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    Paraneoplastic pemphigus associated with Castleman disease: Progression from mucous to mucocutaneous lesions with epitope-spreading phenomena.
    Br J Dermatol 2017 Feb 18. Epub 2017 Feb 18.
    Departments of Dermatology, Kindai University Faculty of Medicine, Osaka-Sayama, Japan.
    Paraneoplastic pemphigus (PNP) is a frequently fatal autoimmune blistering disease of the skin and mucous membranes.(1) PNP is commonly associated with malignant neoplasms or haematological disorders like Castleman disease (CD). The eruptions may resemble those seen in various other conditions such as lichen planus (LP), graft-versus-host disease, erythema multiforme (EM), bullous pemphigoid and pemphigus vulgaris (PV). Read More

    Paraneoplastic Dermatomyositis with Cutaneous and Myopathic Disease Responsive to Adrenocorticotropic Hormone Therapy.
    J Clin Aesthet Dermatol 2017 Jan 1;10(1):57-62. Epub 2017 Jan 1.
    St. Barnabas Hospital Dermatology Residency, Bronx, New York;; Mount Sinai School of Medicine Manhattan, New York.
    Dermatomyositis is a myopathic or amyopathic autoimmune connective tissue disease that presents with classic dermatologic findings ranging from: poikilodermatous photosensitivity (shawl sign), eyelid edema and violaceous-pigmentation (heliotrope sign), lichenoid eruptions on the knuckles and elbows (Gottron's sign), periungual telangiectasias, and ragged cuticles (Samitz sign). Up to 30 percent of adult-onset cases of dermatomyositis may represent a paraneoplastic syndrome warranting a thorough work-up for malignancy. The authors present a case report of paraneoplastic dermatomyositis associated with triple negative, BRCA-1 positive, invasive intraductal carcinoma of the breast, whose myopathic and cuteanous symptoms were recalcitrant to high-dose corticosteroid therapy. Read More

    The crossroads between cancer immunity and autoimmunity: antibodies to self antigens.
    Front Biosci (Landmark Ed) 2017 Mar 1;22:1289-1329. Epub 2017 Mar 1.
    Dept. of Clinical Sciences and Translational Medicine, Faculty of Medicine, University of Rome "Tor Vergata", Via Montpellier 1, 00133, Rome, Italy,
    The production of autoantibodies to self antigens is dependent on the failure of immune tolerance. Cancer cells express antigens which elicit a spontaneous immune response in cancer patients. The repertoire of autoantibodies found in cancer patients partly covers that of patients with autoimmune diseases. Read More

    Transient IKK2 activation in astrocytes initiates selective non-cell-autonomous neurodegeneration.
    Mol Neurodegener 2017 Feb 13;12(1):16. Epub 2017 Feb 13.
    Institute of Physiological Chemistry, Ulm University, Albert-Einstein-Allee 11, 89081, Ulm, Germany.
    Background: Neuroinflammation is associated with a wide range of neurodegenerative disorders, however the specific contribution to individual disease pathogenesis and selective neuronal cell death is not well understood. Inflammatory cerebellar ataxias are neurodegenerative diseases occurring in various autoimmune/inflammatory conditions, e.g. Read More

    Management of neuro-oncologic emergencies.
    Handb Clin Neurol 2017 ;141:715-741
    Neuro-Oncology Center, University of Virginia, Charlottesville, VA, USA. Electronic address:
    Patients with brain tumors and systemic malignancies are subject to diverse neurologic complications that require urgent evaluation and treatment. These neurologic conditions are commonly due to the tumor's direct effects on the nervous system, such as cerebral edema, increased intracranial pressure, seizures, spinal cord compression, and leptomeningeal metastases. In addition, neurologic complications can develop as a result of thrombocytopenia, coagulopathy, hyperviscosity syndromes, infection, immune-related disorders, and adverse effects of treatment. Read More

    [CXCL-13 as a biomarker in the diagnostics of neuroborreliosis].
    Nervenarzt 2017 Feb 9. Epub 2017 Feb 9.
    Abteilung für Neurologie, Universitätsklinikum St. Pölten, KLPU, Propst Führer-Straße 4, 3100, St. Pölten, Österreich.
    Background: The chemokine CXCL-13 is a potential intrathecal biomarker for neuroborreliosis (NB). According to the literature the sensitivity of CXCL-13 in the diagnostics of NB varies between 88% and 100% and the specificity between 63% and 99.7%. Read More

    Association between rheumatic diseases and cancer: results from a clinical practice cohort study.
    Intern Emerg Med 2017 Feb 8. Epub 2017 Feb 8.
    Division of Internal Medicine, Immunorheumatology Unit, "Maggiore della Carità" Hospital, Corso Mazzini 18, 28100, Novara, Italy.
    The association between cancer and immune-mediated rheumatic conditions is controversial, especially as far as polymyalgia rheumatica (PMR) is concerned. Furthermore, no clinical feature has been shown to be suggestive of a paraneoplastic rheumatic syndrome. With the present study, we aim to address both these issues. Read More

    Skin: A mirror of internal malignancy.
    Indian J Med Paediatr Oncol 2016 Oct-Dec;37(4):214-222
    Department of Dermatology, Shree Krishna Hospital, Pramukh Swami Medical College, Karamsad, Gujarat, India.
    Skin manifestations are a reflection of many of the internal diseases. Sometimes, skin disease may be the only manifestation of the internal disease. Internal malignancies may give rise to a number of cutaneous manifestations through their immunological, metabolic, and metastatic consequences. Read More

    Polyneuropathy as Novel Initial Manifestation in a Case of "Nonsecretory" POEMS Syndrome with Sjögren's Syndrome.
    Case Rep Med 2017 4;2017:1276759. Epub 2017 Jan 4.
    Nursing Department, Huashan Hospital, Fudan University, Shanghai 200040, China.
    POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a paraneoplastic syndrome driven by plasma cell dyscrasias. We report a patient with novel initial manifestation of polyneuropathy, which was considered due to Sjögren's syndrome but with poor response to methylprednisolone (120 mg/d) and intravenous immunoglobulin (IVIg). Further investigation by imaging tests and following biopsy eventually confirmed the diagnosis of POEMS syndrome secondary to solitary plasmocytoma. Read More

    Vanishing bile duct syndrome in Hodgkin's lymphoma: A case report and literature review.
    World J Gastroenterol 2017 Jan;23(2):366-372
    Mena Bakhit, Thomas R McCarty, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT 06520, United States.
    Vanishing bile duct syndrome (VBDS) has been described in different pathologic conditions including infection, ischemia, adverse drug reactions, autoimmune diseases, allograft rejection, and humoral factors associated with malignancy. It is an acquired condition characterized by progressive destruction and loss of the intra-hepatic bile ducts leading to cholestasis. Prognosis is variable and partially dependent upon the etiology of bile duct injury. Read More

    Successful treatment of tumour-induced osteomalacia after resection of an oral peripheral ossifying fibroma.
    BMJ Case Rep 2017 Jan 24;2017. Epub 2017 Jan 24.
    Department of Radiology, College of Medicine and Philippine General Hospital, University of the Philippines Manila, Manila, Philippines.
    Tumour-induced osteomalacia is a paraneoplastic syndrome wherein bone is affected by a hormone from a tumour that causes renal phosphate wasting and hypophosphataemia. Here, we present the case of a 31-year-old man who has been suffering from generalised bone pains and a spine deformity that led to loss of height. Pertinent findings are low serum phosphorus, low vitamin D and decreased bone mineral density. Read More

    Impact of chemotherapy on eosinophilia-associated advanced rectal cancer: A case report and review of the literature.
    Oncol Lett 2016 Dec 8;12(6):5269-5274. Epub 2016 Nov 8.
    Department of Cardiovascular and Gastroenterological Surgery, Kagoshima University Medical and Dental Hospital, Kagoshima 890-8544, Japan.
    The present study reports a case of eosinophilia-associated rectal cancer that was successfully stabilized using chemotherapy, and reviews the mechanisms of eosinophilia and the importance of chemotherapy. A 65-year-old man, who had previously been diagnosed with suspected rectal cancer, presented with the chief complaint of melena. Eosinophilia, abnormal blood coagulation, and elevated carcinoembryonic antigen and carbohydrate antigen 19-9 tumor marker levels were observed, and the patient was subsequently diagnosed with advanced rectal cancer accompanied by multiple lymph node metastases that extended from the para-aortic lymph nodes to the left axillary lymph nodes. Read More

    When dizziness becomes sinister: oropharyngeal carcinoma presenting as a paraneoplastic neurological disorder.
    BMJ Case Rep 2017 Jan 19;2017. Epub 2017 Jan 19.
    Edinburgh Cancer Research Centre, Edinburgh Cancer Centre, Edinburgh, UK.
    Paraneoplastic neurological disorders are uncommon presentations of head and neck cancers. We present a case of a 68-year-old male patient who presented with dizziness, nausea and memory problems. MRI of his brain showed bilateral cerebellar leptomeningeal enhancing signal abnormality with cervical lymphadenopathy. Read More

    Anti-N-Methyl-D-Aspartate Receptor Encephalitis in HIV Infection.
    Case Rep Neurol 2016 Sep-Dec;8(3):251-257. Epub 2016 Dec 13.
    Unidade de Doenças Auto-imunes/Serviço Medicina 7.2, Hospital Curry Cabral, Centro Hospitalar Lisboa Central, Lisbon, Portugal.
    Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a rare condition characterized by emotional and behavioral disturbances, dyskinesias, and extrapyramidal signs. It occurs in young women of reproductive age and is classically described as a paraneoplastic phenomenon. We present a 36-year-old, HIV-positive female who was admitted to the hospital in an acute confusional state, with a stiff posture, periods of motor agitation, and myoclonic jerks of the hands. Read More

    Bazex Syndrome in Lung Squamous Cell Carcinoma: High Expression of Epidermal Growth Factor Receptor in Lesional Keratinocytes with Th2 Immune Shift.
    Case Rep Dermatol 2016 Sep-Dec;8(3):358-362. Epub 2016 Dec 8.
    Department of Dermatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.
    An 82-year-old Japanese man was referred for detailed examination of hyperkeratotic erythematous plaques on his palms and soles for 6 months. Two weeks before his first visit, he had undergone lung lobectomy for right lung squamous cell carcinoma (SCC). Laboratory findings showed elevations of eosinophil counts, serum IgE, thymus and activation-regulated chemokine, SCC antigen, and soluble interleukin-2 receptor levels. Read More

    Immune-Mediated Neuropathies following Autologous Stem Cell Transplantation for Multiple Myeloma: Case Series and Review of the Literature.
    Acta Haematol 2017 Jan 17;137(2):86-88. Epub 2017 Jan 17.
    University of Kansas Medical Center, Kansas City, KS, USA.
    Neuropathy is a common finding in patients with multiple myeloma. Several different factors can cause neuropathy in these patients, such as the underlying disease itself, medications used for treatment, or immune-mediated processes. Immune-mediated neuropathies (IMN) consist of a heterogeneous spectrum of peripheral nerve disorders. Read More

    Treatment of patients with Waldenström macroglobulinaemia: clinical practice guidelines from the Myeloma Foundation of Australia Medical and Scientific Advisory Group.
    Intern Med J 2017 Jan;47(1):35-49
    Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.
    Waldenström macroglobulinaemia (WM) is an indolent B-cell malignancy characterised by the presence of immunoglobulin M (IgM) paraprotein and bone marrow infiltration by clonal small B lymphocytes, plasmacytoid lymphocytes and plasma cells. The symptoms of WM are protean, often follow an asymptomatic phase and may include complications related to the paraneoplastic effects of IgM paraprotein. The revised 2016 World Health Organization classification includes the MYD88 L265P mutation, which is seen in >90% of cases, within the diagnostic criteria for WM. Read More

    Microtubule Associated Protein (MAP) 1B: Novel Paraneoplastic Biomarker Running head: MAP 1B-IgG: novel paraneoplastic biomarker.
    Ann Neurol 2017 Jan 11. Epub 2017 Jan 11.
    Departments of Laboratory Medicine, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, USA, 55905.
    Objective: To report the identification of microtubule associated protein (MAP)1B as the antigen of the previously described PCA-2 antibody, its frequency and clinical, oncological and serological associations. Methods Archival serum or cerebrospinal fluid (CSF) specimens were available from 96 of 118 consecutive PCA-2-IgG-seropositive patients identified 1993-2016. The autoantigen, defined in mouse brain lysate by western blot and mass spectrometry, was confirmed by dual immunohistochemical staining using commercial antibodies. Read More

    Intrathoracic tumor of the chest wall: A case of Castleman's disease mimicking myositis of the lower extremities.
    Gen Thorac Cardiovasc Surg 2017 Jan 10. Epub 2017 Jan 10.
    Department of Thoracic Surgery, University Hospital of Basel, Spitalstraße 2, 4056, Basel, Switzerland.
    Castleman's disease refers to a group of uncommon lymphoproliferative disorders which exhibit common lymph-node histological features. A 72-year-old male patient presented with signs of lower limb myositis. Detailed work-up focused initially on evaluating hematological malignancies, the presence of a solid tumor, autoimmune diseases and degenerative disorders of the peripheral nerves. Read More

    Cerebellar ataxia and sensory ganglionopathy associated with light-chain myeloma.
    Cerebellum Ataxias 2017 5;4. Epub 2017 Jan 5.
    Academic Department of Neurosciences, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK ; University of Sheffield, Royal Hallamshire Hospital, Royal Hallamshire Hospital, Glossop Rd, Sheffield, South Yorkshire S10 2JF UK.
    Background: Cerebellar ataxia with sensory ganglionopathy is a rare neurological combination that can occur in some hereditary ataxias including mitochondrial diseases and in gluten sensitivity. Individually each condition can be a classic paraneoplastic neurological syndrome. We report a patient with this combination who was diagnosed with light-chain myeloma ten years after initial presentation. Read More

    Nephrotic syndrome associated with metastatic thymoma treated with chemotherapy.
    Medicine (Baltimore) 2017 Jan;96(1):e5408
    aDepartment of Internal Medicine, Seoul National University Hospital, Seoul bDivision of Hemato-medical Oncology, Department of Internal Medicine, Seoul National University Bundang Hospital, Gyeong-gi cDivision of Hematology-Oncology, Department of Internal Medicine, Gyeongsang Institute of Health Science, Gyeongsang National University Hospital, Gyeongsang National University School of Medicine, Jinju dDepartment of Pathology, Gyeonsang National University College of Medicine, Jinju, Korea.
    Rationale: Nephropathy with concurrent invasive thymoma is a type of paraneoplastic syndrome.

    Patient Concerns And Diagnoses: We report a 32-year-old female with nephrotic syndrome that was first diagnosed along with invasive thymoma and treated by means of cisplatin-based chemotherapy for the thymoma. The patient initially presented with dyspnea and generalized edema. Read More

    Prognostic value of computed tomography pulmonary angiography indices in patients with cancer-related pulmonary embolism: Data from a multicenter cohort study.
    Eur J Radiol 2017 Feb 13;87:66-75. Epub 2016 Dec 13.
    Hematology and Medical Oncology Department, Hospital Universitario Morales Meseguer, Av Marqués de los Vélez, s/n, 30008, Murcia, Spain.
    Objective: To analyze the prognostic value of pulmonary artery obstruction versus right-ventricle (RV) dysfunction radiologic indices in cancer-related pulmonary embolism (PE).

    Methods: We enrolled 303 consecutive patients with paraneoplastic PE, evaluated by computed tomography pulmonary angiography (CTPA) between 2013 and 2014. The primary outcome measure was serious complications at 15days. Read More

    GAD65 neurological autoimmunity.
    Muscle Nerve 2017 Jan 7. Epub 2017 Jan 7.
    Departments of Neurology, College of Medicine, Mayo Clinic, 200 1st St SW, Rochester, Minnesota, 55905.
    The glutamic acid decarboxylase 65-isoform (GAD65) antibody is a biomarker of autoimmune central nervous system (CNS) disorders and, more commonly, non-neurological autoimmune diseases. Type 1 diabetes, autoimmune thyroid disease, and pernicious anemia are the most frequent GAD65 autoimmune associations. One or more of these disorders coexists in approximately 70% of patients with GAD65 neurological autoimmunity. Read More

    Unilateral Recurrent Anterior Uveitis as the Presenting Sign of Bladder Carcinoma.
    Turk J Ophthalmol 2016 Aug 15;46(4):190-193. Epub 2016 Aug 15.
    Necmettin Erbakan University Meram Faculty of Medicine, Department of Ophthalmology, Konya, Turkey.
    A 79-year-old male patient was followed for unilateral uveitis with 3 attacks in 10 months, despite initial improvement with steroid therapy. The patient had visual acuity (VA) of counting fingers in right eye, hypopyon and vitritis with no chorioretinal lesions. The left eye was normal. Read More

    Humoral Hypercalcemia in Uterine Cancers: A Case Report and Literature Review.
    Am J Case Rep 2017 Jan 6;18:22-25. Epub 2017 Jan 6.
    Department of Medicine, Division of Hematology/Oncology, Staten Island University Hospital, Staten Island, NY, USA.
    BACKGROUND Paraneoplastic hypercalcemia is a well-described complication associated with a variety of malignancies. However, its incidence in gynecological malignancies is low. CASE REPORT A 53-year-old woman presented with progressive abdominal distention and irregular vaginal bleeding of several weeks' duration. Read More

    Paraneoplastic Evans syndrome in a patient with adenocarcinoma of the lung: A case report.
    Thorac Cancer 2017 Jan 16;8(1):57-60. Epub 2016 Nov 16.
    Department of Hematology, Tianjin Medical University General Hospital, Tianjin, China.
    We present a rare case of newly diagnosed Evans syndrome associated with lung papillary adenocarcinoma in which the patient showed prompt restoration of blood cell count and long-lasting complete remission of Evans syndrome after lung cancer resection. Detailed investigation led to a diagnosis of Evans syndrome. In the first year of the disease, left lower lung papillary adenocarcinoma was diagnosed. Read More

    A young child of anti-NMDA receptor encephalitis presenting with epilepsia partialis continua: the first pediatric case in Korea.
    Korean J Pediatr 2016 Nov 30;59(Suppl 1):S133-S138. Epub 2016 Nov 30.
    Department of Pediatrics, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine, Changwon, Korea.
    Anti-N-methyl D-aspartate receptor (anti-NMDAR) encephalitis, recently recognized as a form of paraneoplastic encephalitis, is characterized by a prodromal phase of unspecific illness with fever that resembles a viral disease. The prodromal phase is followed by seizures, disturbed consciousness, psychiatric features, prominent abnormal movements, and autonomic imbalance. Here, we report a case of anti-NMDAR encephalitis with initial symptoms of epilepsia partialis continua in the absence of tumor. Read More

    Syndrome of inappropriate antidiuretic hormone secretion in a case of olfactory neuroblastoma without anti-diuretic hormone immunoreactivity: A case report and review of the literature.
    Auris Nasus Larynx 2016 Dec 20. Epub 2016 Dec 20.
    Department of Otorhinolaryngology, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-3-1, Higashi-ku, Fukuoka 812-8582, Japan.
    Olfactory neuroblastoma (ONB) is a relatively rare nasal or paranasal malignant tumor. This tumor is rarely accompanied by paraneoplastic syndromes such as syndrome of inappropriate antidiuretic hormone secretion (SIADH). Here, we report a 31-year-old female with histologically confirmed ONB who had been diagnosed with SIADH three years prior. Read More

    UNILATERAL DIFFUSE UVEAL MELANOCYTIC PROLIFERATION.
    Retin Cases Brief Rep 2016 Dec 21. Epub 2016 Dec 21.
    Vitreous, Retina, Macula Consultants of New York, New York, New York.
    Purpose: To report a unilateral case of what is named bilateral diffuse uveal melanocytic proliferation and consider the consequences of this finding.

    Methods: The ocular findings were investigated with multimodal imaging to include color fundus photography, fluorescein angiography, autofluorescence imaging, and enhanced depth imaging optical coherence tomography.

    Results: A 66-year-old woman had a history of breast cancer 23 years previously that was treated and the patient was free of disease since. Read More

    Distinction between mesothelioma and lung adenocarcinoma based on immunohistochemistry in a patient with asbestos bodies in bronchoalveolar fluid - case report.
    Rom J Morphol Embryol 2016 ;57(3):1171-1174
    Department of Pneumology, University of Medicine and Pharmacy of Craiova, "Victor Babes" Clinical Hospital of Infectious Diseases and Pneumophthisiology, Craiova, Romania;
    Asbestos is a mineral-mined form the rocks, consisting in amosite (brown asbestos), crocidolite (blue asbestos) and÷or chrysotile (white asbestos) used in many industries. Researches about the exposure to asbestos dust and asbestosis related diseases started almost a century ago. The first case report of fatal asbestosis disease was published in 1906, in England, by Dr. Read More

    Seborrheic Keratoses as the First Sign of Bladder Carcinoma: Case Report of Leser-Trélat Sign in a Rare Association with Urinary Tract Cancer.
    Case Rep Med 2016 23;2016:4259190. Epub 2016 Nov 23.
    Internal Medicine Department, Clinics Hospital of the Federal University of Paraná, Curitiba, PR, Brazil.
    Introduction. Skin disorders can be the first manifestation of occult diseases. The recognition of typical paraneoplastic dermatoses may anticipate the cancer diagnosis and improve its prognosis. Read More

    Neutrophil-lymphocyte ratio and platelet-lymphocyte ratio are 2 new inflammatory markers associated with pulmonary involvement and disease activity in patients with dermatomyositis.
    Clin Chim Acta 2017 Feb 10;465:11-16. Epub 2016 Dec 10.
    Department of Clinical Laboratory, The Second Affiliated Hospital of Nanchang University, Nanchang 330006, China. Electronic address:
    Background: The neutrophil-lymphocyte ratio (NLR) and platelet-lymphocyte ratio (PLR) have emerged as useful biomarkers to predict systemic inflammation. However, there is no study to investigate the relationship between the biomarkers and dermatomyositis (DM).

    Methods: Seventy-three newly diagnosed patients with DM and 147 healthy subjects were selected in this retrospective study. Read More

    Intravital FRET imaging reveals osteopontin-mediated polymorphonuclear leukocyte activation by tumor cell emboli.
    Cancer Sci 2016 Dec 13. Epub 2016 Dec 13.
    Department of Pathology and Biology of Diseases, Graduate School of Medicine, Kyoto University, Kyoto, 606-8501, Japan.
    Myeloid-derived suppressor cells (MDSCs) cause paraneoplastic leukemoid reactions and facilitate tumor cell metastasis. However, the interaction of MDSCs with tumor cells in live tissues has not been adequately visualized. To accomplish this task, we developed an intravital imaging protocol to observe metastasized tumor cells in mouse lungs. Read More

    [Autoimmunization and carcinogenesis].
    Wiad Lek 2016 ;69(4):637-641
    Katedra i Zakład Fizjologii Człowieka, Uniwersytet Medyczny, Lublin, Klinika Reumatologii i Układowych Chorób Tkanki Łącznej, Uniwersytet Medyczny, Lublin.
    The connections between autoimmunization and carcinogenesis have been observed in many patients' cases for numerous years. Various autoimmune diseases precede the diagnosis of neoplasm, however the mechanisms of autoimmunization may also be useful in anti-cancer defence. This review presents the analysis of links between the mentioned processes and their possible application in modern anti-neoplasmatic immunotherapy. Read More

    Acral manifestations of paraneoplastic and collagen vascular diseases.
    Clin Dermatol 2017 Jan - Feb;35(1):50-54. Epub 2016 Sep 10.
    Department of Dermatology, Akdeniz University, Faculty of Medicine, Antalya, Turkey. Electronic address:
    The skin often signals a number of systemic disease, making skin findings of paramount significance. Paraneoplastic diseases and collagen vascular diseases are vitally important illnesses. Paraneoplastic diseases and collagen vascular diseases may also occur with many different acral skin findings. Read More

    E2F1-mediated human POMC expression in ectopic Cushing's syndrome.
    Endocr Relat Cancer 2016 Nov 7;23(11):857-870. Epub 2016 Oct 7.
    Pituitary CenterCedars-Sinai Medical Center, Los Angeles, California, USA
    Cushing's syndrome is caused by excessive adrenocorticotropic hormone (ACTH) secretion derived from pituitary corticotroph tumors (Cushing disease) or from non-pituitary tumors (ectopic Cushing's syndrome). Hypercortisolemic features of ectopic Cushing's syndrome are severe, and no definitive treatment for paraneoplastic ACTH excess is available. We aimed to identify subcellular therapeutic targets by elucidating transcriptional regulation of the human ACTH precursor POMC (proopiomelanocortin) and ACTH production in non-pituitary tumor cells and in cell lines derived from patients with ectopic Cushing's syndrome. Read More

    Hypertrophic Osteoarthropathy: Clinical and Imaging Features.
    Radiographics 2017 Jan-Feb;37(1):157-195. Epub 2016 Dec 9.
    From the Department of Radiology, Keck School of Medicine, University of Southern California, 1500 San Pablo St, 2nd Floor Imaging, Los Angeles, CA 90033 (F.Y.Y., D.B.P., A.J.S., E.A.W., A.T., G.R.M.); Department of Radiology, Southern California University of Health Sciences, Whittier, Calif (M.R.S.); and Department of Radiology, Greater Los Angeles Veterans Affairs Medical Center, Los Angeles, Calif (S.M.).
    Hypertrophic osteoarthropathy (HOA) is a medical condition characterized by abnormal proliferation of skin and periosteal tissues involving the extremities and characterized by three clinical features: digital clubbing (also termed Hippocratic fingers), periostosis of tubular bones, and synovial effusions. HOA can be a primary entity, known as pachydermoperiostosis, or can be secondary to extraskeletal conditions, with different prognoses and management implications for each. There is a high association between secondary HOA and malignancy, especially non-small cell lung cancer. Read More

    Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) case presentation and comparison with other polyarthritides affecting older people.
    Age Ageing 2016 Dec 7. Epub 2016 Dec 7.
    Laboratorio Clinico - Hospital Universitario Central de Asturias, Spain.
    Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) syndrome mainly affects elderly men and responds well to steroids. Since this syndrome can resemble other diseases, its diagnosis is a significant challenge. Through the following paper, we hope to improve the diagnosis of RS3PE by presenting a table comparing RS3PE to two other common polyarthritic conditions affecting the elderly. Read More

    Specific autoantibodies in dermatomyositis: a helpful tool to classify different clinical subsets.
    Arch Dermatol Res 2017 Mar 7;309(2):87-95. Epub 2016 Dec 7.
    Di.S.Sal. Section of Dermatology, IRCCS Azienda Ospedaliera Universitaria San Martino-IST, Via Pastore 1, 16132, Genoa, Italy.
    Autoantibodies are important in the diagnosis of dermatomyositis. They can be divided in two different groups: myositis-associated autoantibodies (MAA) prevailing in overlap syndromes, and myositis-specific autoantibodies (MSA), with diagnostic specificity exceeding 90%. Our purpose was to detect retrospectively the prevalence of the most common MSAs in a group of 19 adult DM patients (13 women, 6 men). Read More

    Autoimmune choreas.
    J Neurol Neurosurg Psychiatry 2016 Dec 1. Epub 2016 Dec 1.
    Chorea, a movement disorder characterised by a continuous flow of unpredictable muscle contractions, has a myriad of genetic and non-genetic causes. Although autoimmune processes are rare aetiology of chorea, they are relevant both for researchers and clinicians. The aim of this article is to provide a review of the epidemiology, clinical and laboratory features, pathogenesis and management of the most common autoimmune causes of chorea. Read More

    Non-paraneoplastic autoimmune subepidermal bullous disease associated with fatal bronchiolitis obliterans.
    J Dermatol 2016 Dec 3. Epub 2016 Dec 3.
    Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
    Bronchiolitis obliterans is a small-airway obstructive lung disease for which immunologically mediated pathogenesis is supposed. Frequent association of bronchiolitis obliterans with paraneoplastic pemphigus is well known, but its association with other autoimmune bullous diseases has not been reported except for a case of anti-laminin-332-type mucous membrane pemphigoid in a patient with chronic graft-versus-host disease. We report a case of non-paraneoplastic autoimmune subepidermal bullous disease associated with fatal bronchiolitis obliterans in a patient without transplantation. Read More

    Electrodiagnosis in Cancer Rehabilitation.
    Phys Med Rehabil Clin N Am 2017 Feb;28(1):193-203
    Rehabilitation Medicine Service, Department of Neurology, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA; Division of Rehabilitation Medicine, Weill Cornell Medicine, 525 East 68th Street, New York, NY 10065, USA. Electronic address:
    With numerous advancements in early detection and multimodal therapy, cancer has become a chronic disease. As the number of cancer survivors continues to increase, physiatrists and other neuromuscular disease specialists are more likely to encounter individuals with residual impairments, disabilities, and/or handicaps resulting from cancer or related treatments. The patient with cancer is especially prone to injury directed at the peripheral nervous system at multiple anatomic levels. Read More

    The enlarged extraocular muscle: to relax, reflect or refer?
    Eye (Lond) 2016 Dec 2. Epub 2016 Dec 2.
    Department of Ophthalmology, University Hospital of Coventry and Warwickshire, Coventry, UK.
    PurposeExtraocular muscle enlargement (EOME) is most commonly associated with thyroid eye disease, but there are other causes. We report our outcomes of investigating and managing non-thyroid-related EOME (NTR-EOME).MethodsRetrospective consecutive case series. Read More

    A case of extragastrointestinal stromal tumor complicated by severe hypoglycemia: a unique presentation of a rare tumor.
    BMC Cancer 2016 Dec 1;16(1):930. Epub 2016 Dec 1.
    Departments of Medicine, Indiana University School of Medicine, Indianapolis, USA.
    Background: Non-Islet Cell Tumor Hypoglycemia (NICTH) is a rare paraneoplastic cause of hypoglycemia arising from excess tumor production of insulin-like growth factor. The objective of this report is to describe an unusual case of Extragastrointestinal Stromal Tumor (EGIST) associated NICTH.

    Case Presentation: A 64 year-old African female was brought to the emergency room with a 1-month history of recurrent syncope, weight loss, and abdominal bloating. Read More

    Anti-Hu antibodies activate enteric and sensory neurons.
    Sci Rep 2016 Dec 1;6:38216. Epub 2016 Dec 1.
    Human Biology, Technical University of Munich, Freising, Germany.
    IgG of type 1 anti-neuronal nuclear antibody (ANNA-1, anti-Hu) specificity is a serological marker of paraneoplastic neurological autoimmunity (including enteric/autonomic) usually related to small-cell lung carcinoma. We show here that IgG isolated from such sera and also affinity-purified anti-HuD label enteric neurons and cause an immediate spike discharge in enteric and visceral sensory neurons. Both labelling and activation of enteric neurons was prevented by preincubation with the HuD antigen. Read More

    Pemphigus: Pathogenesis to Treatment.
    R I Med J (2013) 2016 Dec 1;99(12):28-31. Epub 2016 Dec 1.
    Assistant Professor, Department of Dermatology, The Warren Alpert Medical School of Brown University.
    Pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP) are a group of rare and fatal blistering diseases involving autoantibodies that target desmosomal proteins. The pathogenesis of pemphigus involves the production of activated B-cells and IgG with stimulation by IL-4 by T-helper 2 cells. Clinically these diseases present most often with epidermal erosions of the mucosae and skin caused by rapid rupturing of flaccid bullae. Read More

    Paraneoplastic pityriasis rubra pilaris: case report and literature review.
    Clin Exp Dermatol 2017 Jan 29;42(1):54-57. Epub 2016 Nov 29.
    Department of Dermatology, Tel Aviv Sourasky Medical Centre, Tel Aviv, Israel.
    Pityriasis rubra pilaris (PRP; MIM 173200) is an uncommon papulosquamous inflammatory dermatosis. Only a few cases of PRP associated with an underlying malignancy have been documented. We investigated a 59-year-old patient presenting with a fulminant form of PRP recalcitrant to systemic retinoid therapy, in whom the skin disease heralded a diagnosis of cholangiocarcinoma. Read More

    Trousseau's syndrome caused by bladder cancer producing granulocyte colony-stimulating factor and parathyroid hormone-related protein: A case report.
    Oncol Lett 2016 Nov 20;12(5):4214-4218. Epub 2016 Sep 20.
    Department of Urology, Graduate School of Medicine and Pharmaceutical Sciences for Research, University of Toyama, Toyama 930-0194, Japan.
    The present study reports a rare case of bladder cancer that caused Trousseau's syndrome, a malignancy-associated hypercoagulative state leading to venous or arterial thrombosis. A 38-year-old Japanese female patient experienced a sudden onset of visual field defects and lower limb weakness. Cerebral magnetic resonance imaging revealed multi-regional hypointense areas compatible with acute cerebral infarction. Read More

    Blockade of the IL-6 trans-signalling/STAT3 axis suppresses cachexia in Kras-induced lung adenocarcinoma.
    Oncogene 2016 Nov 28. Epub 2016 Nov 28.
    Centre for Innate Immunity and Infectious Diseases, Hudson Institute of Medical Research, Clayton, Victoria, Australia.
    Lung cancer is the leading cause of cancer death worldwide, and is frequently associated with the devastating paraneoplastic syndrome of cachexia. The potent immunomodulatory cytokine interleukin (IL)-6 has been linked with the development of lung cancer as well as cachexia; however, the mechanisms by which IL-6 promotes muscle wasting in lung cancer cachexia are ill-defined. In this study, we report that the gp130(F/F) knock-in mouse model displaying hyperactivation of the latent transcription factor STAT3 via the common IL-6 cytokine family signalling receptor, gp130, develops cachexia during Kras-driven lung carcinogenesis. Read More

    [Autoimmune and inflammatory disorders associated with lymphoid hematological malignancies].
    Rev Med Interne 2016 Nov 24. Epub 2016 Nov 24.
    Service de médecine interne, université Paris 6, hôpital Saint-Antoine, DHU i2B, AP-HP, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France.
    In this literature review, we reported autoimmune and inflammatory disorders associated with lymphoid hematological malignancies, including non-Hodgkin's lymphoma, Hodgkin's lymphoma and chronic lymphocytic leukemia. The different types of systemic involvement are classified by affected organ. We listed in this review the joint diseases, skin, neurologic, hematologic, renal, and vasculitis. Read More

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