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Abstract

Introduction: Lymphoproliferative disorders (LPD) are common in immunosuppressed patients and in patients with connective tissue disorders (CTD). There is increased risk in Sjögren's syndrome (SS) with moderate risk in systemic lupus erythematosis (SLE). Methods: We present a case of 50-year-old African-American female with SLE diagnosed in 1982. She underwent open lung biopsy in 2007 for evaluation of bilateral lower lobe interstitial infiltrates and dyspnea, which showed findings of follicular bronchiolitis. Over the years she was treated with prednisone, methotrexate, mycophenolate mofetil, plaquenil and azathioprine. In December 2012, she presented with dyspnea, chest tightness, low-grade fever, night sweats and productive cough. CT scan of chest showed bilateral patchy ground glass opacities (stable) and new bulky mediastinal, hilar and axillary lymphadenopathy. Sputum was positive for MRSA but negative for AFB and PCP. QuantiFERON-TB Gold test, HIV test, hepatitis B antigen and hepatitis C antibody were negative. Serology for EBV IGM was negative but IgG antibody was positive. ANA was positive. C4 complement level was low. Anti-Ro and Anti-La antibodies were negative. She underwent CT guided FNA of left axillary lymph node, which was inconclusive. She then underwent mediastinoscopy with excisional biopsy of pre-tracheal lymph node which showed atypical lymphoid infiltrate but was non diagnostic of lymphoma. Two months later patient presented with worsening dyspnea and increasing lymphadenopathy on CT chest and abdomen. Excisional biopsy of the left axillary lymph node was performed. Results: The pathology was positive for diffuse large B-cell lymphoma, an aggressive sub-type of non-Hodgkin's lymphoma. The patient was started on chemotherapy with R-CHOP. Conclusions: The continuum of LPD in patients with SLE can vary from follicular bronchiolitis to aggressive lymphoma. Understanding the spectrum of LPD and the various clinical and radiological manifestations in patients with CTD is essential for diagnosis. The time to progression from follicular bronchiolitis to lymphoma is unknown. Patients developing benign LPD like follicular bronchiolitis may have a higher chance of progression to lymphoma. Several mechanisms like chronic immunosuppressive therapy, abnormal immune-regulatory process and autoimmunity has been postulated. CLINICAL IMPLICATIONS: Our case highlights that lymphoma should be high in the differential diagnoses in patients with SLE and lymphadenopathy. The patients who develop benign lymphoproliferative disorder like follicular bronchiolitis should be closely monitored, for their progression to lymphoma.