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We describe a novel case of<jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>gG4\u2010<jats:styled-content style=\"fixed-case\">RD<\/jats:styled-content>with hypergammaglobulinemic hyperviscosity responsive to fludarabine and rituximab. A 33\u2010year\u2010old Asian man developed bilateral lacrimal gland and submandibular salivary gland swelling with cervical lymphadenopathy. Biopsies of the affected tissues revealed reactive follicular hyperplasia. Seven years later, he presented with bilateral retinal hemorrhages due to hyperviscosity syndrome from profound polyclonal increase in<jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>gG, including marked<jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>gG4 elevation. Despite plasmapheresis, overproduction of<jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>gG continued and he was refractory to systemic steroids, azathioprine, interferon alpha, and cyclophosphamide. IgG4\u2010<jats:styled-content style=\"fixed-case\">RD<\/jats:styled-content>was suspected following a myocardial infarction and detection of aneurysmal coronary arteries indicating large vessel vasculitis. Review of the cervical lymph node and lacrimal gland biopsies with immunohistochemical staining for<jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>gG4\u2010positive plasma cells confirmed<jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>gG4\u2010<jats:styled-content style=\"fixed-case\">RD<\/jats:styled-content>. B\u2010cell depletion with rituximab produced a partial response, but clinical symptoms and elevated protein levels persisted. Fludarabine was added to rituximab to suppress<jats:styled-content style=\"fixed-case\">T<\/jats:styled-content>\u2010cell activity, and this resulted in an excellent clinical and biochemical response. Combination therapy with fludarabine and rituximab in<jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>gG4\u2010<jats:styled-content style=\"fixed-case\">RD<\/jats:styled-content>has not previously been reported and can be considered in patients with severe refractory disease.<\/jats:p>","DOI":"10.1111\/ejh.12059","type":"journal-article","created":{"date-parts":[[2012,12,22]],"date-time":"2012-12-22T12:13:49Z","timestamp":1356178429000},"page":"250-256","source":"Crossref","is-referenced-by-count":25,"title":["<scp>I<\/scp>gG4\u2010related disease with hypergammaglobulinemic hyperviscosity and retinopathy"],"prefix":"10.1111","volume":"90","author":[{"given":"Patrick C. 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