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.