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Autoimmune Gastrointestinal Paralysis: Failure of Conventional Treatment without Immunomodulation.

Weinkauf C, McPhillips S, Krouse R, Levine I - Case Rep Surg (2014)

Bottom Line: 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).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.

View Article: PubMed Central - PubMed

Affiliation: University of Arizona, 1501 N Campbell, Tucson, AZ 85721, USA.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus

Mid-power cross section showing lymphocytic infiltrate into the myenteric plexus.
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Related In: Results  -  Collection


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fig2: Mid-power cross section showing lymphocytic infiltrate into the myenteric plexus.

Mentions: Postoperatively, the patient was started on TPN. He remained stable but had no signs of bowel function recovery despite treatment with methylnaltrexone, erythromycin, metoclopramide, senna, enemas, and bisacodyl suppositories. The patient developed paroxysmal atrial flutter and atrial fibrillation and his blood pressure and heart rate were markedly labile. Initially, intravenous metoprolol and later oral scheduled metoprolol controlled his heart rate. After his atypical course, we requested reexamination of the proximal portion of resected bowel (the region that was aperistaltic). This showed lymphoplasmacytic infiltrate consistent with myenteric ganglioneuritis (Figures 1 and 2). This supported our new working diagnosis of AP-CIPO. Based on the limited literature available to guide therapy, a trial of octreotide (100 mcg BID) was initiated and was increased to 200 mcg TID [2]. There was no clinical benefit of octreotide during his month course. Later, a lab showed anti-Hu antibodies, further reinforcing the diagnosis of AP-CIPO. After discussion with the patient and his oncologists, it was decided that chemotherapy for the lung cancer would be initiated. His regimen was started with a dexamethasone taper, cisplatin, and etoposide.


Autoimmune Gastrointestinal Paralysis: Failure of Conventional Treatment without Immunomodulation.

Weinkauf C, McPhillips S, Krouse R, Levine I - Case Rep Surg (2014)

Mid-power cross section showing lymphocytic infiltrate into the myenteric plexus.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4209773&req=5

fig2: Mid-power cross section showing lymphocytic infiltrate into the myenteric plexus.
Mentions: Postoperatively, the patient was started on TPN. He remained stable but had no signs of bowel function recovery despite treatment with methylnaltrexone, erythromycin, metoclopramide, senna, enemas, and bisacodyl suppositories. The patient developed paroxysmal atrial flutter and atrial fibrillation and his blood pressure and heart rate were markedly labile. Initially, intravenous metoprolol and later oral scheduled metoprolol controlled his heart rate. After his atypical course, we requested reexamination of the proximal portion of resected bowel (the region that was aperistaltic). This showed lymphoplasmacytic infiltrate consistent with myenteric ganglioneuritis (Figures 1 and 2). This supported our new working diagnosis of AP-CIPO. Based on the limited literature available to guide therapy, a trial of octreotide (100 mcg BID) was initiated and was increased to 200 mcg TID [2]. There was no clinical benefit of octreotide during his month course. Later, a lab showed anti-Hu antibodies, further reinforcing the diagnosis of AP-CIPO. After discussion with the patient and his oncologists, it was decided that chemotherapy for the lung cancer would be initiated. His regimen was started with a dexamethasone taper, cisplatin, and etoposide.

Bottom Line: 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).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.

View Article: PubMed Central - PubMed

Affiliation: University of Arizona, 1501 N Campbell, Tucson, AZ 85721, USA.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus