Abstract
Sjögren's syndrome (SS) in its classical form, which includes kerato-conjunctivitis sicca, xerostomia and recurrent enlargement of the salivary glands, is associated with a connective tissue disease in at least half the patients.
According to the present study of three patients with SS, achalasia and gastric hyposecretion seem to be either further manifestations of SS, or separate phenomena associated with SS. The gastric hyposecretion involves both the hydrochloric acid and the total volume of the secretion, but the gastric mucosa has a normal appearance on microscopy.
Because of the simultaneous presence of achalasia, gastric hyposecretion and reduced salivation, we have called the combination “achalasia sicca”. The reduction in the secretions of the upper gastrointestinal tract might have a pathogenic association with achalasia.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Athreya, B. H., Norman, M. E., Myers, A. R., South, M. A.: Sjögren's syndrome in children. Pediatrics 59, 931–938 (1977)
Bartsocas, S., Michail, J.: Sur un cas rare de syndrome de Gouregot-Houwers-Sjögren ou “Kerodermostéose” avec développment précoce des organes génitaux et du corps, chez un enfant. Arch. Franc. Ped. 9, 266–269 (1952)
Biasini, G. C., Acito, P., Agostini, U.: Un caso di sindrome di Sjögren in eta pediatrica. Clinica Pediat. (Bologna) 45, 47–59 (1963)
Duncan, H., Epker, B. N., Sheldon, G. M.: Sjögren's syndrome in childhood: Report of a case. Henry Ford Hosp. Med. J. 17, 35–41 (1969)
Eliacher, E., Perelman, R., Hambourg, M., Levasseur, C., Marie, J.: Syndrome de type “Gouregot-Sjögren” chez un enfant. Ann. Pediat. (Paris) 13, 184–186 (1966)
Koivukangas, T., Similä, S., Heikkinen, E., Räsänen, O., Wasz-Höckert, O.: Sjögren's syndrome and achalasia of the cardia in two siblings. Pediatrics 51, 943–946 (1973)
Ludwiczak, H., Tomaszewska, E.: Zespól Sjögrena u 13-letniej dziewczynki. Pediat. Pol. 45, 585–587 (1970)
O'Neill, E. M.: Sjögren's syndrome with onset at 10 years of age. Proc. Roy. Soc. Med. 58, 689–690 (1965)
Rudobielska, M., Lewicka, J., Krasowska, I., Zaleski, B.: Przypadek zespolu Sjögrena lacznie z cukrzyca u 8-letniej dziewczynki. Wiad. Lek. 26, 857–860 (1973)
Shearn, M. A.: Sjögren's syndrome. Major Problems in Internal Medicine Series. Vol. 2. Philadelphia: W. B. Saunders Co. 1971
Strickland, R. G., Mackay, I. R.: A reappraisal of the nature and significance of chronic atrophic gastritis. Dig. Dis. 18, 426–440 (1973)
Wegelius, O., Fyhrquist, F., Adner, P.-L.: Sjögren's syndrome associated with vitamin B12 deficiency. Acta Rheum. Scand. 16, 184–190 (1970)
Weissmann, G.: Sjögren's syndrome. Review of the literature and report of a case with achalasia of the esophagus. Amer. J. Med. 24, 475–481 (1958)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Similä, S., Kokkonen, J. & Kaski, M. Achalasia sicca-juvenile Sjögren's syndrome with achalasia and gastric hyposecretion. Eur J Pediatr 129, 175–181 (1978). https://doi.org/10.1007/BF00442161
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00442161