title: Primary Sjögren's syndrome presenting as a case of sarcoidosis and a suspected pancreatic tumour.
abstract: A 45 year old man, previously diagnosed as having sarcoid, presented with signs and symptoms of a pancreatic malignancy. An explorative laparotomy, however, showed only chronic pancreatitis. He was found to have a raised erythrocyte sedimentation rate, normocytic normochromic anaemia, renal insufficiency, hypergammaglobulinaemia, and a strongly positive rheumatoid factor and antinuclear antibody titre. Bilateral hilar lymph node enlargement was noted on chest x ray. Subsequently, the patient complained of xerostomia and keratoconjunctivitis sicca. Large lymphocytic infiltrates and a shift in the relative number of IgA bearing plasma cells in favour of IgG and IgM bearing plasma cells were seen in tissue obtained by sublabial salivary gland biopsy. A transbronchial lung biopsy and review of the biopsies of the pancreas, the lung, liver, and a lymph node all failed to show granulomatous disease. These findings strongly suggested a diagnosis of Sjögren's syndrome instead of sarcoidosis. This case shows the difficulty sometimes encountered in differentiating between sarcoid and systemic Sjögren's syndrome, and the value of a sublabial salivary gland biopsy.
authors.affiliation: Department of Medicine, University Hospital, Nijmegen, The Netherlands.
authors.forename: P P
authors.initials: PP
authors.lastname: Koopmans
authors.forename: C
authors.initials: C
authors.lastname: Bodeutsch
authors.forename: P C
authors.initials: PC
authors.lastname: de Wilde
authors.forename: A M
authors.initials: AM
authors.lastname: Boerbooms
mesh_terms: D002908:Chronic Disease; D003937:Diagnosis, Differential; D006801:Humans; D008168:Lung / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000175:diagnosis*; D010195:Pancreatitis / Q000175:diagnosis; D012469:Salivary Glands / Q000473:pathology; D012507:Sarcoidosis / Q000175:diagnosis*; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000473:pathology