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Nederlands Tijdschrift Voor Geneeskunde Jun 2019A paraneoplastic syndrome is a phenomenon or complex of symptoms that can occur with malignancy, without this being the result of tumour cells in the affected area. In...
A paraneoplastic syndrome is a phenomenon or complex of symptoms that can occur with malignancy, without this being the result of tumour cells in the affected area. In this article, we describe the following paraneoplastic syndromes: thrombophlebitis migrans, clubbing, pemphigus, acanthosis nigricans, blue fingers, dermatomyositis, and myasthenia gravis.
Topics: Acanthosis Nigricans; Dermatomyositis; Humans; Neoplasm Recurrence, Local; Paraneoplastic Syndromes; Pemphigus; Thrombophlebitis
PubMed: 31283129
DOI: No ID Found -
Der Hautarzt; Zeitschrift Fur... Apr 2021Paraneoplastic skin manifestations associated with malignancies are extremely polymorphous. Clinicians should be familiar with paraneoplastic dermatoses to establish an... (Review)
Review
Paraneoplastic skin manifestations associated with malignancies are extremely polymorphous. Clinicians should be familiar with paraneoplastic dermatoses to establish an early diagnosis of the underlying neoplasm. Lack of familiarity with cutaneous clues for internal malignancies may delay diagnosis and treatment of cancer. In this review, we describe several paraneoplastic autoimmune dermatoses, including paraneoplastic autoimmune multiorgan syndrome, paraneoplastic bullous pemphigoid, and paraneoplastic dermatomyositis.
Topics: Autoantibodies; Autoimmune Diseases; Humans; Paraneoplastic Syndromes; Pemphigoid, Bullous; Skin Diseases
PubMed: 33646324
DOI: 10.1007/s00105-021-04773-w -
Journal of the European Academy of... Jun 2023Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ...
S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV).
BACKGROUND
Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans.
OBJECTIVES
These guidelines summarize evidence-based and expert-based recommendations (S2k level) for the clinical characterization, diagnosis and management of PNP/PAMS. They have been initiated by the Task Force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology with the contribution of physicians from all relevant disciplines. The degree of consent among all task force members was included.
RESULTS
Chronic severe mucositis and polymorphic skin lesions are clue clinical characteristics of PNP/PAMS. A complete assessment of the patient with suspected PNP/PAMS, requiring histopathological study and immunopathological investigations, including direct and indirect immunofluorescence, ELISA and, where available, immunoblotting/immunoprecipitation, is recommended to achieve a diagnosis of PNP/PAMS. Detection of anti-envoplakin antibodies and/or circulating antibodies binding to the rat bladder epithelium at indirect immunofluorescence is the most specific tool for the diagnosis of PNP/PAMS in a patient with compatible clinical and anamnestic features. Treatment of PNP/PAMS is highly challenging. Systemic steroids up to 1.5 mg/kg/day are recommended as first-line option. Rituximab is also recommended in patients with PNP/PAMS secondary to lymphoproliferative conditions but might also be considered in cases of PNP/PAMS associated with solid tumours. A multidisciplinary approach involving pneumologists, ophthalmologists and onco-haematologists is recommended for optimal management of the patients.
CONCLUSIONS
These are the first European guidelines for the diagnosis and management of PNP/PAMS. Diagnostic criteria and therapeutic recommendations will require further validation by prospective studies.
Topics: Animals; Rats; Autoimmune Diseases; Neoplasms; Paraneoplastic Syndromes; Paraneoplastic Syndromes, Nervous System; Societies, Medical
PubMed: 36965110
DOI: 10.1111/jdv.18931 -
Journal of Thoracic Oncology : Official... Nov 2019
Topics: Humans; Lung Neoplasms; Lymphocytes, Tumor-Infiltrating; Paraneoplastic Syndromes; Paraneoplastic Syndromes, Nervous System; Prognosis
PubMed: 31668313
DOI: 10.1016/j.jtho.2019.07.033 -
Journal of Neurology Oct 2022Paraneoplastic syndrome is a group of clinical symptoms that occur in the state of systemic malignant tumors. Paraneoplastic syndrome of the nervous system can affect... (Review)
Review
Paraneoplastic syndrome is a group of clinical symptoms that occur in the state of systemic malignant tumors. Paraneoplastic syndrome of the nervous system can affect any part of the central and peripheral nervous system and may also affect the eyes. In neuroophthalmology, paraneoplastic syndrome has a variety of manifestations that can affect both the afferent and efferent visual systems. The afferent system may involve the optic nerve, retina and uvea; the efferent system may involve eye movement, neuromuscular joints or involuntary eye movements and pupil abnormalities and may also have other neurological symptoms outside the visual system. This article discusses the clinical manifestations, pathological mechanisms, detection methods and treatment methods of paraneoplastic syndrome in neuroophthalmology. The performance of paraneoplastic syndrome is diverse, the diagnosis is difficult, and the treatment should be considered systematically. Differential diagnosis, optimal evaluation and management of these manifestations is not only the key to treatment but also a challenge.
Topics: Humans; Immunotherapy; Lambert-Eaton Myasthenic Syndrome; Neoplasms; Paraneoplastic Syndromes
PubMed: 35779086
DOI: 10.1007/s00415-022-11247-z -
Journal of Neuro-ophthalmology : the... Sep 2015Paraneoplastic syndromes that affect the visual pathways and present with neuro-ophthalmologic signs or symptoms may involve the afferent or efferent systems. Afferent... (Review)
Review
BACKGROUND
Paraneoplastic syndromes that affect the visual pathways and present with neuro-ophthalmologic signs or symptoms may involve the afferent or efferent systems. Afferent syndromes may involve the optic nerve or retina and, in some cases, these may be associated with systemic neurologic disease. Efferent symptoms typically affect eye movements and may involve the neuromuscular junction or involuntary eye movements.
EVIDENCE ACQUISITION
Literature review and personal clinical and research experience.
RESULTS
Diagnosis of paraneoplastic syndromes relies on clinical and laboratory evaluations. In the appropriate clinical setting, the presence of specific antibodies may help confirm the diagnosis.
CONCLUSIONS
In some cases, the visual pathway disturbance precedes a diagnosis of malignancy. Astute observation and selective evaluation and management are critical to establish the correct diagnosis and institute therapeutic approaches that can be sight or life saving.
Topics: Humans; Neurology; Ophthalmology; Paraneoplastic Syndromes
PubMed: 26172160
DOI: 10.1097/WNO.0000000000000280 -
Brain and Nerve = Shinkei Kenkyu No... Jun 2023Paraneoplastic neurologic syndromes (PNS) are a group of neurological disorders that are possibly caused by immunological mechanisms triggered by an underlying tumor...
Paraneoplastic neurologic syndromes (PNS) are a group of neurological disorders that are possibly caused by immunological mechanisms triggered by an underlying tumor that involves every part of the nervous system. Autoantibodies were categorized according to the risk of cancer association. Antibodies against intracellular proteins are excellent markers for tumor detection, however, without functional roles in neuronal loss, the direct effector of neuronal damage is thought to be cytotoxic T cells. The frequently associated symptoms include limbic encephalitis, cerebellar ataxia and sensory neuronopathy. The associated tumors are mainly small-cell lung cancer, breast/ovarian/uterine cancers, and thymoma. Timely diagnosis, prompt immunotherapy, and treatment of the underlying tumor are essential for managing PNS. However, we need to be cautious about the high frequency of false-positive/negative results of antibodies using commercial antibody tests. This highlight the importance of the careful evaluation of clinical features. Recently, PNS emerged after immune check point inhibitor administration, and this became a subject of attention exploring its pathogenesis. Other basic studies to understand the immunological background of PNS have been progressing.
Topics: Humans; Paraneoplastic Syndromes, Nervous System; Limbic Encephalitis; Autoantibodies; Small Cell Lung Carcinoma; Lung Neoplasms
PubMed: 37287359
DOI: 10.11477/mf.1416202411 -
Dermatologic Clinics Apr 2021The cutaneous paraneoplastic syndromes are rare and intrinsically devoid of any neoplastic nature. The manifestations on the skin and the nails are due to various... (Review)
Review
The cutaneous paraneoplastic syndromes are rare and intrinsically devoid of any neoplastic nature. The manifestations on the skin and the nails are due to various mechanisms caused by the tumor, either due to production of bioactive substances or in response to it. These disorders evolve in parallel to the malignancy, in that, they regress when the tumor is removed and reappear in the case of tumor recurrence. The aim of this article is to aid with the early recognition of the signs, leading to the early detection of cancer and therefore to better clinical outcomes for the patients.
Topics: Humans; Nails; Neoplasm Recurrence, Local; Paraneoplastic Syndromes; Skin; Skin Diseases
PubMed: 33745631
DOI: 10.1016/j.det.2020.12.003 -
Medicina Clinica Jan 2019
Topics: Adrenal Cortex Hormones; Combined Modality Therapy; Deglutition Disorders; Dermatomyositis; Diagnostic Errors; Facial Dermatoses; Female; Humans; Immunoglobulins, Intravenous; Lymphoma, Non-Hodgkin; Middle Aged; Muscle Weakness; Paraneoplastic Syndromes; Splenic Neoplasms; Urticaria
PubMed: 29759882
DOI: 10.1016/j.medcli.2018.03.024 -
Kidney International Nov 2022
Topics: Humans; Osteomalacia; Paraneoplastic Syndromes
PubMed: 36272746
DOI: 10.1016/j.kint.2022.04.042