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A6552 - Lymphocytic Interstitial Pneumonitis with Lung-Localised MALT LymphomaPresenting as Infiltrates on Chest X-Ray in an Asymptomatic Patient
Author Block: D. C. Watchorn1, M. O'Callaghan2, A. Fabre2, E. F. McKone2; 1St Vincent's University Hospital, Dublin, Ireland, 2St Vincent's University Hospital, Dublin 4, Ireland.
The case of a 60 year-old female with deteriorating renal indices, infiltrates on chest X-ray and a history of Sjögren’s syndrome is described. The patient had been noted to have deterioriating renal indices over the course of several months. On admission for renal biopsy, the patient underwent routine chest X-ray which demonstrated nodular infiltrates. The patient denied respiratory symptoms and ‘B’ symptoms. She did not have lymphadenopathy or hepatosplenomegaly on examination. Further investigation with CT thorax showed multiple focal nodules and cysts with associated ground-glass attenuation. Imaging and CT-guided biopsy specimens of the nodules were reviewed at a multidisciplinary histopathology conference and consensus favoured a diagnosis of reactive lymphocytic interstitial pneumonitis (LIP). This was felt to represent an LIP variant consistent with her history of Sjögren's disease. The decision was made to follow her closely and, should she show progression of disease, to consider video-assisted thoracoscopic surgical (VATS) biopsy to outrule the possibility of low grade lymphoma.Assessment for progression of disease was to include assessment of her clinical status, physiological (pulmonary function testing, 6 minute walk test) parameters and imaging. Four months subsequently, the patient was increasingly breathlessness on exertion and suffering a non-productive cough. Pulmonary function tests had also deterioriated, now demonstrating restrictive physiology. There was a severe reduction in gas transfer, now 43% predicted. The patient was therefore referred for VATS biopsy. Histopathology reported the specimen as largely consistent with LIP but with some atypical features. The patient was commenced on high dose systemic corticosteroids which she continued for two months with a subsequent slow taper. Additional testing of the biopsy specimen subsequently confirmed a diagnosis of B cell Non Hodgkins Lymphoma, extranodal marginal lymphoma MALT type. These diagnoses have all been reported previously with Sjögren's syndrome. Of note, the earlier renal biopsy findings were in keeping with a membranous pattern of injury secondary to immune complex deposition. Her renal indices stabilised on angiotensin-converting enzyme inhibitor therapy. The patient was referred to haematology and for positron emission tomography-computed tomography imaging which did not show disease elsewhere. 18 months after her initial presentation, the patient deteriorated further symptomatically and physiologically. She was commenced on high dose corticosteroids and four cycles of Rituximab, continuing on maintenance Rituximab for five months subsequently, with good response. The corticosteroids were slowly tapered after one month at high dose. She has since remained off treatment.