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Clinical course and treatment outcome of SS and MALT lymphoma were evaluated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results.<\/jats:title><jats:p>From a total of 329 patients with SS, 35 MALT-SS patients were identified, with a median followup of 76 months (range 16\u2013153 mo). MALT lymphoma was localized in the parotid gland in all cases. Treatment consisted of \u201cwatchful waiting\u201d (n = 10), surgery (n = 3), radiotherapy (n = 1), surgery combined with radiotherapy (n = 2), rituximab only (n = 13), or rituximab combined with chemotherapy (n = 6). Complete response was observed in 14 patients, partial response in 1 patient, and stable disease in 20 patients. In 6 of 7 patients with initially high SS disease activity (M-protein, cryoglobulins, IgM rheumatoid factor &gt; 100 KIU\/l, severe extraglandular manifestations), MALT lymphoma progressed and\/or SS disease activity increased after a median followup of 39 months (range 4\u201398 mo), necessitating retreatment. Only 1 patient with MALT who had low SS disease activity showed progression of lymphoma when left untreated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion.<\/jats:title><jats:p>An initially high SS disease activity likely constitutes an adverse prognostic factor for progression of lymphoma and\/or SS. Such patients may require treatment for both MALT lymphoma and SS. In SS patients with localized asymptomatic MALT lymphoma and low SS disease activity, a \u201cwatchful waiting\u201d strategy seems justified.<\/jats:p><\/jats:sec>","DOI":"10.3899\/jrheum.110077","type":"journal-article","created":{"date-parts":[[2011,8,16]],"date-time":"2011-08-16T02:29:19Z","timestamp":1313461759000},"page":"2198-2208","source":"Crossref","is-referenced-by-count":82,"title":["Treatment of Mucosa-associated Lymphoid Tissue Lymphoma in Sj\u00f6gren\u2019s Syndrome: A Retrospective Clinical Study"],"prefix":"10.3899","volume":"38","author":[{"given":"RODNEY P.E.","family":"POLLARD","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"JUSTIN","family":"PIJPE","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"HENDRIKA","family":"BOOTSMA","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"FRED 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