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Primary intestinal autoimmune disease as a cause of chronic intestinal pseudo-obstruction
  1. S Ghirardo1,*,
  2. B Sauter1,*,
  3. G Levy2,
  4. I M Fiel2,
  5. T Schiano3,
  6. G Gondolesi3
  1. 1Intestinal Rehabilitation and Transplantation, Recanati Miller Transplantation Institute, The Mount Sinai Medical Center, New York, USA
  2. 2Department of Pathology, The Mount Sinai Medical Center, New York, USA
  3. 3Intestinal Rehabilitation and Transplantation, Recanati Miller Transplantation Institute, The Mount Sinai Medical Center, New York, USA
  1. Correspondence to:
    G Gondelesi
    Surgical Director of Intestinal Rehabilitation and Transplantation, The Mount Sinai Medical Center, One Gustave L Levy Place, Box 1104, New York, NY 10029-6574, USA; gabriel.gondolesimsnyuhealth.org

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The purpose of this letter is to elucidate on the pathophysiology of a disease that is often considered to be idiopathic. Chronic intestinal pseudo-obstruction (CIPO) is a clinical syndrome characterised by ineffective intestinal propulsion in the absence of organic intestinal obstruction. It is a common cause of intestinal failure requiring total parenteral nutrition (TPN). It can be either a primary/idiopathic (neurogenic or myogenic) disorder or secondary to another recognised underlying disease. Most cases of childhood CIPO are congenital enteral neuromuscular diseases; however, neuropathy due to Hirschprung’s disease, Chagas disease, infections, and toxins occur in later childhood. In adults, most cases of CIPO are secondary to progressive systemic sclerosis, dermatomyositis, systemic lupus erythematosus, rheumatoid arthritis, and Sjogren’s syndrome.1,2

We report a case of an adult onset of CIPO secondary to an autoimmune process affecting exclusively the small intestine without any other systemic organ involvement. A 53 year old Black man with an unremarkable past medical history experienced symptoms of “mechanical obstruction” (nausea/vomiting). After three abdominal explorations, including small bowel resections, he failed enteral feeding rendering him fully TPN dependent. Antroduodenal manometry demonstrated low amplitude contractions in the distal duodenum, and gastrointestinal scintigraphy revealed normal stomach emptying and colonic transit, but delayed small bowel transit. Trypanosoma cruzi antibodies and an extensive serological work up for collagen-vascular disease were negative, except for antinuclear antibody (ANA 1/1280). During five years on TPN, the patient developed multiple episodes of line sepsis and progressive liver disease. He then successfully underwent isolated intestinal transplantation.

Intraoperatively, the small bowel was dilated only in the proximal 270 cm (18 cm circumference). Microscopic examination showed marked degeneration of the muscularis propria with pronounced atrophy of muscle fibres (fig 1). Eosinophilic hyaline globular inclusions were detected within smooth muscle cells, predominantly in the perinuclear regions. Masson-trichrome stain revealed fibrous tissue deposition around atrophic muscle bundles. The neuronal plexus was entirely preserved. Histological findings were compatible with an idiopathic visceral myopathy.3 Positive immunofluorescence staining for anti-IgG and anti-IgA was found in degenerated muscle fibres but not in areas of intact musculature (fig 1). Nine months post transplant, a full thickness biopsy of the intestine showed no evidence of recurrent disease in the graft. The patient’s ANA became negative one month after transplant and remained undetectable after 15 months of follow up.

Figure 1

 (A) Thinning of the small bowel wall with normal appearing mucosa and inner circular muscular layer. The outer longitudinal layer is severely thinned and some muscle fibres contain cytoplasmic globules. (B, C) Immunofluorescent study using anti-IgA (B) and anti-IgG (C). Positive green fluorescent staining is seen along the edges of degenerated muscle fibres of the outer longitudinal layer.

Only one similar case of a two year old boy who developed intestinal pseudo-obstruction following an episode of gastroenteritis has been reported.4 In that case, ANA, antineutrophil cytoplasmic, and antismooth muscle antibodies became negative on immunosuppressive therapy. Histology after two years of treatment showed profound loss of myocytes in the outermost circular muscle layer with T lymphocyte infiltration. Deposition of (auto)antibodies was not mentioned. Other cases of CIPO and systemic autoimmune disorders have been published.5–7

Our report is the first to describe an adult without previous gastrointestinal symptoms or other signs of systemic autoimmune disease who developed subacute ANA positive CIPO, resulting in myocytolysis of the intestinal muscularis propria. Documentation of IgG and IgA deposits in the areas of muscle degeneration and fibrosis is suggestive of an autoimmune-type disease involving the humoral immune system. The findings however do not exclude a role for cell mediated cytotoxicity at the beginning of the disease and may only represent a late stage of a complex autoimmune disorder.

In summary, some patients with idiopathic CIPO may suffer from a primary intestinal autoimmune disease, an autoimmune process exclusively directed towards the intestine. An early full thickness intestinal biopsy may indicate the need for immunosuppression. At late stages, timely intestinal transplantation is an acceptable option before patients develop irreversible liver disease.8,9,10

References

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Footnotes

  • * S Ghirardo and B Sauter contributed equally to this letter.

  • Conflict of interest: none declared

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