Autoimmune Gastrointestinal Paralysis: Failure of Conventional Treatment without Immunomodulation

The treatment of the rare enteric nervous system (ENS) manifestations of paraneoplastic syndromes, which are most frequently associated with small cell lung cancer (SCLC), is poorly understood and described. Patients with neuroendocrine-derived tumors can develop B-cell reactivity towards the tumor...

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Main Authors: Craig Weinkauf, Sean McPhillips, Robert Krouse, Ira Levine
Format: Article
Language:English
Published: Wiley 2014-01-01
Series:Case Reports in Surgery
Online Access:http://dx.doi.org/10.1155/2014/180654
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author Craig Weinkauf
Sean McPhillips
Robert Krouse
Ira Levine
author_facet Craig Weinkauf
Sean McPhillips
Robert Krouse
Ira Levine
author_sort Craig Weinkauf
collection DOAJ
description The treatment of the rare enteric nervous system (ENS) manifestations of paraneoplastic syndromes, which are most frequently associated with small cell lung cancer (SCLC), is poorly understood and described. Patients with neuroendocrine-derived tumors can develop B-cell reactivity towards the tumor with cross-reactivity for neurons located in the submucosal and myenteric ganglia of the ENS. The ensuing autoimmune neuritis causes aperistalsis and severe gastrointestinal (GI) dysfunction. Immune-directed therapy is not the standard of care but may be paramount for patient recovery. Our patient, a 63-year-old man with recent symptoms of esophageal dysmotility and newly diagnosed SCLC was hospitalized with nausea, emesis, and constipation. After an extensive work-up that included laparoscopy and celiotomy with bowel resection, we diagnosed what we refer to as Autoimmune Paraneoplastic Chronic Intestinal Pseudoobstruction (AP-CIPO). Unlike the few clinically similar reports, SCLC and AP-CIPO were diagnosed in our patient within weeks of each other, which presented the dilemma of treating the two processes simultaneously. In this report, we review the relevant literature and describe our patient’s course. We believe standard chemotherapy is not effective treatment for AP-CIPO. Based on evidence discussed herein, we suggest initiating autoimmune-directed therapy before or simultaneous with cancer-directed therapy.
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spelling doaj-art-8c0ec04f8896487e950509bb7a5526372025-02-03T05:50:50ZengWileyCase Reports in Surgery2090-69002090-69192014-01-01201410.1155/2014/180654180654Autoimmune Gastrointestinal Paralysis: Failure of Conventional Treatment without ImmunomodulationCraig Weinkauf0Sean McPhillips1Robert Krouse2Ira Levine3University of Arizona, 1501 N Campbell, Tucson, AZ 85721, USAUniversity of Arizona, 1501 N Campbell, Tucson, AZ 85721, USASouthern Arizona VA Health Care System, 3601 S 6th, Tucson, AZ 85723, USASouthern Arizona VA Health Care System, 3601 S 6th, Tucson, AZ 85723, USAThe treatment of the rare enteric nervous system (ENS) manifestations of paraneoplastic syndromes, which are most frequently associated with small cell lung cancer (SCLC), is poorly understood and described. Patients with neuroendocrine-derived tumors can develop B-cell reactivity towards the tumor with cross-reactivity for neurons located in the submucosal and myenteric ganglia of the ENS. The ensuing autoimmune neuritis causes aperistalsis and severe gastrointestinal (GI) dysfunction. Immune-directed therapy is not the standard of care but may be paramount for patient recovery. Our patient, a 63-year-old man with recent symptoms of esophageal dysmotility and newly diagnosed SCLC was hospitalized with nausea, emesis, and constipation. After an extensive work-up that included laparoscopy and celiotomy with bowel resection, we diagnosed what we refer to as Autoimmune Paraneoplastic Chronic Intestinal Pseudoobstruction (AP-CIPO). Unlike the few clinically similar reports, SCLC and AP-CIPO were diagnosed in our patient within weeks of each other, which presented the dilemma of treating the two processes simultaneously. In this report, we review the relevant literature and describe our patient’s course. We believe standard chemotherapy is not effective treatment for AP-CIPO. Based on evidence discussed herein, we suggest initiating autoimmune-directed therapy before or simultaneous with cancer-directed therapy.http://dx.doi.org/10.1155/2014/180654
spellingShingle Craig Weinkauf
Sean McPhillips
Robert Krouse
Ira Levine
Autoimmune Gastrointestinal Paralysis: Failure of Conventional Treatment without Immunomodulation
Case Reports in Surgery
title Autoimmune Gastrointestinal Paralysis: Failure of Conventional Treatment without Immunomodulation
title_full Autoimmune Gastrointestinal Paralysis: Failure of Conventional Treatment without Immunomodulation
title_fullStr Autoimmune Gastrointestinal Paralysis: Failure of Conventional Treatment without Immunomodulation
title_full_unstemmed Autoimmune Gastrointestinal Paralysis: Failure of Conventional Treatment without Immunomodulation
title_short Autoimmune Gastrointestinal Paralysis: Failure of Conventional Treatment without Immunomodulation
title_sort autoimmune gastrointestinal paralysis failure of conventional treatment without immunomodulation
url http://dx.doi.org/10.1155/2014/180654
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