ABSTRACT
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)
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)
Subject(s)
Humans , Male , Aged , Retinal Neoplasms/diagnosis , Lymphoma, B-Cell/diagnosis , Vitreoretinal SurgeryABSTRACT
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.
Subject(s)
Eye Neoplasms , Lymphoma, Large B-Cell, Diffuse , Macular Edema , Retinal Neoplasms , Uveitis, Posterior , Male , Humans , Aged , Retinal Neoplasms/pathology , Vitreous Body , Macular Edema/diagnosis , Macular Edema/etiology , Eye Neoplasms/diagnosis , Eye Neoplasms/pathology , Lymphoma, Large B-Cell, Diffuse/diagnostic imaging , Lymphoma, Large B-Cell, Diffuse/surgeryABSTRACT
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.
Subject(s)
COVID-19/complications , Sarcoidosis/complications , Adult , Aged , Aged, 80 and over , COVID-19/mortality , COVID-19/physiopathology , COVID-19/therapy , Cohort Studies , Comorbidity , Female , France , Hospitalization/statistics & numerical data , Humans , Male , Middle Aged , Registries , Sarcoidosis/mortality , Sarcoidosis/physiopathology , Sarcoidosis/therapy , Treatment OutcomeABSTRACT
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 47â¯yrs.) who showed the following Scadding radiologic stages at diagnosis: stage 0 (nâ¯=â¯98), stage I (nâ¯=â¯395), stage II (nâ¯=â¯500), stage III (nâ¯=â¯195) and stage IV (nâ¯=â¯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.
Subject(s)
Sarcoidosis/diagnostic imaging , Female , Humans , Male , Middle Aged , Phenotype , Radiography , Sarcoidosis/complications , Sarcoidosis/geneticsABSTRACT
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.
Subject(s)
Antibodies, Monoclonal, Murine-Derived/therapeutic use , Autoimmune Diseases/drug therapy , Immunologic Factors/therapeutic use , Off-Label Use , Severity of Illness Index , Adolescent , Adult , Aged , Aged, 80 and over , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/ethnology , Antibodies, Monoclonal, Murine-Derived/adverse effects , Autoimmune Diseases/ethnology , Cryoglobulinemia/drug therapy , Cryoglobulinemia/ethnology , Female , Humans , Immunologic Factors/adverse effects , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/ethnology , Male , Middle Aged , Myositis/drug therapy , Myositis/ethnology , Retrospective Studies , Rituximab , Sjogren's Syndrome/drug therapy , Sjogren's Syndrome/ethnology , Spain , Treatment Outcome , Young AdultABSTRACT
No disponible
Subject(s)
Humans , Male , Middle Aged , Abdominal Pain/etiology , Polycythemia Vera/complications , Polycythemia Vera/diagnosis , Fever/etiology , Splenomegaly/complications , Abdominal Pain/complications , Fever/complicationsABSTRACT
No disponible
Subject(s)
Male , Aged , Humans , Anemia, Hemolytic, Autoimmune/complications , Colonic Neoplasms/complications , Adenocarcinoma/pathology , Autoantibodies/analysisABSTRACT
No disponible
Subject(s)
Aged , Humans , Male , Cholangiocarcinoma , Bile Ducts, Intrahepatic , Contrast Media , Thorium Dioxide , Bile Ducts, Intrahepatic , Bile Duct NeoplasmsSubject(s)
Amyloidosis/complications , Goiter, Nodular/complications , Parathyroid Diseases/complications , Aged , Aged, 80 and over , Amyloid/metabolism , Amyloidosis/pathology , Amyloidosis/therapy , Female , Goiter, Nodular/pathology , Goiter, Nodular/therapy , Humans , Parathyroid Diseases/pathology , Thyroid Gland/pathology , Thyroidectomy , Treatment OutcomeABSTRACT
No disponible
Subject(s)
Humans , Aged , Female , Aged, 80 and over , Thyroidectomy , Treatment Outcome , Thyroid Gland , Parathyroid Diseases , Amyloidosis , Amyloid , Goiter, NodularSubject(s)
Anticoagulants/adverse effects , Enoxaparin/adverse effects , Hematoma/chemically induced , Hemorrhage/chemically induced , Retroperitoneal Space/pathology , Aged , Anticoagulants/administration & dosage , Enoxaparin/administration & dosage , Female , Humans , Retroperitoneal Space/diagnostic imaging , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
No disponible
Subject(s)
Aged , Female , Humans , Tomography, X-Ray Computed , Treatment Outcome , Enoxaparin , Retroperitoneal Space , Anticoagulants , Hemorrhage , HematomaSubject(s)
Anaphylaxis/parasitology , Echinococcosis, Hepatic/complications , Echinococcus/isolation & purification , Albendazole/therapeutic use , Anaphylaxis/drug therapy , Anaphylaxis/immunology , Animals , Antibodies, Helminth/blood , Antiprotozoal Agents/therapeutic use , Digestive System Surgical Procedures/methods , Echinococcosis, Hepatic/drug therapy , Echinococcosis, Hepatic/immunology , Echinococcus/immunology , Humans , Liver/diagnostic imaging , Liver/parasitology , Male , Middle Aged , Treatment Outcome , UltrasonographySubject(s)
Bone Marrow/pathology , Leukemia, Plasma Cell/etiology , Paraproteinemias/complications , Aged , Fatal Outcome , Humans , Immunoglobulins/blood , Leukemia, Plasma Cell/pathology , Leukemia, Plasma Cell/therapy , Leukemic Infiltration , Male , Paraproteinemias/diagnosis , Paraproteinemias/therapyABSTRACT
No disponible
Subject(s)
Aged , Male , Humans , Leukemic Infiltration , Fatal Outcome , Paraproteinemias , Leukemia, Plasma Cell , Immunoglobulins , Bone MarrowABSTRACT
No disponible
Subject(s)
Middle Aged , Animals , Male , Humans , Digestive System Surgical Procedures , Albendazole , Treatment Outcome , Antiprotozoal Agents , Antibodies, Helminth , Echinococcus , Echinococcosis, Hepatic , Anaphylaxis , LiverABSTRACT
No disponible
