Skip to main content
PMC home page
Gut logoLink to Gut
. 1993 Jul;34(7):999–1001. doi: 10.1136/gut.34.7.999

Myofibroblasts in hollow visceral myopathy: the origin of gastrointestinal fibrosis?

J E Martin 1, M Benson 1, M Swash 1, V Salih 1, A Gray 1
PMCID: PMC1374242  PMID: 8344591

Abstract

A patient with hollow visceral myopathy is reported in whom light microscopical studies of the small and large intestine showed typical features of degeneration, thinning, and fibrous replacement of smooth muscle of the gastrointestinal tract. Electron microscopy showed a striking increase in collagen with minimal fibroblast proliferation. Smooth muscle fibres had a range of ultrastructural abnormalities including myofilament disarray, electron lucency of the cytoplasm, and proliferation of the endoplasmic reticulum. Some fibres seemed to have typical ultrastructural characteristics of myofibroblasts, and others to be transition forms between typical smooth muscle cells and typical myofibroblasts. It seems likely that the fibrosis typical of this disorder has its origin in the transformation of smooth muscle fibres from a purely contractile to a myofibroblast collagen synthetic phenotype.

Full text

PDF
999

Images in this article

999Figure 1
on p.999
1000Figure 2
on p.1000
1000Figure 3
on p.1000
1001Figure 4
on p.1001

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Adler K. B., Low R. B., Leslie K. O., Mitchell J., Evans J. N. Contractile cells in normal and fibrotic lung. Lab Invest. 1989 Apr;60(4):473–485. [PubMed] [Google Scholar]
  2. Balázs M., Kovács A. The 'transitional' mucosa adjacent to large bowel carcinoma--electron microscopic features and myofibroblast reaction. Histopathology. 1982 Sep;6(5):617–629. doi: 10.1111/j.1365-2559.1982.tb02755.x. [DOI] [PubMed] [Google Scholar]
  3. Feigl W., Susani M., Ulrich W., Matejka M., Losert U., Sinzinger H. Organisation of experimental thrombosis by blood cells. Evidence of the transformation of mononuclear cells into myofibroblasts and endothelial cells. Virchows Arch A Pathol Anat Histopathol. 1985;406(2):133–148. doi: 10.1007/BF00737081. [DOI] [PubMed] [Google Scholar]
  4. Fitzgibbons P. L., Chandrasoma P. T. Familial visceral myopathy. Evidence of diffuse involvement of intestinal smooth muscle. Am J Surg Pathol. 1987 Nov;11(11):846–854. [PubMed] [Google Scholar]
  5. François Y., Descos L., Berger F., Beurlet J., Cenni J. C., Mallet-Guy Y., Vignal J. Rare type of visceral myopathy mimicking anorexia nervosa. J R Soc Med. 1990 Nov;83(11):748–749. doi: 10.1177/014107689008301125. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Gabbiani G., Hirschel B. J., Ryan G. B., Statkov P. R., Majno G. Granulation tissue as a contractile organ. A study of structure and function. J Exp Med. 1972 Apr 1;135(4):719–734. doi: 10.1084/jem.135.4.719. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Gabbiani G., Ryan G. B., Majne G. Presence of modified fibroblasts in granulation tissue and their possible role in wound contraction. Experientia. 1971 May 15;27(5):549–550. doi: 10.1007/BF02147594. [DOI] [PubMed] [Google Scholar]
  8. Hwang W. S., Kelly J. K., Shaffer E. A., Hershfield N. B. Collagenous colitis: a disease of pericryptal fibroblast sheath? J Pathol. 1986 May;149(1):33–40. doi: 10.1002/path.1711490108. [DOI] [PubMed] [Google Scholar]
  9. Krishnamurthy S., Schuffler M. D. Pathology of neuromuscular disorders of the small intestine and colon. Gastroenterology. 1987 Sep;93(3):610–639. doi: 10.1016/0016-5085(87)90926-7. [DOI] [PubMed] [Google Scholar]
  10. Lipper S., Kahn L. B., Reddick R. L. The myofibroblast. Pathol Annu. 1980;15(Pt 1):409–441. [PubMed] [Google Scholar]
  11. Majno G., Gabbiani G., Hirschel B. J., Ryan G. B., Statkov P. R. Contraction of granulation tissue in vitro: similarity to smooth muscle. Science. 1971 Aug 6;173(3996):548–550. doi: 10.1126/science.173.3996.548. [DOI] [PubMed] [Google Scholar]
  12. Mitros F. A., Schuffler M. D., Teja K., Anuras S. Pathologic features of familial visceral myopathy. Hum Pathol. 1982 Sep;13(9):825–833. doi: 10.1016/s0046-8177(82)80079-8. [DOI] [PubMed] [Google Scholar]
  13. Richman P. I., Tilly R., Jass J. R., Bodmer W. F. Colonic pericrypt sheath cells: characterisation of cell type with new monoclonal antibody. J Clin Pathol. 1987 Jun;40(6):593–600. doi: 10.1136/jcp.40.6.593. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Roche W. R. Myofibroblasts. J Pathol. 1990 Aug;161(4):281–282. doi: 10.1002/path.1711610402. [DOI] [PubMed] [Google Scholar]
  15. Ross R., Klebanoff S. J. Fine structural changes in uterine smooth muscle and fibroblasts in response to estrogen. J Cell Biol. 1967 Jan;32(1):155–167. doi: 10.1083/jcb.32.1.155. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Ross R., Klebanoff S. J. The smooth muscle cell. I. In vivo synthesis of connective tissue proteins. J Cell Biol. 1971 Jul;50(1):159–171. doi: 10.1083/jcb.50.1.159. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Schuffler M. D., Lowe M. C., Bill A. H. Studies of idiopathic intestinal pseudoobstruction. I. Hereditary hollow visceral myopathy: clinical and pathological studies. Gastroenterology. 1977 Aug;73(2):327–338. [PubMed] [Google Scholar]
  18. Schuffler M. D., Pope C. E., 2nd Studies of idiopathic intestinal pseudoobstruction. II. Hereditary hollow visceral myopathy: family studies. Gastroenterology. 1977 Aug;73(2):339–344. [PubMed] [Google Scholar]
  19. Schuffler M. D., Rohrmann C. A., Chaffee R. G., Brand D. L., Delaney J. H., Young J. H. Chronic intestinal pseudo-obstruction. A report of 27 cases and review of the literature. Medicine (Baltimore) 1981 May;60(3):173–196. [PubMed] [Google Scholar]
  20. Schürch W., Seemayer T. A., Lagacé R., Gabbiani G. The intermediate filament cytoskeleton of myofibroblasts: an immunofluorescence and ultrastructural study. Virchows Arch A Pathol Anat Histopathol. 1984;403(4):323–336. doi: 10.1007/BF00737283. [DOI] [PubMed] [Google Scholar]
  21. Skalli O., Schürch W., Seemayer T., Lagacé R., Montandon D., Pittet B., Gabbiani G. Myofibroblasts from diverse pathologic settings are heterogeneous in their content of actin isoforms and intermediate filament proteins. Lab Invest. 1989 Feb;60(2):275–285. [PubMed] [Google Scholar]
  22. Tomasek J. J., Schultz R. J., Episalla C. W., Newman S. A. The cytoskeleton and extracellular matrix of the Dupuytren's disease "myofibroblast": an immunofluorescence study of a nonmuscle cell type. J Hand Surg Am. 1986 May;11(3):365–371. doi: 10.1016/s0363-5023(86)80143-5. [DOI] [PubMed] [Google Scholar]

Articles from Gut are provided here courtesy of BMJ Publishing Group

RESOURCES