Autoimmune enteric leiomyositis: A rare cause of chronic intestinal pseudo-obstruction with specific morphological features

• Published in: Human pathology Vol. 36; no. 5; pp. 576 - 580
• Main Authors: Haas, Susanne, Bindl, Lutz, Fischer, Hans-Peter
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01.05.2005; Elsevier; Elsevier Limited
• Subjects: Autoimmune diseases; Autoimmune Diseases - complications; Autoimmune Diseases - immunology; Autoimmune Diseases - pathology; Autoimmune hepatitis; Autoimmune leiomyositis; Biological and medical sciences; Child, Preschool; Children; Children & youth; Chronic intestinal pseudo-obstruction; Cytotoxic T cells; Cytotoxicity; Diagnosis, Differential; Disease; Female; Gastroenterology. Liver. Pancreas. Abdomen; Hepatitis; Hepatitis - complications; Hepatitis - immunology; Humans; Immune system; Immunohistochemistry; Infant; Intestinal Mucosa - immunology; Intestinal Mucosa - pathology; Intestinal Pseudo-Obstruction - etiology; Intestinal Pseudo-Obstruction - immunology; Intestinal Pseudo-Obstruction - pathology; Intestine, Small - immunology; Intestine, Small - pathology; Investigative techniques, diagnostic techniques (general aspects); Liver. Biliary tract. Portal circulation. Exocrine pancreas; Lymphocytes; Medical sciences; Motility; Muscle, Smooth - immunology; Muscle, Smooth - pathology; Muscular system; Other diseases. Semiology; Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques; Smooth muscle; Chronic intestinal pseudo-obstruction; Autoimmune leiomyositis; Autoimmune hepatitis; Children; Cytotoxic T cells; Human; Autoimmunity; Digestive system; Gut; Hepatic disease; Autoimmune disease; Obstruction; Hepatitis; Anatomic pathology; Chronic; Morphology; Cause; Digestive diseases; Child; Cytotoxic T lymphocyte
• ISSN: 0046-8177; 1532-8392
• DOI: 10.1016/j.humpath.2005.01.005

Autoimmune enteric leiomyositis is an extraordinary rare cause of acquired chronic intestinal pseudo-obstruction in children. We report a 5-year-old girl who developed chronic intestinal pseudo-obstruction 3 years after an autoimmune hepatitis. Mucosal biopsies of the upper gastrointestinal tract and colon showed minimal inflammatory changes. On full-thickness biopsies of the small intestine, a dense lymphocytic infiltrate of the muscularis propria was seen, mainly consisting of cytotoxic T lymphocytes. Smooth muscle fibers were degenerated and diminished, but the myenteric plexus was intact. The coexistence of an autoimmune hepatitis in our case indicates an expansion of autoreactive T cells to homologous self-antigens. It is of practical importance for histopathological diagnosis that inflammation in autoimmune enteric leiomyositis affects the muscularis propria of the small intestine, whereas mucosa and submucosa do not show severe inflammatory changes. Therefore, correct diagnosis may be missed in peroral and peranal mucosal biopsies, but full-thickness biopsies are required.