RESUMEN

Se presenta el caso de un varón de 66años remitido por uveítis posterior con edema macular quístico recurrente en relación con posible enfermedad de Lyme previamente tratada. Debido a la recurrencia del edema macular pese al tratamiento corticoideo sistémico y local con dexametasona intravítrea, se instaura tratamiento biológico con adalimumab. Durante el seguimiento el paciente desarrolla lesiones subretinianas bilaterales compatibles con linfoma vitreorretiniano, por lo que se realiza vitrectomía, confirmando el diagnóstico de linfoma de célulaB grande.Se inicia tratamiento con quimioterapia sistémica con BRAM (carmustina, metotrexato, ara-C y rituximab), con buena respuesta. Dos años después, el paciente permanece sin recurrencias a nivel ocular ni sistémico.El linfoma vitreorretiniano es un tipo poco frecuente de linfoma primario del sistema nervioso central. El diagnóstico se ve frecuentemente retrasado debido a la inespecificidad de su sintomatología, que simula una uveítis posterior crónica; de ahí la importancia de la sospecha diagnóstica. (AU)

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A 66-year-old man with posterior uveitis and recurrent cystic macular edema related to possible previously treated Lyme disease is presented. Due to the recurrence of macular edema despite systemic and local corticosteroid treatment with intravitreal dexamethasone, biological treatment with adalimumab was established. During follow-up, the patient developed bilateral subretinal lesions compatible with vitreoretinal lymphoma, so vitrectomy was performed, confirming the diagnosis of large B-cell lymphoma.Treatment with systemic chemotherapy with BRAM (carmustine, metotrexate, ara C, and rituximab) was started with a good answer. Two years later, the patient remains without ocular or systemic recurrences.Vitreoretinal lymphoma is a rare type of primary central nervous system lymphoma. The diagnosis is frequently delayed due to the nonspecific symptoms, which mimic chronic posterior uveitis, hence the importance with a diagnostic suspicion. (AU)

Asunto(s)

Humanos , Masculino , Anciano , Neoplasias de la Retina/diagnóstico , Linfoma de Células B/diagnóstico , Cirugía Vitreorretiniana

RESUMEN

A 66-year-old man with posterior uveitis and recurrent cystic macular edema related to possible previously treated Lyme disease is presented. Due to the recurrence of macular edema despite systemic and local corticosteroid treatment with intravitreal dexamethasone, biological treatment with Adalimumab was established. During follow-up, the patient developed bilateral subretinal lesions compatible with Vitreoretinal Lymphoma (VRL), so vitrectomy was performed, confirming the diagnosis of large B-cell lymphoma. Treatment with systemic chemotherapy with BRAM-Carmustine, Metrotexate, Ara C, and Rituximab was started with a good answer. Two years later, the patient remains without ocular or systemic recurrences. Vitreoretinal Lymphoma is a rare type of primary central nervous system lymphoma. The diagnosis is frequently delayed due to the nonspecific symptoms, which mimic chronic posterior uveitis, hence the importance with a diagnostic suspicion.

Asunto(s)

Neoplasias del Ojo , Linfoma de Células B Grandes Difuso , Edema Macular , Neoplasias de la Retina , Uveítis Posterior , Masculino , Humanos , Anciano , Neoplasias de la Retina/patología , Cuerpo Vítreo , Edema Macular/diagnóstico , Edema Macular/etiología , Neoplasias del Ojo/diagnóstico , Neoplasias del Ojo/patología , Linfoma de Células B Grandes Difuso/diagnóstico por imagen , Linfoma de Células B Grandes Difuso/cirugía

RESUMEN

To analyze the clinical characteristics and outcomes of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in patients with sarcoidosis from a large multicenter cohort from Southern Europe and to identify the risk factors associated with a more complicated infection. We searched for patients with sarcoidosis presenting with SARS-CoV-2 infection (defined according to the European Centre for Disease Prevention and Control guidelines) among those included in the SarcoGEAS Registry, a nationwide, multicenter registry of patients fulfilling the American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and Other Granulomatous Disorders 1999 classification criteria for sarcoidosis. A 2:1 age-sex-matched subset of patients with sarcoidosis without SARS-CoV-2 infection was selected as control population. Forty-five patients with SARS-CoV-2 infection were identified (28 women, mean age 55 years). Thirty-six patients presented a symptomatic SARS-CoV-2 infection and 14 were hospitalized (12 required supplemental oxygen, 2 intensive care unit admission and 1 mechanical ventilation). Four patients died due to progressive respiratory failure. Patients who required hospital admission had an older mean age (64.9 vs. 51.0 years, p = 0.006), a higher frequency of baseline comorbidities including cardiovascular disease (64% vs. 23%, p = 0.016), diabetes mellitus (43% vs. 13%, p = 0.049) and chronic liver/kidney diseases (36% vs. 0%, p = 0.002) and presented more frequently fever (79% vs. 35%, p = 0.011) and dyspnea (50% vs. 3%, p = 0.001) in comparison with patients managed at home. Age- and sex-adjusted multivariate analysis identified the age at diagnosis of SARS-Cov-2 infection as the only independent variable associated with hospitalization (adjusted odds ratio 1.18, 95% conficence interval 1.04-1.35). A baseline moderate/severe pulmonary impairment in function tests was associated with a higher rate of hospitalization but the difference was not statistically significant (50% vs. 23%, p = 0.219). A close monitoring of SARS-CoV-2 infection in elderly patients with sarcoidosis, especially in those with baseline cardiopulmonary diseases and chronic liver or renal failure, is recommended. The low frequency of severe pulmonary involvement in patients with sarcoidosis from Southern Europe may explain the weak prognostic role of baseline lung impairment in our study, in contrast to studies from other geographical areas.

Asunto(s)

COVID-19/complicaciones , Sarcoidosis/complicaciones , Adulto , Anciano , Anciano de 80 o más Años , COVID-19/mortalidad , COVID-19/fisiopatología , COVID-19/terapia , Estudios de Cohortes , Comorbilidad , Femenino , Francia , Hospitalización/estadística & datos numéricos , Humanos , Masculino , Persona de Mediana Edad , Sistema de Registros , Sarcoidosis/mortalidad , Sarcoidosis/fisiopatología , Sarcoidosis/terapia , Resultado del Tratamiento

RESUMEN

BACKGROUND: To analyze the association between Scadding radiological stages of sarcoidosis at diagnosis and the disease phenotype (epidemiology, clinical presentation and extrathoracic involvement) in one of the largest cohorts of patients with sarcoidosis reported from southern Europe. METHODS: The SARCOGEAS-Study Group includes a multicenter database of consecutive patients diagnosed with sarcoidosis according to the WASOG 1999 criteria. Extrathoracic disease at diagnosis was defined according to the 2014 instrument and the clusters proposed by Schupp et al. RESULTS: We analyzed 1230 patients (712 female, mean age 47 yrs.) who showed the following Scadding radiologic stages at diagnosis: stage 0 (n = 98), stage I (n = 395), stage II (n = 500), stage III (n = 195) and stage IV (n = 42). Women were overrepresented in patients presenting with extrathoracic/extrapulmonary disease, while the diagnosis was made at younger ages in patients presenting with BHL, and at older ages in those presenting with pulmonary fibrosis (q values <0.05). Multivariable adjusted analysis showed that patients presenting with pulmonary involvement (especially those with stages II and III) had a lower frequency of concomitant systemic involvement in some specific extrathoracic clusters (cutaneous-adenopathic/musculoskeletal, ENT and neuro-ocular/OCCC) but a higher frequency for others (hepatosplenic), in comparison with patients with extrapulmonary involvement (stages 0 and I). The presence of either BHL or fibrotic lesions did not influence the systemic phenotype of patients with pulmonary involvement. CONCLUSIONS: The key determinant associated with a differentiated systemic phenotype of sarcoidosis at diagnosis was interstitial pulmonary involvement rather than the individual Scadding radiological stage.

Asunto(s)

Sarcoidosis/diagnóstico por imagen , Femenino , Humanos , Masculino , Persona de Mediana Edad , Fenotipo , Radiografía , Sarcoidosis/complicaciones , Sarcoidosis/genética

RESUMEN

OBJECTIVES: To analyse the safety and efficacy of the off-label use of rituximab in patients with severe, refractory systemic autoimmune diseases. METHODS: In 2006, the Study Group on Autoimmune Diseases of the Spanish Society of Internal Medicine created the BIOGEAS project, a multicenter study devoted to collecting data on the use of biological agents in adult patients with systemic autoimmune diseases refractory to standard therapies (failure of at least two immunosuppressive agents). RESULTS: One hundred and ninety-six patients with systemic autoimmune diseases treated with rituximab have been included in the Registry (158 women and 38 men, mean age 43 years). Systemic autoimmune diseases included systemic lupus erythematosus (107 cases), inflammatory myopathies (20 cases), ANCA-related vasculitides (19 cases), Sjögren's syndrome (15 cases) and other diseases (35 cases). A therapeutic response was evaluable in 194 cases: 99 (51%) achieved a complete response, 51 (26%) a partial response and 44 (23%) were classified as non-responders. After a mean follow-up of 27.56+/-1.32 months, 44 (29%) out of the 150 responders patients relapsed. There were 40 adverse events reported in 33 (16%) of the 196 patients. The most frequent adverse events were infections, with 24 episodes being described in 19 patients. Thirteen (7%) patients died, mainly due to disease progression (7 cases) and infection (3 cases). CONCLUSIONS: Although not yet licensed for this use, rituximab is currently used to treat severe, refractory systemic autoimmune diseases, with the most favourable results being observed in Sjögren's syndrome, inflammatory myopathies, systemic lupus erythematosus and cryoglobulinemia.

Asunto(s)

Anticuerpos Monoclonales de Origen Murino/uso terapéutico , Enfermedades Autoinmunes/tratamiento farmacológico , Factores Inmunológicos/uso terapéutico , Uso Fuera de lo Indicado , Índice de Severidad de la Enfermedad , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/tratamiento farmacológico , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/etnología , Anticuerpos Monoclonales de Origen Murino/efectos adversos , Enfermedades Autoinmunes/etnología , Crioglobulinemia/tratamiento farmacológico , Crioglobulinemia/etnología , Femenino , Humanos , Factores Inmunológicos/efectos adversos , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/etnología , Masculino , Persona de Mediana Edad , Miositis/tratamiento farmacológico , Miositis/etnología , Estudios Retrospectivos , Rituximab , Síndrome de Sjögren/tratamiento farmacológico , Síndrome de Sjögren/etnología , España , Resultado del Tratamiento , Adulto Joven

RESUMEN

No disponible

Asunto(s)

Humanos , Masculino , Persona de Mediana Edad , Dolor Abdominal/etiología , Policitemia Vera/complicaciones , Policitemia Vera/diagnóstico , Fiebre/etiología , Esplenomegalia/complicaciones , Dolor Abdominal/complicaciones , Fiebre/complicaciones

Asunto(s)

Adenocarcinoma/complicaciones , Aglutininas/inmunología , Anemia Hemolítica Autoinmune/complicaciones , Neoplasias del Colon/complicaciones , Adenocarcinoma/inmunología , Anciano , Anemia Hemolítica Autoinmune/inmunología , Neoplasias del Colon/inmunología , Crioglobulinas , Humanos , Masculino

RESUMEN

No disponible

Asunto(s)

Masculino , Anciano , Humanos , Anemia Hemolítica Autoinmune/complicaciones , Neoplasias del Colon/complicaciones , Adenocarcinoma/patología , Autoanticuerpos/análisis

Asunto(s)

Neoplasias de los Conductos Biliares/inducido químicamente , Colangiocarcinoma/inducido químicamente , Medios de Contraste/efectos adversos , Dióxido de Torio/efectos adversos , Anciano , Neoplasias de los Conductos Biliares/diagnóstico , Conductos Biliares Intrahepáticos , Colangiocarcinoma/diagnóstico , Humanos , Masculino

RESUMEN

No disponible

Asunto(s)

Anciano , Humanos , Masculino , Colangiocarcinoma , Conductos Biliares Intrahepáticos , Medios de Contraste , Dióxido de Torio , Conductos Biliares Intrahepáticos , Neoplasias de los Conductos Biliares

Asunto(s)

Amiloidosis/complicaciones , Bocio Nodular/complicaciones , Enfermedades de las Paratiroides/complicaciones , Anciano , Anciano de 80 o más Años , Amiloide/metabolismo , Amiloidosis/patología , Amiloidosis/terapia , Femenino , Bocio Nodular/patología , Bocio Nodular/terapia , Humanos , Enfermedades de las Paratiroides/patología , Glándula Tiroides/patología , Tiroidectomía , Resultado del Tratamiento

RESUMEN

No disponible

Asunto(s)

Humanos , Anciano , Femenino , Anciano de 80 o más Años , Tiroidectomía , Resultado del Tratamiento , Glándula Tiroides , Enfermedades de las Paratiroides , Amiloidosis , Amiloide , Bocio Nodular

Asunto(s)

Anticoagulantes/efectos adversos , Enoxaparina/efectos adversos , Hematoma/inducido químicamente , Hemorragia/inducido químicamente , Espacio Retroperitoneal/patología , Anciano , Anticoagulantes/administración & dosificación , Enoxaparina/administración & dosificación , Femenino , Humanos , Espacio Retroperitoneal/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Resultado del Tratamiento

RESUMEN

No disponible

Asunto(s)

Anciano , Femenino , Humanos , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Enoxaparina , Espacio Retroperitoneal , Anticoagulantes , Hemorragia , Hematoma

Asunto(s)

Anafilaxia/parasitología , Equinococosis Hepática/complicaciones , Echinococcus/aislamiento & purificación , Albendazol/uso terapéutico , Anafilaxia/tratamiento farmacológico , Anafilaxia/inmunología , Animales , Anticuerpos Antihelmínticos/sangre , Antiprotozoarios/uso terapéutico , Procedimientos Quirúrgicos del Sistema Digestivo/métodos , Equinococosis Hepática/tratamiento farmacológico , Equinococosis Hepática/inmunología , Echinococcus/inmunología , Humanos , Hígado/diagnóstico por imagen , Hígado/parasitología , Masculino , Persona de Mediana Edad , Resultado del Tratamiento , Ultrasonografía

Asunto(s)

Médula Ósea/patología , Leucemia de Células Plasmáticas/etiología , Paraproteinemias/complicaciones , Anciano , Resultado Fatal , Humanos , Inmunoglobulinas/sangre , Leucemia de Células Plasmáticas/patología , Leucemia de Células Plasmáticas/terapia , Infiltración Leucémica , Masculino , Paraproteinemias/diagnóstico , Paraproteinemias/terapia

Asunto(s)

Artralgia/etiología , Enfermedad de Lyme/complicaciones , Enfermedades Musculares/etiología , Parálisis Respiratoria/etiología , Humanos , Masculino , Persona de Mediana Edad

RESUMEN

No disponible

Asunto(s)

Anciano , Masculino , Humanos , Infiltración Leucémica , Resultado Fatal , Paraproteinemias , Leucemia de Células Plasmáticas , Inmunoglobulinas , Médula Ósea

RESUMEN

No disponible

Asunto(s)

Persona de Mediana Edad , Animales , Masculino , Humanos , Procedimientos Quirúrgicos del Sistema Digestivo , Albendazol , Resultado del Tratamiento , Antiprotozoarios , Anticuerpos Antihelmínticos , Echinococcus , Equinococosis Hepática , Anafilaxia , Hígado

RESUMEN

No disponible

Asunto(s)

Persona de Mediana Edad , Masculino , Humanos , Artralgia , Enfermedades Musculares , Parálisis Respiratoria , Enfermedad de Lyme