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International Journal of Rheumatology
Volume 2012, Article ID 283459, 6 pages
Clinical Study

Evaluation and Clinical Validity of a New Questionnaire for Mikulicz's Disease

1First Department of Internal Medicine, School of Medicine, Sapporo Medical University, Hokkaido 0608543, Japan
2Advanced Clinical Research Center, The Institute of Medical Science, The University of Tokyo, Tokyo 1088639, Japan

Received 10 October 2011; Revised 9 January 2012; Accepted 20 February 2012

Academic Editor: John H. Stone

Copyright © 2012 Motohisa Yamamoto et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Objectives. The characteristic features of Mikulicz’s disease (MD) are diffuse enlargement of the lacrimal and submandibular glands, elevated levels of serum immunoglobulin (Ig)G4, and abundant infiltration of IgG4-positive plasmacytes into both glands. No disease index is available to properly evaluate MD, so we developed a functional assessment of MD, the Mikulicz’s disease activity questionnaire (MAQ), and evaluated its clinical efficacy. Methods. We selected 18 patients who were either being treated for MD or who had presented with recurrence. The patients completed a self-assessment and were scored according to the MAQ sheet during each visit between December 2009 and August 2011. Assessment items were in regard to increases or decreases in lacrimal and salivary gland enlargement and severity of sicca symptoms. Results. On the first visits, MAQ scores were high, but scores decreased rapidly as treatment progressed. When doses of glucocorticoid were reduced, some patients showed increased scores. Dry-symptom scores increased initially. MAQ scores for patients with recurrent MD gradually increased over several months before relapse. However, some patients displayed no elevation in MAQ scores due to relapses at other sites. Conclusion. MAQ score can be used to quantify flares and treatment response and is useful for functional assessment of MD.