Renal Lymphoma: Unusual Lymphoproliferative Manifestation of Sjögren’s Syndrome

Abstract

To the Editor: Sjogren’s syndrome (SS) is a chronic autoimmune disease characterized early in its course by lymphocytic infiltration in the salivary and lacrimal glands, resulting in the major manifestations of keratoconjunctivitis sicca and xerostomia. Presumably through a multistep lymphoproliferative process, SS has been associated with the development of non-Hodgkin’s lymphoma (NHL) in 5%–10% of patients1. Mucosa-associated lymphoid tissue (MALT) lymphomas constitute the majority of NHL subtypes seen in SS, followed by nodal marginal zone lymphomas and diffuse large B-cell lymphomas (DLBCL)2. We describe what could be the first case of primary DLBCL of the kidney, a rare extranodal lymphoma, as a lymphoproliferative complication of SS. A 64-year-old woman presented at age 39 with polyarthralgias and morning stiffness attributed to rheumatoid arthritis (RA). She was treated with hydroxychloroquine and nonsteroidal agents. She subsequently developed dry eyes, dry mouth, difficulty swallowing without drinking fluids, and vaginal and nasal dryness, which she treated with topical moisturizing agents. At age 58, she was noted to have mild pancytopenia with leukocytes 3600/mm3, hemoglobin 11.3 g/dl, and platelets 95,000/mm3. Bone marrow examination revealed erythroid hyperplasia, megakaryocytic hyperplasia, and atypical lymphocytosis. Urinalysis showed 20–30 white blood cells/high-powered field (HPF), without red cells, bacteria, nitrite, or proteinuria. Serological testing revealed antinuclear antibody (ANA) 1:640 speckled pattern; absence of antibodies to dsDNA and Sm/RNP; anti-SSA antibody 413 U (strongly positive); anti-SSB 129 U (weakly positive); rheumatoid factor (RF) 32 IU (normal … Address correspondence to Dr. E.D. Rosenstein, Institute for Rheumatic and Autoimmune Diseases, Overlook Medical Center, 33 Overlook Road, Summit, New Jersey 07901, USA., E-mail: elliot.rosenstein{at}atlantichealth.org