Acute cerebral infarction associated with thrombocytopenia in primary Sjogren's syndrome : A Case Report

저혈소판증을 동반한 급성 대뇌경색을 보인 원발성 쇼그렌 증후군 1례

  • Choi, Pahn Kyu (Department of Neurology, Chosun University School of Medicine) ;
  • Kang, Hyun Goo (Department of Neurology, Chosun University School of Medicine)
  • 최판규 (조선대학교의과대학 신경과학 교실) ;
  • 강현구 (조선대학교의과대학 신경과학 교실)
  • Received : 2017.05.08
  • Accepted : 2017.07.07
  • Published : 2017.07.31


Sjogren's syndrome is an autoimmune disease characterized by dry mouth and neutropenia. Although it does not commonly involve the central nervous system, Sjogren's syndrome sometimes affects small vessels through microangiopathic alterations. A 34-year-old woman was hospitalized for left upper quadrantanopia and a tingling sensation in the left hemibody. Brain magnetic resonance imaging revealed acute infarction in the right posterior cerebral artery territory. In laboratory tests, antinuclear (FANA2+) and anti-DNA antibodies (anti-SS-A (Ro)) were detected. Salivary gland scintigraphy revealed moderately decreasedexcretion of saliva. Based on these findings, we concluded she had Sjogren's syndrome. As in this patient, large vessel involvement in Sjogren's syndrome is far less common. Furthermore, it is difficult to administer antiplatelet drugsto patients with thrombocytopenia in Sjogren's syndrome. This is a case of the patient with Sjogren's syndrome that involved thrombocytopenia and large vessel invasion who was treated with antiplatelet drugs and hydroxychloroquine.


Autoimmune disease;Neutropenia;Sjogren's syndrome;Stroke;Thrombocytopenia


  1. Fauci A. S., Martin J. B., Braunwald E., AKasper D. L., Isselbacher K. J., Hauser S. L., "Sjogren's Syndrome", Herrison'sprinciplesofinternalmedicine. 14th ed. NewYork McGraw-Hill, pp. 1901-02, 1998.
  2. M. Govoni, G. Bajocchi, N. Rizzo, M. R. Tola, L. Caniatti, V. Tugnoli, P. Colamussi, and F. Trotta, "Neurological Involvement in Primary Sjogren's Syndrome: Clinical and Instrumental Evaluation in a Cohort of Italian Patients", Clin Rheumatol,18, pp. 299-303, 1999. DOI:
  3. B. Zoller, X. Li, J. Sundquist,"Risk of subsequent ischemic and hemorrhagic stroke in patients hospitalized for immune-mediated diseases: a nationwide follow-up study from Sweden", BMC Neurol, 12, p. 41, 2012. DOI:
  4. Y. Ii, A. Shindo, R. Sasaki, "Reversible stenosis of large cerebral arteries in a patient with combined Sjogren's syndrome and neuromyelitis optica spectrum disorder", Rheumatol Int, 28, pp. 1277-1280, 2008. DOI:
  5. Y. Matsuki, M. Kawakami, T. Ishizuka, "SLE and Sjogren's syndrome associated with unilateral moyamoya vessels in cerebral arteries", Scand J Rheumatol, 26, pp. 392-394, 1997. DOI:
  6. S. Nagahiro, A. Mantani, K. Yamada,."Multiple cerebral arterial occlusions in a young patient with Sjogren's syndrome: case report" Neurosurgery, 38, pp. 592-595, 1996. DOI:
  7. P. Manganelli, P. Fietta, and F. Quaini, "Hematologic Manifestations of Primary Sjogren's Syndrome", Clin Exp Rheumatol, 24, pp. 438-48, 2006.
  8. J. Friedman, A. Klepfish, E. B. Miller, V. Ognenovski, R. W. Ike, and A. Schattner, "Agranulocytosis in Sjogren's Syndrome: Two Case Reports and Analysis of 11 Additional Reported Cases", Semin Arthritis Rheum, 31, pp. 338-45, 2002. DOI:
  9. C. Lafitte, Z. Amoura, P. Cacoub, P. Pradat-Diehl, C. Picq, F. Salachas, J. M. Leger, J. C. Piette, and J. Y. Delattre, "Neurological Complications of Primary Sjogren's Syndrome", J Neurol, 248, pp. 577-84, 2001. DOI:
  10. malinow K, Yannakakis GD, Glusman SM, Edlow DW, Griffin J, Pestronk A, "subacute sensory neuronopathy secondary to dorsal root ganglionitis in primary sjgren's syndrome", Ann Neurol vol. 20, pp. 535-537, 1986. DOI: