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Current Opinion in Rheumatology Nov 2011Most epidemiologic studies have demonstrated an increased risk of cancer in scleroderma patients. Reasons for this risk increase have been poorly understood and often... (Review)
Review
PURPOSE OF REVIEW
Most epidemiologic studies have demonstrated an increased risk of cancer in scleroderma patients. Reasons for this risk increase have been poorly understood and often attributed to cytotoxic therapies or damage from scleroderma. Recognition that some patients have a close temporal relationship between cancer diagnosis and scleroderma clinical onset has focused attention on the possibility that scleroderma may be a paraneoplastic syndrome in a subset of patients. This review will discuss the latest epidemiologic data linking cancer and scleroderma and explore a model for the development of paraneoplastic scleroderma.
RECENT FINDINGS
New investigations have demonstrated an association between RNA polymerase III autoantibodies and a close temporal relationship between cancer diagnosis and the development of clinical scleroderma. A unique nucleolar RNA polymerase III expression pattern has been identified in malignant tissue from these scleroderma patients suggesting that autoantigen expression in the cancer and the autoantibody response are associated. Similar data in inflammatory myositis have illustrated that disease-specific autoantigens may be expressed in cancers and damaged target tissues (muscle) undergoing regeneration.
SUMMARY
These data suggest a model of paraneoplastic autoimmunity in which cross-reactive immune responses may target autoantigens that are expressed in both cancers and diseased autoimmune target tissues.
Topics: Autoantigens; Autoimmunity; Esotropia; Humans; Models, Immunological; Neoplasms; Paraneoplastic Syndromes; RNA Polymerase III; Risk Factors; Scleroderma, Systemic
PubMed: 21825998
DOI: 10.1097/BOR.0b013e32834a5081 -
Frontiers in Immunology 2018Systemic sclerosis (SSc) is an autoimmune connective tissue disorder, characterized by multisystem involvement, vasculopathy, and fibrosis. An increased risk of... (Review)
Review
Systemic sclerosis (SSc) is an autoimmune connective tissue disorder, characterized by multisystem involvement, vasculopathy, and fibrosis. An increased risk of malignancy is observed in SSc (including breast and lung cancers), and in a subgroup of patients with specific autoantibodies (i.e., anti-RNA polymerase III and related autoantibodies), SSc could be a paraneoplastic syndrome and might be directly related to an immune response against cancer. Herein, we reviewed the literature, focusing on the most recent articles, and shed light onto the potential relationship between cancer and scleroderma regarding temporal and immunological dimensions.
Topics: Autoantibodies; Autoimmune Diseases; Fibrosis; Humans; Neoplasms; Paraneoplastic Syndromes; RNA Polymerase III; Scleroderma, Systemic; Skin
PubMed: 30687318
DOI: 10.3389/fimmu.2018.03112 -
The Journal of Clinical Endocrinology... Mar 2014Non-islet cell tumor hypoglycemia (NICTH) is a rare but serious paraneoplastic syndrome in which a tumor secretes high molecular weight IGF-II, causing hypoglycemia.... (Review)
Review
CONTEXT
Non-islet cell tumor hypoglycemia (NICTH) is a rare but serious paraneoplastic syndrome in which a tumor secretes high molecular weight IGF-II, causing hypoglycemia. Complete tumor resection is curative but is often delayed or unfeasible. There is no clear "standard of care" for managing these patients.
EVIDENCE ACQUISITION
PubMed searches were conducted for: "non-islet-cell tumor hypoglycemia," "NICTH," "Doege-Potter," "Doege-Potter syndrome," "high molecular weight IGF-II," and "big IGF-II." Relevant articles were reviewed in detail. We limited our review to English-language articles, focusing on 1988-2013 (corresponding with the elucidation of the pathophysiology of NICTH).
EVIDENCE SYNTHESIS
The available literature exists as case reports or small case series, with a void of higher-order treatment studies. Thus, an evidence-based approach to data synthesis was difficult. Nevertheless, the available literature is presented objectively with an attempt to describe clinically useful trends and findings in the management of NICTH.
CONCLUSIONS
Appropriate identification of NICTH and prompt and complete tumor resection represents ideal management. However, when prompt resection is not feasible, iv glucose or dextrose often does not suffice to prevent hypoglycemia. In such cases, we suggest consideration of local antitumor therapies for disease control and trial of glucocorticoids alone or in combination with GH. Continuous glucagon infusion can be successful if the patient has a positive response to a glucagon stimulation test, and parenteral nutrition may allow higher glucose delivery, but both are limited by the need for continuous iv infusion. Diazoxide and octreotide have no role in NICTH.
Topics: Humans; Hypoglycemia; Islets of Langerhans; Pancreatic Neoplasms; Paraneoplastic Syndromes
PubMed: 24423303
DOI: 10.1210/jc.2013-3382 -
Anais Brasileiros de Dermatologia 2019Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms....
Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms. Diagnostic criteria include the presence of chronic mucositis and polymorphic cutaneous lesions with occult or confirmed neoplasia; histopathological analysis exhibiting intraepidermal acantholysis, necrotic keratinocytes, and vacuolar interface dermatitis; direct immunofluorescence with intercellular deposits (IgG and C3) and at the basement membrane zone (IgG); indirect immunofluorescence with intercellular deposition of IgG (substrates: monkey esophagus and simple, columnar, and transitional epithelium); and, autoreactivity to desmogleins 1 and 3, desmocollins 1, 2, and 3, desmoplakins I and II, envoplakin, periplakin, epiplakin, plectin, BP230, and α-2-macroglobulin-like protein 1. Neoplasias frequently related to paraneoplastic pemphigus include chronic lymphocytic leukemia, non-Hodgkin lymphoma, carcinomas, Castleman disease, thymoma, and others. Currently, there is no standardized treatment for paraneoplastic pemphigus. Systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclosporine, rituximab, cyclophosphamide, plasmapheresis, and intravenous immunoglobulin have been used, with variable outcomes. Reported survival rates in 1, 2, and 5 years are 49%, 41%, and 38%, respectively.
Topics: Autoantibodies; Erythema; Humans; Mouth Diseases; Paraneoplastic Syndromes; Pemphigus; Skin
PubMed: 31644609
DOI: 10.1590/abd1806-4841.20199165 -
Frontiers in Immunology 2022Recently, the paraneoplastic neurologic syndrome (PNS) diagnostic criteria have received a major update with a new score system over the past 16 years. We aimed to...
BACKGROUND
Recently, the paraneoplastic neurologic syndrome (PNS) diagnostic criteria have received a major update with a new score system over the past 16 years. We aimed to evaluate the diagnostic performance and clinical utility in China.
METHODS
An eligible cohort of 113 Chinese patients diagnosed with PNSs from the Second Affiliated Hospital School of Medicine Zhejiang University and published data were enrolled retrospectively. Data including clinical phenotype, antibody type, the presence of cancer, and duration of follow-up were reviewed and re-evaluated to classify the diagnostic levels for the 2004 and 2021 PNS criteria. The performances of these 2 criteria were compared. The critical parameters of antibody and cancer for the updated criteria were further explored.
RESULTS
The cohort consisted of 69 males and 44 females with a median age of 60 years. Limbic encephalitis (23, 20.4%), anti-Hu antibody (32, 28.3%), and small-cell lung cancer (32, 28.3%) were the most common clinical phenotype, detected antibody, and concomitant cancer, respectively. A total of 97 (85.8%) patients were diagnosed with definite PNS according to the 2004 criteria: only 42.3% (41/97) fulfilled the 2021 criteria, while the remaining 40, 14, and 2 re-diagnosed with probable PNS, possible PNS, and non-PNS. The requirement of cancers consistent with antibody and phenotype increased the specificity and thus greatly enhanced the accuracy of the 2021 criteria.
CONCLUSION
The updated criteria for PNS emphasized the consistency between cancer phenotype and antibody and showed a better diagnostic value. A better diagnostic yield could benefit disease management.
Topics: Adult; Aged; Aged, 80 and over; Antibodies, Neoplasm; China; Female; Humans; Limbic Encephalitis; Male; Middle Aged; Paraneoplastic Syndromes, Nervous System; Phenotype; Retrospective Studies
PubMed: 35173717
DOI: 10.3389/fimmu.2022.790400 -
The National Medical Journal of India 2018
Topics: Breast Neoplasms; Carcinoma, Ductal, Breast; Fasciitis; Female; Hand; Humans; Middle Aged; Palliative Care; Paraneoplastic Syndromes; Spinal Neoplasms
PubMed: 31134939
DOI: 10.4103/0970-258X.258234 -
BMC Psychiatry May 2017Patients with intracellular onconeural antibodies may present with neuro-psychiatric syndromes. We aimed to evaluate the evidence for an association between... (Review)
Review
BACKGROUND
Patients with intracellular onconeural antibodies may present with neuro-psychiatric syndromes. We aimed to evaluate the evidence for an association between well-characterized onconeural antibodies and psychiatric symptoms in patients with and without paraneoplastic central nervous system syndromes.
METHODS
Eligible studies were selected from 1980 until February 2017 according to standardized review criteria and evaluated using Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2). We included studies describing the psychiatric symptomatology of onconeural antibody positive patients and the prevalence of onconeural antibodies in patients with psychiatric disorders.
RESULTS
Twenty-seven studies met the inclusion criteria. Six studies reported on the prevalence of well-characterized onconeural antibodies in patients with different psychiatric disorders, ranging from 0% to 4.9%. Antibody prevalence in controls was available from three studies, ranging from 0% to 2.8%. Data heterogeneity precluded a meta-analysis. Two cerebrospinal fluid studies found well-characterized onconeural antibodies in 3.5% and 0% of patients with psychotic and depressive syndromes, respectively.
CONCLUSIONS
The available evidence suggests that the prevalence of well-characterized onconeural antibodies in patients with psychiatric disorders is generally low. However, the question whether onconeural antibodies are important in select patients with a purely psychiatric phenotype needs to be addressed by appropriately designed studies in the future.
Topics: Antibodies, Neoplasm; Humans; Mental Disorders; Paraneoplastic Syndromes, Nervous System
PubMed: 28468645
DOI: 10.1186/s12888-017-1325-z -
The Oncologist Mar 2006Paraneoplastic neurological syndromes (PNS) are remote effects of cancer that are not caused by invasion of the tumor or its metastases. Immunologic factors appear... (Review)
Review
Paraneoplastic neurological syndromes (PNS) are remote effects of cancer that are not caused by invasion of the tumor or its metastases. Immunologic factors appear important in the pathogenesis of PNS because antineuronal autoantibodies and T-cell responses against nervous system antigens have been defined for many of these disorders. The immunologic response is elicited by the ectopic expression of neuronal antigens by the tumor. Expression of these so-called "onconeural" antigens is limited to the tumor and the nervous system and sometimes also the testis. At the time of presentation of the neurological symptoms, most patients have not yet been diagnosed with cancer. Detection of paraneoplastic antibodies is extremely helpful in diagnosing an otherwise unexplained and often rapidly progressive neurological syndrome as paraneoplastic. In addition, the paraneoplastic antibodies may also direct the search for an underlying neoplasm. On the other hand, in patients known to have cancer, the presentation of a PNS may herald recurrence of the tumor or a second tumor. The number of paraneoplastic antibodies is still growing, and at least seven of these can now be considered well characterized. Based on the clinical syndrome, the type of antibody, and the presence or absence of cancer, patients are classified as having a "definite" or "possible" PNS. Despite the presumed autoimmune etiology of PNS, the results of various forms of immunotherapy have been disappointing, with some exceptions. Rapid detection and immediate treatment of the underlying tumor appears to offer the best chance of stabilizing the patient and preventing further neurological deterioration.
Topics: Antibodies; Dermatomyositis; Encephalomyelitis; Humans; Incidence; Paraneoplastic Syndromes, Nervous System; Prognosis; Sensation Disorders
PubMed: 16549814
DOI: 10.1634/theoncologist.11-3-292 -
Acta Medica Portuguesa Feb 1995Some neurologic syndromes are often associated with neoplasm, as a paraneoplastic disorder. Among them, namely, subacute cerebellar degeneration, sensory neuronopathy,... (Review)
Review
Some neurologic syndromes are often associated with neoplasm, as a paraneoplastic disorder. Among them, namely, subacute cerebellar degeneration, sensory neuronopathy, dermatomyositis/polymyositis, Lambert-Eaton myasthenic syndrome and subacute motor neuronopathy, prove sooner or later to be harbouring a neoplasm, therefore a thorough clinical evaluation should be carried out to identify the primary tumour site. The AA present a brief review of these neurologic syndromes, illustrating the first three mentioned with clinical cases. A reference is made to the immunological associated aspects and their clinical importance.
Topics: Aged; Female; Humans; Male; Middle Aged; Nervous System Diseases; Paraneoplastic Syndromes
PubMed: 7771203
DOI: No ID Found -
Bulletin of the NYU Hospital For Joint... 2012Review of the literature addressing the rheumatic manifestations of various malignancies as well as of common chemotherapeutic agents. (Review)
Review
OBJECTIVE
Review of the literature addressing the rheumatic manifestations of various malignancies as well as of common chemotherapeutic agents.
METHODS
A literature search was performed to identify key articles regarding the association of rheumatic disease with malignancy.
RESULTS
Our review focused on the association of rheumatic disease with malignancy, paraneoplastic syndromes with rheumatic manifestations, and chemotherapeutic agents related to rheumatic syndromes. We have discussed the importance of a newly described autoantibody that may identify patients at risk for malignancy associated myositis.
CONCLUSION
Based on our literature review, recommendations are suggested regarding who and how patients should be screened for malignancy when presenting with various rheumatic symptoms.
Topics: Antineoplastic Agents; Autoantibodies; Biomarkers; Humans; Neoplasms; Paraneoplastic Syndromes; Rheumatic Diseases; Risk Assessment; Risk Factors
PubMed: 22892000
DOI: No ID Found