IgG4-positive multi-organ lymphoproliferative syndrome manifesting as chronic symmetrical sclerosing dacryo-sialadenitis with subsequent secondary portal hypertension and remarkable IgG4-linked IL-4 elevation

Sir, Immunoglobulin G (IgG) 4-positive multi-organ lymphoproliferative syndrome (IgG4 + MOLPS), also known as IgG4-related systemic disease [1–3] and others, is an emerging clinical entity [2, 3] that exhibits a variety of clinical manifestations, including autoimmune pancreatitis, inflammatory pseudotumour and chronic symmetrical sclerosing dacryo-sialadenitis [CSSD; or Mikulicz disease (MD)] [2–8]. We report a patient initially diagnosed with SS, but subsequently recognized with CSSD-manifesting IgG4 + MOLPS after re-presenting with symptoms of secondary portal hypertension (SPH). A 55-year-old female was admitted in September 2004 with a 2-month history of abdominal distention against a 18-month background of SS satisfying the 2002 Revised American–European Classification Criteria [9], with dryness of the eyes and mouth, bilateral lacrimal and salivary gland enlargement, lymphocyte infiltration on salivary gland biopsy, positive Schirmer’s test and decreased salivary gland function on 99mTc scintigraphy. Abdominal findings confirmed ascites, and lacrimal and salivary glands were enlarged bilaterally. Serum autoantibodies including anti-Ro or SSA and anti-La or SSB were negative and abdominal CT showed multiple abdominal masses in the hepatic hilar and mesenteric regions. Pathological findings are shown in Figure 1A–C.