farmacológico , Adulto JovenRESUMEN
PURPOSE: To report on the various clinical presentations, etiological diagnosis, prognosis and treatment of patients with scleritis evaluated at a tertiary care eye center. METHODS: Retrospective, monocentric study on a series of 32 patients in a tertiary center. RESULTS: The mean age of included patients with scleritis was 46.8 years (range, 22 to 77 years). Nineteen patients were women and 13 were men. Twenty-six patients (81%) had anterior scleritis (15 nodular, 8 diffuse and 3 necrotizing), six (19%) had posterior scleritis. Unilateral inflammation was present in 24 patients (75%). Twelve out of the 32 patients (37.5%) had an underlying systemic disease: granulomatosis with polyangiitis (n=3), Behçet's disease (n=2), unspecified inflammatory arthritis (n=2), psoriatic arthritis (n=1), ankylosing spondylitis (n=1), sarcoidosis (n=1), Cogan's syndrome (n=1) and ulcerative colitis (n=1). Six patients (18.8%) were suspected of having infectious disease with herpes virus: clinical context and positive treatment response with oral valacyclovir. Systemic agents and topical agents were required in 28 patients (87.5%). The first line therapy was mainly oral non-steroidal anti-inflammatory drugs in 15 patients (47%) and oral corticosteroids in 8 (25%). Immunosuppressive drugs were required in 6 patients. The mean follow-up was 16.3 months. Six patients (19%) had a decrease in visual acuity. CONCLUSION: The number of systemic disease in our series is similar to the main series in the literature. Treatment with valaciclovir might be effective in patients with suspected herpes simplex scleritis.
Asunto(s)
Antiinflamatorios no Esteroideos/uso terapéutico , Glucocorticoides/uso terapéutico , Inmunosupresores/uso terapéutico , Escleritis/tratamiento farmacológico , Escleritis/etiología , Centros Médicos Académicos , Aciclovir/análogos & derivados , Aciclovir/uso terapéutico , Adulto , Anciano , Antivirales/uso terapéutico , Quimioterapia Combinada , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Escleritis/diagnóstico , Escleritis/virología , Resultado del Tratamiento , Valaciclovir , Valina/análogos & derivados , Valina/uso terapéuticoRESUMEN
AIM: To describe the main characteristics and the treatment of cryptococcosis in patients with sarcoidosis. DESIGN: Multicenter study including all patients notified at the French National Reference Center for Invasive Mycoses and Antifungals. METHODS: Retrospective chart review. Each case was compared with two controls without opportunistic infections. RESULTS: Eighteen cases of cryptococcosis complicating sarcoidosis were analyzed (13 men and 5 women). With 2749 cases of cryptococcosis registered in France during the inclusion period of this study, sarcoidosis accounted for 0.6% of all the cryptococcosis patients and for 2.9% of the cryptococcosis HIV-seronegative patients. Cryptococcosis and sarcoidosis were diagnosed concomitantly in four cases; while sarcoidosis was previously known in 14/18 patients, including 12 patients (67%) treated with steroids. The median rate of CD4 T cells was 145 per mm(3) (range: 55-1300) and not related to steroid treatment. Thirteen patients had cryptococcal meningitis (72%), three osteoarticular (17%) and four disseminated infections (22%). Sixteen patients (89%) presented a complete response to antifungal therapy. After a mean follow-up of 6 years, no death was attributable to cryptococcosis. Extra-thoracic sarcoidosis and steroids were independent risk factors of cryptococcosis in a logistic regression model adjusted with the sex of the patients. CONCLUSIONS: Cryptococcosis is a significant opportunistic infection during extra-thoracic sarcoidosis, which occurs in one-third of the cases in patients without any treatment; it is not associated to severe CD4 lymphocytopenia and has a good prognosis.
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Criptococosis/complicaciones , Infecciones Oportunistas/complicaciones , Sarcoidosis/complicaciones , Adolescente , Adulto , Antifúngicos/uso terapéutico , Recuento de Linfocito CD4 , Criptococosis/diagnóstico , Criptococosis/tratamiento farmacológico , Criptococosis/inmunología , Femenino , Glucocorticoides/uso terapéutico , Humanos , Inmunosupresores/uso terapéutico , Masculino , Persona de Mediana Edad , Infecciones Oportunistas/diagnóstico , Infecciones Oportunistas/tratamiento farmacológico , Infecciones Oportunistas/inmunología , Pronóstico , Estudios Retrospectivos , Sarcoidosis/tratamiento farmacológico , Sarcoidosis/inmunología , Adulto JovenRESUMEN
Although primary immunodeficiencies (PID) are typically marked by increased susceptibility to infections, autoimmune manifestations have increasingly been recognized as an important component of several forms of PID. Here, we discuss two forms of PID in which autoimmune cytopenias are particularly common and may be the first manifestation of the disease in adults: autoimmune lymphoproliferative syndrome (ALPS) and common variable immunodeficiency (CVID). Approximately one fifth of patients with CVID develop autoimmune diseases, and immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AHA) are the most common. Since autoimmune cytopenias frequently precede the diagnosis of CVID, testing for immunoglobulin levels should be performed in patients diagnosed with AITP and AHA. Patients with CVID in association with autoimmune cytopenias have a "particular phenotype" with lower susceptibility to infection and higher susceptibility to autoimmune manifestations and, for patients with AHA, a more frequent development of splenomegaly and lymphoma. Corticosteroids and high doses of intravenous immunoglobulins (IVIg) seem to have the same efficacy as in idiopathic AITP and AHA. Splenectomy and rituximab are as effective as in idiopathic autoimmune cytopenias but are associated with an increased risk of severe infection and should, in our opinion, be considered only for those rare patients with "refractory diseases". The course and outcome of autoimmune cytopenias is not affected by supportive IVIg therapy. Autoimmune destruction of blood cells affects over 70% of ALPS patients. The median age of first presentation is 24 months of age, but with increasing awareness of this condition, adults with autoimmune cytopenias are now being diagnosed more frequently. Testing for ALPS should therefore be considered in young adults with unexplained Evan's syndrome. Patients usually respond to immunosuppressive medications, including corticosteroids. Unlike many patients with idiopathic autoimmune cytopenias, the cytopenias in patients with ALPS typically do not respond to IVIg. After corticosteroids, the immunosuppressive drug that is the most studied in ALPS patients is mycophenolate mofetyl. Rituximab and splenectomy are relatively contraindicated in ALPS because of an increase risk of severe infection and should be reserved for patients who fail all other therapies.
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Síndrome Linfoproliferativo Autoinmune/complicaciones , Inmunodeficiencia Variable Común/complicaciones , Corticoesteroides/uso terapéutico , Anemia Hemolítica Autoinmune/complicaciones , Anemia Hemolítica Autoinmune/tratamiento farmacológico , Síndrome Linfoproliferativo Autoinmune/tratamiento farmacológico , Inmunodeficiencia Variable Común/tratamiento farmacológico , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Inmunosupresores/uso terapéutico , Púrpura Trombocitopénica Idiopática/complicaciones , Púrpura Trombocitopénica Idiopática/tratamiento farmacológicoRESUMEN
AIM: To assess clinical features, treatment and outcome of Hypothalamo-pituitary (HP) sarcoidosis and to determine whether HP is associated with a particular clinical phenotype of sarcoidosis. DESIGN: Multicentric retrospective study. METHODS: Retrospective chart review. Each patient was matched with two controls. RESULTS: Twenty-four patients were identified (10 women, 14 men). Their median age at the sarcoidosis diagnosis was 31.5 years (range: 8-69 years). HP involvement occurred in the course of a previously known sarcoidosis in 11 cases (46%), whereas it preceded the diagnosis in 13 patients (54%). All but two patients had anterior pituitary dysfunction, 12 patients presented with diabetes insipidus. The most common hormonal features were gonadotropin deficiency (n=21), TSH deficiency (n=15) and hyperprolactinemia (n=12). Magnetic Resonance Imaging (MRI) revealed infundibulum involvement (n=8), pituitary stalk thickness (n=12) and involvement of the pituitary gland (n=14). All but two patients received prednisone. After a mean follow-up of 4 years, only two patients recovered from hormonal deficiencies. MRI abnormalities improved or disappeared in 12 cases under corticosteroid. There was no correlation between the hormonal dysfunctions and the radiologic outcomes. Patients with HP sarcoidosis had significantly more frequent sinonasal localizations and neurosarcoidosis and required a systemic treatment more frequently than controls. CONCLUSION: Although HP sarcoidosis is unusual, physicians should be aware that such specific localization could be the first manifestation of sarcoidosis. HP involvement is associated with general severity of sarcoidosis. MRI abnormalities can improve or disappear under corticosteroid treatment, but most endocrine defects are irreversible.
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Enfermedades del Sistema Nervioso Central , Enfermedades Hipotalámicas , Hormonas Hipotalámicas , Sistema Hipotálamo-Hipofisario/efectos de los fármacos , Hormonas Hipofisarias , Sarcoidosis , Adulto , Anciano , Biopsia , Estudios de Casos y Controles , Enfermedades del Sistema Nervioso Central/diagnóstico , Enfermedades del Sistema Nervioso Central/tratamiento farmacológico , Enfermedades del Sistema Nervioso Central/metabolismo , Enfermedades del Sistema Nervioso Central/fisiopatología , Niño , Monitoreo de Drogas , Femenino , Glucocorticoides/administración & dosificación , Humanos , Enfermedades Hipotalámicas/diagnóstico , Enfermedades Hipotalámicas/tratamiento farmacológico , Enfermedades Hipotalámicas/metabolismo , Enfermedades Hipotalámicas/fisiopatología , Hormonas Hipotalámicas/análisis , Hormonas Hipotalámicas/metabolismo , Sistema Hipotálamo-Hipofisario/fisiopatología , Hipotálamo/metabolismo , Hipotálamo/patología , Imagen por Resonancia Magnética/métodos , Masculino , Hipófisis/metabolismo , Hipófisis/patología , Hormonas Hipofisarias/análisis , Hormonas Hipofisarias/metabolismo , Prednisona/administración & dosificación , Sarcoidosis/diagnóstico , Sarcoidosis/tratamiento farmacológico , Sarcoidosis/metabolismo , Sarcoidosis/fisiopatología , Resultado del TratamientoRESUMEN
OBJECTIVE: To describe effectiveness, steroid-sparing effect, and tolerance of the antiproliferative immunosuppressant mycophenolate mofetil (MMF) in neurosarcoidosis. METHODS: We describe a retrospective case series of 10 consecutive patients with a diagnosis of neurosarcoidosis who were treated with MMF, alone or in association with corticosteroids, in our teaching hospital. RESULTS: At the time of our study, the mean duration of MMF treatment was 21 months. All but one patient with CNS involvement (n = 8) were in remission (except for hormonal dysfunction) which was complete in 6 patients. MMF was efficient as single-agent induction therapy in one patient. The 3 patients who received MMF as a maintenance therapy after initial response to corticosteroids did not relapse even though steroids were stopped. Out of 4 subjects who demonstrated insufficient response to prior therapy including corticosteroids and immunosuppressive agents, 3 demonstrated significant clinical and radiologic improvement. However, the 2 patients who presented muscular sarcoidosis did not respond to MMF. Among patients treated with steroids at MMF introduction and after excluding those with sarcoid myopathy, the mean dose of corticosteroids was 6 mg/day at the end of the follow-up while it was 59 mg/day at the initiation of MMF. No significant side effects were observed. CONCLUSIONS: These data suggest that MMF is effective in CNS sarcoidosis but not in sarcoid myopathy, with a corticosteroid sparing effect and a better tolerance profile than other immunosuppressive agents.