RESUMEN
Tumors can induce systemic disturbances in distant organs, leading to physiological changes that enhance host morbidity. In Drosophila cancer models, tumors have been known for decades to cause hypervolemic "bloating" of the abdominal cavity. Here we use allograft and transgenic tumors to show that hosts display fluid retention associated with autonomously defective secretory capacity of fly renal tubules, which function analogous to those of the human kidney. Excretion from these organs is blocked by abnormal cells that originate from inappropriate activation of normally quiescent renal stem cells (RSCs). Blockage is initiated by IL-6-like oncokines that perturb renal water-transporting cells and trigger a damage response in RSCs that proceeds pathologically. Thus, a chronic inflammatory state produced by the tumor causes paraneoplastic fluid dysregulation by altering cellular homeostasis of host renal units.
Asunto(s)
Modelos Animales de Enfermedad , Células Madre , Animales , Células Madre/metabolismo , Inflamación/patología , Humanos , Túbulos Renales/patología , Túbulos Renales/metabolismo , Drosophila melanogaster , Enfermedades Renales/patología , Enfermedades Renales/etiología , Enfermedades Renales/inmunología , Riñón/patología , Riñón/metabolismo , Síndromes Paraneoplásicos/inmunología , Síndromes Paraneoplásicos/metabolismo , Síndromes Paraneoplásicos/patología , Animales Modificados Genéticamente , Interleucina-6/metabolismo , DrosophilaRESUMEN
Waldenstrom macroglobulinaemia (WM), the predominant subtype of lymphoplasmacytic lymphoma with bone marrow involvement and serum IgM paraprotein, is a haematological condition commonly associated with renal parenchymal involvement. However, antineutrophil cytoplasmic antibody (ANCA)-negative pauci-immune crescentic glomerulonephritis (PICGN) in kidney infiltrated by lymphoma is very rare, with only two cases described in chronic lymphocytic leukaemia in English literature so far. We herein report the first patient with WM developing ANCA-negative PICGN. He was a 76-year-old male who presented with elevated serum globulin level and bilateral groin lymph node enlargement, subsequently diagnosed to have WM after pathologic examination of the bone marrow and groin lymph node. One month later, he was found to have acute kidney injury and proteinuria. Renal biopsy confirmed the presence of parenchymal involvement by WM accompanied by PICGN; while ANCA testing was negative. He was treated with pulse methylprednisolone followed by oral prednisolone. In addition, six courses of intravenous rituximab and oral cyclophosphamide were given. There was significant improvement in both his renal and haematological conditions. The clinical course of this case suggested that ANCA-negative PICGN may represent a paraneoplastic syndrome and a rare manifestation of WM-associated renal lesion. Early kidney biopsy and prompt treatment may improve the outcome of patients.
Asunto(s)
Anticuerpos Anticitoplasma de Neutrófilos , Glomerulonefritis , Macroglobulinemia de Waldenström , Humanos , Masculino , Anciano , Macroglobulinemia de Waldenström/complicaciones , Macroglobulinemia de Waldenström/inmunología , Macroglobulinemia de Waldenström/diagnóstico , Macroglobulinemia de Waldenström/tratamiento farmacológico , Glomerulonefritis/inmunología , Glomerulonefritis/tratamiento farmacológico , Glomerulonefritis/diagnóstico , Glomerulonefritis/etiología , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Resultado del Tratamiento , Biopsia , Ciclofosfamida/uso terapéutico , Ciclofosfamida/administración & dosificación , Rituximab/uso terapéutico , Inmunosupresores/uso terapéutico , Síndromes Paraneoplásicos/inmunología , Síndromes Paraneoplásicos/etiología , Síndromes Paraneoplásicos/diagnóstico , Síndromes Paraneoplásicos/tratamiento farmacológico , Biomarcadores/sangreRESUMEN
Paraneoplastic syndromes such as dermatomyositis, often emerge as the initial clinical manifestation across various cancer types and are characterized by the development of B-cell responses targeting cancer-cell antigens that cross-react with normal skin and muscle cells. While these syndromes may alleviate following antineoplastic intervention, their response to immunotherapy remains elusive due to the exclusion of patients with autoimmune phenomena from clinical trials. In this report, we present the case of a female patient with advanced urothelial cancer presenting with dermatomyositis, who subsequently underwent treatment with anti-PD1 immunotherapy and experienced the onset of Stevens-Johnson syndrome. We discuss these two autoimmune entities and provide a comprehensive review of the existing literature to elucidate similar associations.
Dermatomyositis, an inflammatory disorder that causes a skin rash, might be the first sign that someone has cancer. But when scientists test new cancer treatments, they often don't include people with this skin problem. So, we do not know much about how safe or effective these treatments are for them. Here's a story about someone who had bladder cancer and dermatomyositis. They received a treatment called immunotherapy, but it caused a serious problem called Stevens-Johnson syndrome. We also found similar cases in medical papers.
Asunto(s)
Dermatomiositis , Inmunoterapia , Síndromes Paraneoplásicos , Síndrome de Stevens-Johnson , Femenino , Humanos , Dermatomiositis/inmunología , Dermatomiositis/terapia , Inhibidores de Puntos de Control Inmunológico/efectos adversos , Inhibidores de Puntos de Control Inmunológico/uso terapéutico , Inmunoterapia/efectos adversos , Inmunoterapia/métodos , Síndromes Paraneoplásicos/etiología , Síndromes Paraneoplásicos/inmunología , Síndromes Paraneoplásicos/terapia , Síndrome de Stevens-Johnson/terapia , Síndrome de Stevens-Johnson/etiologíaRESUMEN
Paraneoplastic pemphigus is a rare, life-threatening autoimmune disease that is clinically characterized by mostly extensive and refractory mucosal erosions and polymorphous skin lesions. We report here on a 16-year-old girl with isolated oral erosions, in whom mucosal pemphigoid was initially suspected and after treatment with prednisolone and dapsone marked improvement was achieved. However, a few months later the patient developed massive respiratory insufficiency as a result of bronchiolitis obliterans, so that a lung transplant was planned. As part of the preparatory diagnostic workup, unicentric, abdominally localized Castleman's disease was diagnosed, which ultimately led to the diagnosis of paraneoplastic pemphigus as evidenced by envoplakin autoantibodies. Tumor resection and subsequent lung transplantation achieved good results with sustained mucocutaneous remission.
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Enfermedad de Castleman , Síndromes Paraneoplásicos , Pénfigo , Humanos , Femenino , Enfermedad de Castleman/complicaciones , Enfermedad de Castleman/inmunología , Enfermedad de Castleman/diagnóstico , Enfermedad de Castleman/tratamiento farmacológico , Enfermedad de Castleman/patología , Adolescente , Pénfigo/diagnóstico , Pénfigo/inmunología , Pénfigo/complicaciones , Pénfigo/patología , Pénfigo/tratamiento farmacológico , Síndromes Paraneoplásicos/diagnóstico , Síndromes Paraneoplásicos/inmunología , Síndromes Paraneoplásicos/patología , Trasplante de Pulmón , Bronquiolitis Obliterante/inmunología , Bronquiolitis Obliterante/diagnóstico , Bronquiolitis Obliterante/etiología , Bronquiolitis Obliterante/tratamiento farmacológicoRESUMEN
BACKGROUND: Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disorder that may be drug-induced or paraneoplastic. We aim to characterize features of LABD and determine differentiating factors among idiopathic, drug-induced, or malignancy-associated diseases. METHODS: We conducted a single-center retrospective chart review of adult patients with linear IgA bullous dermatosis at a large tertiary referral center and a literature review of adult linear IgA bullous dermatosis. RESULTS: Eighty-one patients were included in the study. Ten patients (12.3%) had comorbid malignancy and nine (11.1%) had inflammatory bowel disease. Median disease duration was significantly shorter in both drug-induced (1.2 vs. 48.8 months; P < 0.001) and malignancy-associated (1.7 vs. 48.8 months; P < 0.001) LABD compared with idiopathic LABD. Recurrent episodes occurred significantly more often in idiopathic LABD compared to those with drug-induced (76.1 vs. 11.5%; P < 0.001) or malignancy-associated disease (76.1 vs. 33.3%; P = 0.019). Time to diagnosis was significantly shorter in the drug-induced (0.2 vs. 5.4 months; P < 0.001) and malignancy-associated groups (0.7 vs. 5.4 months; P = 0.049) compared with idiopathic; similarly, time to improvement was significantly shorter in both drug-induced (0.4 vs. 3.0 months; P < 0.001) and malignancy-associated disease (1.1 vs. 3.0 months; P = 0.016). Clinical morphology was indistinguishable between groups. Limitations included retrospective data collection, data from tertiary referral centers, and limited racial and ethnic diversity. CONCLUSION: Screening for underlying malignancy, as well as for a predisposing medication or possibly inflammatory bowel disease, may be advisable in patients with LABD, particularly when it is newly diagnosed.
Asunto(s)
Dermatosis Bullosa IgA Lineal , Adulto , Femenino , Humanos , Masculino , Edad de Inicio , Erupciones por Medicamentos/etiología , Erupciones por Medicamentos/diagnóstico , Erupciones por Medicamentos/epidemiología , Enfermedades Inflamatorias del Intestino/complicaciones , Enfermedades Inflamatorias del Intestino/tratamiento farmacológico , Dermatosis Bullosa IgA Lineal/diagnóstico , Dermatosis Bullosa IgA Lineal/tratamiento farmacológico , Dermatosis Bullosa IgA Lineal/epidemiología , Neoplasias/complicaciones , Síndromes Paraneoplásicos/inmunología , Síndromes Paraneoplásicos/diagnóstico , Síndromes Paraneoplásicos/etiología , Recurrencia , Estudios RetrospectivosAsunto(s)
Autoanticuerpos , Desmogleína 3 , Linfoma Folicular , Síndromes Paraneoplásicos , Pénfigo , Humanos , Pénfigo/inmunología , Pénfigo/diagnóstico , Pénfigo/patología , Pénfigo/complicaciones , Linfoma Folicular/complicaciones , Linfoma Folicular/diagnóstico , Linfoma Folicular/inmunología , Linfoma Folicular/patología , Linfoma Folicular/tratamiento farmacológico , Síndromes Paraneoplásicos/inmunología , Síndromes Paraneoplásicos/diagnóstico , Síndromes Paraneoplásicos/etiología , Síndromes Paraneoplásicos/patología , Desmogleína 3/inmunología , Autoanticuerpos/sangre , Autoanticuerpos/inmunología , Masculino , Piel/patología , Piel/inmunología , Femenino , Persona de Mediana Edad , AncianoRESUMEN
Paraneoplastic pemphigus (PNP) is an autoimmune bullous disease associated with underlying neoplasms and characterized by antibodies against desmoglein 3 (Dsg 3) and plakins. Autoantibodies against desmoglein 3 in sera of patients with PNP have been proven to cause acantholysis in vivo in neonatal mice. As a member of the plakin family, autoantibodies against desmoplakin were detected frequently by immunoprecipitation in the sera of PNP. The recombinant C-terminus of desmoplakin was expressed and purified to adsorb the specific autoantibodies against the C-terminus of desmoplakin. In vitro dispase-dependent keratinocyte dissociation assay and in vivo IgG passive transfer into neonatal mice assay were performed, followed by the electronic microscopy examination and TUNEL assay. We found that anti-C terminus of desmoplakin autoantibodies caused blisters and acantholysis in mice skin at a dose-dependent manner. Moreover, dissociated fragments were observed after incubation with the purified IgG against desmoplakin, compared with normal human IgG (P-value =0.0207). The electronic microscopy examination showed the disconnection of keratin intermediate filaments from desmosomes. Lastly, apoptosis of keratinocytes in the TUNEL assay was all detected in the skins of neonatal mice after injection of the anti-C terminus of desmoplakin autoantibodies. Taken together, the study suggests that autoantibodies against the C-terminus of desmoplakin might be pathogenic in PNP.
Asunto(s)
Acantólisis , Autoanticuerpos , Desmoplaquinas , Acantólisis/etiología , Acantólisis/inmunología , Animales , Enfermedades Autoinmunes/complicaciones , Desmogleína 3 , Desmoplaquinas/inmunología , Humanos , Inmunoglobulina G , Ratones , Síndromes Paraneoplásicos/inmunología , Pénfigo/inmunologíaRESUMEN
Autoantigen discovery is a critical challenge for the understanding and diagnosis of autoimmune diseases. While autoantibody markers in current clinical use have been identified through studies focused on individual disorders, we postulated that a reverse approach starting with a putative autoantigen to explore multiple disorders might hold promise. We here targeted the epidermal protein transglutaminase 1 (TGM1) as a member of a protein family prone to autoimmune attack. By screening sera from patients with various acquired skin disorders, we identified seropositive subjects with the blistering mucocutaneous disease paraneoplastic pemphigus. Validation in further subjects confirmed TGM1 autoantibodies as a 55% sensitive and 100% specific marker for paraneoplastic pemphigus. This gene-centric approach leverages the wealth of data available for human genes and may prove generally applicable for biomarker discovery in autoimmune diseases.
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Autoantígenos/sangre , Síndromes Paraneoplásicos/inmunología , Pénfigo/inmunología , Transglutaminasas/inmunología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores/sangre , Estudios de Casos y Controles , Niño , Femenino , Humanos , Masculino , Persona de Mediana Edad , Síndromes Paraneoplásicos/sangre , Pénfigo/sangre , Adulto JovenRESUMEN
ABSTRACT: Paraneoplastic granulomatous disease occurs in approximately 7.3% of patients with non-Hodgkin lymphoma, most commonly among patients with chronic lymphocytic leukemia (CLL). These lesions are often reported to appear similar to sarcoidosis in clinical presentation and under light microscopy. However, comprehensive descriptions of the cytomorphologic characteristics of these paraneoplastic granulomas are lacking, and the mechanisms involved in their formation remain ill-defined. Noninfectious dermal granulomatous reactions have also been reported in many primary immunodeficiencies, including common variable immune deficiency and ataxia-telangiectasia. We present a case of noninfectious CD8+ predominant granulomatous dermatitis with ocular involvement occurring in the setting of CLL and marked hypogammaglobulinemia. Based on the analysis of shared factors in patients with primary immunodeficiencies and CLL, we conclude that the presence of pan-humoral immunodeficiency could itself be a risk factor for developing a CD8+ lymphogranulomatous reaction. This report and associated discussion evince that CD8+ predominant granulomatous reactions, distinct from sarcoidosis, may represent a previously unappreciated segment of the paraneoplastic granulomas observed in hematologic malignancies.
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Linfocitos T CD8-positivos/patología , Dermatitis/inmunología , Granuloma/inmunología , Huésped Inmunocomprometido , Leucemia Linfocítica Crónica de Células B/complicaciones , Síndromes Paraneoplásicos/inmunología , Anciano , Dermatitis/patología , Granuloma/patología , Humanos , Masculino , Síndromes Paraneoplásicos/patologíaRESUMEN
In up to 34% of cases, thymoma, itself a rare neoplasm, is accompanied by autoimmune disorders, two of which are thymoma-associated multiorgan autoimmunity (TAMA) and paraneoplastic autoimmune multiorgan syndrome (PAMS). Unfortunately, differential diagnosis between these two entities can be challenging since no strict PAMS definition exists and PAMS can overlap with a subgroup of TAMA patients with skin lesions as leading presentation. We present a case of a 68-year-old woman with a diagnosis of thymoma accompanied by myasthenia gravis, hypothyroidism and GvHD-like mucocutaneous lesions that initially could account to both TAMA and PAMS diagnosis. However, following the exclusion of humoral autoimmunity against components of epithelial cells junction, TAMA was finally established. Interestingly, the introduction of corticosteroid therapy for TAMA symptom management resulted in unexpected partial remission of thymoma with no impact on mucocutaneous lesions. Our case study is an example of two extremely rare phenomena accompanying thymomas: unprecedented TAMA presentation with GvHD-like mucositis, which as we postulate should be placed in the spectrum of TAMA, and tumor remission on steroids.
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Autoinmunidad/inmunología , Timoma/inmunología , Neoplasias del Timo/inmunología , Corticoesteroides/uso terapéutico , Anciano , Femenino , Humanos , Miastenia Gravis/complicaciones , Síndromes Paraneoplásicos/diagnóstico , Síndromes Paraneoplásicos/tratamiento farmacológico , Síndromes Paraneoplásicos/inmunología , Inducción de Remisión , Timoma/complicaciones , Timoma/tratamiento farmacológico , Neoplasias del Timo/complicaciones , Neoplasias del Timo/tratamiento farmacológicoRESUMEN
BACKGROUND: Immune checkpoint inhibitors (ICIs) as a cancer immunotherapy have emerged as a treatment for multiple advanced cancer types. Because of enhanced immune responses, immune-related adverse events (irAEs), including endocrinopathies such as hypophysitis, have been associated with the use of ICIs. Most underlying mechanisms of ICI-related hypophysitis remain unclear, especially for programmed cell death-1 (PD-1)/PD-1 ligand 1 (PD-L1) inhibitors. We hypothesized that ICI-related hypophysitis is associated with paraneoplastic syndrome caused by ectopic expression of pituitary-specific antigens. METHODS: Twenty consecutive patients with ICI-related hypophysitis between 2017 and 2019 at Kobe University Hospital were retrospectively analyzed. Circulating anti-pituitary antibodies were detected using immunofluorescence staining and immunoblotting. Ectopic expression of pituitary autoantigens in tumor specimens was also examined. RESULTS: Eighteen patients were treated with PD-1/PD-L1 inhibitors, and two were treated with a combination of cytotoxic T-lymphocyte antigen-4 (CTLA-4) and PD-1 inhibitors. All patients showed adrenocorticotropic hormone (ACTH) deficiency and additionally, three showed thyroid-stimulating hormone (TSH) deficiency, and one showed gonadotropin-releasing hormone (GnRH) deficiency. Among these patients, three exhibited anti-pituitary antibodies, two with anti-corticotroph antibody and one with anti-somatotroph antibody. Interestingly, the anti-corticotroph antibody recognized proopiomelanocortin (POMC) and those two patients exhibited ectopic ACTH expression in the tumor, while the patients without anti-corticotroph antibody did not. CONCLUSIONS: We demonstrated 10% of PD-1/PD-L1 inhibitors-related hypophysitis were associated with the autoimmunity against corticotrophs and maybe caused as a form of paraneoplastic syndrome, in which ectopic expression of ACTH in the tumor was observed. It is also suggested that the pathophysiology is heterogenous in ICI-related hypophysitis.
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Hipofisitis/inmunología , Hipofisitis/terapia , Inhibidores de Puntos de Control Inmunológico/uso terapéutico , Síndromes Paraneoplásicos/inmunología , Síndromes Paraneoplásicos/terapia , Insuficiencia Suprarrenal/inmunología , Insuficiencia Suprarrenal/terapia , Adulto , Anciano , Anciano de 80 o más Años , Animales , Antígeno B7-H1/inmunología , Antígeno CTLA-4/inmunología , Corticotrofos/inmunología , Femenino , Humanos , Inmunoterapia/métodos , Masculino , Ratones , Persona de Mediana Edad , Neoplasias/inmunología , Neoplasias/terapia , Proopiomelanocortina/inmunología , Receptor de Muerte Celular Programada 1/inmunología , Estudios RetrospectivosRESUMEN
INTRODUCTION: Paraneoplastic pemphigus (PNP) is a life-threatening autoimmune blistering disease associated with underlying neoplasms. Currently, this disease is very difficult to treat. PATIENT CONCERNS: We reported a rare case of paraneoplastic pemphigus associated with small lymphocytic lymphoma responsive to desmoglein 3 (Dsg3) and bullous pemphigoid (BP) antigen 180. DIAGNOSES: The initial diagnosis was hypothesized to be Stevens-Johnson syndrome based on the severe mucosal erosion and polymorphous skin lesions. However, the histopathological examination of the skin biopsy and immunology revealed PNP. INTERVENTIONS: Anti-tumor therapy, immunosuppression and anti-infective therapy were administered. OUTCOMES: After a series of treatments, the skin lesions had been alleviated remarkably. Enzyme-linked immunoassays indices for Dsg3 and bullous pemphigoid antigen 180 decreased (Dsg3, 32; bullous pemphigoid antigen 180, 70.44). Unfortunately, 2 months later, the patient suffered respiratory failure due to the lung impairment of small lymphocytic lymphoma and infection. Eventually, the patient chose to be discharged from the hospital and lost the opportunity for follow-up treatment as he could not afford the expensive treatment costs. LESSONS: It is highly susceptible to misdiagnosis due to polymorphous skin lesions. In this case, it was also initially misdiagnosed as Stevens-Johnson syndrome. Therefore, we should pay great attention to differential diagnosis. When refractory stomatitis and mucosal erosions occur, the possibility of PNP should be considered first. At the same time, pathology, immunology and other related tests as well as the examination of primary tumors should be carried out as soon as possible.
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Leucemia Linfocítica Crónica de Células B/complicaciones , Síndromes Paraneoplásicos/complicaciones , Pénfigo/complicaciones , Anciano , Antibacterianos/uso terapéutico , Antineoplásicos/uso terapéutico , Desmogleína 3/biosíntesis , Diagnóstico Diferencial , Humanos , Inmunosupresores/uso terapéutico , Leucemia Linfocítica Crónica de Células B/tratamiento farmacológico , Masculino , Síndromes Paraneoplásicos/tratamiento farmacológico , Síndromes Paraneoplásicos/inmunología , Pénfigo/tratamiento farmacológico , Pénfigo/inmunologíaRESUMEN
Small cell lung cancer (SCLC) is a deadly and rapidly progressive disease that can present with various paraneoplastic syndromes on initial workup. Acquired factor VIII (FVIII) deficiency, also known as acquired haemophilia A (AHA), has been identified as a rare paraneoplastic syndrome in SCLC. Here, we present a 61-year-old woman with a massive gastrointestinal bleed and prolonged activated partial thromboplastin time (PTT) in the emergency department. She was diagnosed with rare paraneoplastic AHA secondary to extensive-stage SCLC (ES-SCLC). She was treated with high-dose steroids and factor bypassing agents, which led to the resolution of bleeding and undetectable FVIII inhibitor levels. She was subsequently treated for ES-SCLC with carboplatin, etoposide and atezolizumab. This case report highlights a rare clinical presentation of paraneoplastic AHA that necessitates prompt recognition in patients with SCLC with ongoing bleeding and elevated PTT.
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Neoplasias de las Glándulas Suprarrenales/diagnóstico , Hemorragia Gastrointestinal/diagnóstico , Hemofilia A/diagnóstico , Neoplasias Pulmonares/diagnóstico , Síndromes Paraneoplásicos/diagnóstico , Carcinoma Pulmonar de Células Pequeñas/diagnóstico , Abdomen , Neoplasias de las Glándulas Suprarrenales/complicaciones , Neoplasias de las Glándulas Suprarrenales/tratamiento farmacológico , Neoplasias de las Glándulas Suprarrenales/secundario , Anemia/diagnóstico , Anemia/etiología , Antineoplásicos/uso terapéutico , Autoanticuerpos/inmunología , Coagulantes/uso terapéutico , Transfusión de Eritrocitos , Factor VIII/inmunología , Factor VIII/uso terapéutico , Factor VIIa/uso terapéutico , Femenino , Hemorragia Gastrointestinal/etiología , Hemofilia A/etiología , Hemofilia A/inmunología , Hemofilia A/terapia , Humanos , Inmunosupresores/uso terapéutico , Neoplasias Pulmonares/complicaciones , Neoplasias Pulmonares/patología , Linfadenopatía , Mediastino , Persona de Mediana Edad , Síndromes Paraneoplásicos/etiología , Síndromes Paraneoplásicos/inmunología , Síndromes Paraneoplásicos/terapia , Carcinoma Pulmonar de Células Pequeñas/complicaciones , Carcinoma Pulmonar de Células Pequeñas/tratamiento farmacológico , Carcinoma Pulmonar de Células Pequeñas/secundarioRESUMEN
INTRODUCTION: Neurological symptoms associated with neuroendocrine tumours (NETs) may be related to metastatic disease or paraneoplastic syndromes (PNSs); these last are often associated with autoantibodies targeting various onconeural antigens. To better characterize neurological PNSs related to NETs, we report the largest case-series study to date. METHODS: We retrospectively reviewed the charts of all patients diagnosed with NETs of the gastrointestinal tract who presented with neurological symptoms at either of 2 tertiary academic hospitals (Henri Mondor and Beaujon, France) between 1994 and 2016. All patients underwent extensive neurological tests including clinical, laboratory, and radiological investigations. The clinical response to immunomodulating agents was recorded. RESULTS: In the 13 identified patients, the most common presentations were peripheral neuropathy (46.2%) and encephalopathy (26.6%). Of the 6 (53.3%) patients whose serum anti-neuronal antibodies were assayed, 5 had high titres. Short-term oral corticosteroid and immunosuppressant drug therapy was given to 4 of these patients, of whom 3 had a clinical response and 1 no response. Repeated high-dose intravenous immunoglobulin therapy induced a complete clinical response in 1 patient. Encephalopathy resolved fully after hepatectomy or intrahepatic chemoembolization for liver metastases in another 2 patients. DISCUSSION: The neurological symptoms associated with NETs may be due in part to autoimmune PNS. Based on experience at our 2 centres, we estimate that autoimmune PNS occurs in about 1% of patients with NETs. Early symptom recognition allows the initiation of effective treatments including corticosteroids, immunosuppressive drugs, and/or intravenous immunoglobulins.
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Enfermedades Autoinmunes del Sistema Nervioso/inmunología , Neoplasias Gastrointestinales/complicaciones , Neoplasias Gastrointestinales/inmunología , Tumores Neuroendocrinos/complicaciones , Tumores Neuroendocrinos/inmunología , Síndromes Paraneoplásicos/inmunología , Autoanticuerpos/sangre , Femenino , Francia , Neoplasias Gastrointestinales/diagnóstico , Humanos , Masculino , Persona de Mediana Edad , Tumores Neuroendocrinos/diagnóstico , Estudios RetrospectivosRESUMEN
INTRODUCTION: Little is known regarding characteristics of hospitalized dermatomyositis (DM) patients. Understanding the unique characteristics of hospitalized DM patients with underlying malignancy is important in guiding development of specific work-up and treatment algorithms. OBJECTIVES: We aim to characterize the inpatient burden of DM patients with malignancy (DM malignancy), determine unique characteristics of DM-malignancy inpatients, and assess trends and predictors of cost of care and length of stay for hospitalized DM-malignancy patients. METHODS: Hospitalized DM patients with and without malignancy were characterized and compared using 2009-2015 National Inpatient Sample. Associated malignancies, risk factors for malignancy, and trends/predictors for cost of care and length of stay were evaluated using multivariable models. RESULTS: Prevalence of malignancies among hospitalized DM inpatients was 10.9%. Age > 40 years and female sex were significantly associated with increased malignancy risk in DM inpatients. Numerous malignancies were significantly more common in men with DM compared to women, including bronchial, non-Hodgkin's lymphoma, head/neck, bladder, esophageal, kidney, and stomach. The most common malignancies in women with DM were breast and ovarian. Head/neck carcinomas were more common in hospitalized DM patients than previous cohorts evaluating outpatients. Socioeconomic characteristics differed between DM patients with/without malignancy. The presence of underlying malignancy did not affect hospitalization cost, length of stay, or mortality in the hospitalized DM population. The economic burden of hospitalized DM patients is increasing over time. CONCLUSIONS: DM inpatients with malignancy display numerous differences compared to DM inpatients without malignancy. Further research characterizing hospitalized DM patients is warranted in order to optimize work-up and treatment guidelines for these patients.
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Dermatomiositis/epidemiología , Síndromes Paraneoplásicos/epidemiología , Adolescente , Adulto , Factores de Edad , Anciano , Anciano de 80 o más Años , Dermatomiositis/inmunología , Dermatomiositis/terapia , Femenino , Hospitalización/estadística & datos numéricos , Humanos , Masculino , Persona de Mediana Edad , Síndromes Paraneoplásicos/inmunología , Síndromes Paraneoplásicos/terapia , Prevalencia , Estudios Retrospectivos , Factores de Riesgo , Factores Sexuales , Estados Unidos/epidemiología , Adulto JovenAsunto(s)
Eosinofilia/diagnóstico , Exantema/diagnóstico , Leucemia Mieloide Aguda/complicaciones , Síndromes Paraneoplásicos/diagnóstico , Prurito/diagnóstico , Administración Cutánea , Administración Oral , Biopsia , Eosinofilia/tratamiento farmacológico , Eosinofilia/inmunología , Eosinofilia/patología , Exantema/tratamiento farmacológico , Exantema/inmunología , Exantema/patología , Femenino , Glucocorticoides/administración & dosificación , Humanos , Leucemia Mieloide Aguda/inmunología , Persona de Mediana Edad , Síndromes Paraneoplásicos/tratamiento farmacológico , Síndromes Paraneoplásicos/inmunología , Síndromes Paraneoplásicos/patología , Prurito/tratamiento farmacológico , Prurito/inmunología , Prurito/patología , Piel/inmunología , Piel/patología , Resultado del TratamientoRESUMEN
Haematological malignancies induce important alterations of the immune system, which account for the high frequency of autoimmune complications observed in patients. Cutaneous immune-mediated diseases associated with haematological malignancies encompass a heterogeneous group of dermatoses, including, among others, neutrophilic and eosinophilic dermatoses, autoantibody-mediated skin diseases, vasculitis and granulomatous dermatoses. Some of these diseases, such as paraneoplastic pemphigus, are associated with an increased risk of death; others, such as eosinophilic dermatoses of haematological malignancies, run a benign clinical course but portend a significant negative impairment on a patient's quality of life. In rare cases, the skin eruption reflects immunological alterations associated with an unfavourable prognosis of the associated haematological disorder. Therapeutic management of immune-mediated skin diseases in patients with haematological malignancies is often challenging. Systemic corticosteroids and immunosuppressive drugs are considered frontline therapies but may considerably augment the risk of serious infections. Indeed, developing a specific targeted therapeutic approach is of crucial importance for this particularly fragile patient population. This review provides an up-to-date overview on the immune-mediated skin diseases most frequently encountered by patients with onco-haematological disorders, discussing new pathogenic advances and therapeutic options on the horizon.
Asunto(s)
Neoplasias Hematológicas/complicaciones , Inmunosupresores/administración & dosificación , Síndromes Paraneoplásicos/inmunología , Enfermedades de la Piel/inmunología , Neoplasias Hematológicas/inmunología , Neoplasias Hematológicas/mortalidad , Humanos , Inmunosupresores/efectos adversos , Síndromes Paraneoplásicos/diagnóstico , Síndromes Paraneoplásicos/tratamiento farmacológico , Pronóstico , Calidad de Vida , Índice de Severidad de la Enfermedad , Piel/inmunología , Enfermedades de la Piel/diagnóstico , Enfermedades de la Piel/tratamiento farmacológico , Resultado del TratamientoRESUMEN
The search for autoantibodies in patients with acute and chronic neuropathies has become widespread in neurological practice. These tests are more routinely available and therefore are more commonly requested in larger hospitals with neuroscience centres, although they are now also regularly requested from district general hospital settings, including by non-neurologists. However, the clinical value of these frequently expensive tests is often unclear and their impact on management not always obviously beneficial. This article reviews the main immunological tests used to search for specific autoantibodies in the setting of neuropathy and discusses their potential diagnostic importance, together with the eventual therapeutic implications of results obtained.
Asunto(s)
Autoanticuerpos/inmunología , Enfermedades del Sistema Nervioso Periférico/inmunología , Enfermedades del Sistema Nervioso Periférico/patología , Enfermedad Aguda , Enfermedad Crónica , Humanos , Síndromes Paraneoplásicos/inmunología , Síndromes Paraneoplásicos/patología , Paraproteinemias/inmunología , Paraproteinemias/patología , Síndrome de Sjögren/inmunología , Síndrome de Sjögren/patologíaRESUMEN
Oncorheumatology is the meeting point of tumour formation and rheumatic diseases. Multiple interactions exist between these two medical specialties. One major field is the topic of malignancies associated with rheumatic diseases, while the other topic covers the development of musculoskeletal disease in cancer patients. In the first group, secondary malignancies associated with rheumatic diseases, role of tumour-associated antigens in rheumatology, the possible carcinogenicity of conventional and targeted antirheumatic drugs and physical therapy of rheumatic patients with recent or current cancer will be discussed. The second large topic includes paraneoplastic syndromes, autoimmune-rheumatic side effects of oncotherapies (chemotherapy and immunotherapy), effects of hormone-deprivation therapies on bone and primary and secondary malignancies of the musculoskeletal system. Orv Hetil. 2020; 161(28): 1151-1165.