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Annals ofthe Rheumatic Diseases 1990; 49: 407-409 407

Primary Sjogren's syndrome presenting as a case of Ann Rheum Dis: first published as 10.1136/ard.49.6.407 on 1 June 1990. Downloaded from and a suspected pancreatic tumour

P P Koopmans, C Bodeutsch, P C M de Wilde, A M Th Boerbooms

Abstract examination of the pancreas showed onlfy exten- A 45 year old man, previously diagnosed as sive fibrosis and a diffuse mononuclear infiltrate, having sarcoid, presented with signs and compatible with a diagnosis of chronic pan- symptoms of a pancreatic malignancy. An creatitis. There were no signs of a malignancy in explorative laparotomy, however, showed only the pancreas or the associated . chronic pancreatitis. He was found to have a The patient complained of extreme tiredness, raised erythrocyte sedimentation rate, normo- a dry mouth, and dysphagia and difficulty in cytic normochromic anaemia, renal insuf- chewing. The submandibulary salivary glands ficiency, hypergammaglobulinaemia, and a seemed to be enlarged, and six months strongly positive and anti- previously he had consulted an ophthalmologist nuclear antibody titre. Bilateral hilar lymph for dry eyes. There was no , anorexia, node enlargement was noted on chest x ray. or . He had had Raynaud's pheno- Subsequently, the patient complained of xero- menon stomia and keratoconjunctivitis sicca. Large One year previously he had attended his local lymphocytic infiltrates and a shift in the hospital because of tiredness. An x ray of the relative number of IgA bearing plasma cells in chest had shown bilateral hilar lymph node favour of IgG and IgM bearing plasma cells enlargement and as sarcoidosis was suspected a were seen in obtained by sublabial transbronchial had been performed. salivary gland biopsy. A transbronchial lung Microscopic examination of these biopsy speci- biopsy and review of the of the mens had shown epitheloid cells and a few pancreas, the lung, liver, and a lymph node all granulomatous structures. Therefore it was failed to show granulomatous disease. These concluded that the patient had sarcoidosis. A findings strongly suggested a diagnosis of few months later he again attended his local Sjogren's syndrome instead of sarcoidosis. hospital because of pain in the right upper This case shows the difficulty sometimes abdominal quadrant. Biochemical examination encountered in differentiating between showed raised values for http://ard.bmj.com/ sarcoid and systemic Sjogren's syndrome, and 390 U/l (normal <125), y-glutamyltransferase the value of a sublabial salivary gland biopsy. 519 U/l (normal <30), S-alanine aminotrans- ferase (91 U/1) (normal <25), and S-aspartate aminotransferase 262 U/l (normal <25). A liver Primary Sjogren s syndrome is a chronic inflam- biopsy specimen showed small infiltrates of matory autoimmune disorder, which may granulocytes and lymphocytes but no evidence present as the sicca complex only, but may also of granulomatous disease. An endoscopic retro- on September 28, 2021 by guest. Protected copyright. affect many organs. Sarcoidosis can mimic the grade cholangiopancreatography was performed, clinical symptoms of Sjogren's syndrome and showing narrowing of the pancreatic and the between these disorders common bile duct, raising suspicions of a clinically and histologically may be difficult.' tumour of the pancreas. Computed tomography Recently, Melsom et al reported a patient showed an enlarged pancreatic head. At this Department of Medicine, presenting with clinical and histological features point the patient was referred for explorative Division of General of Sjogren's syndrome, who seemed to have laparotomy. Internal Medicine, sarcoidosis.2 at the of University Hospital Physical examination department Nijmegen, PO Box 9101, We present a patient with sarcoidosis, who general internal medicine showed a patient who The Netherlands underwent an exploratory laparotomy for a looked anaemic with slightly enlarged, tender P P Koopmans suspected pancreatic malignancy. Microscopic submandibulary glands, and a dry oral mucosa; Department of examination of biopsy specimens of the lung, otherwise the findings were unremarkable. Pathologic Anatomy, liver, pancreas, and sublabial salivary gland University Hospital There was no , , or Nijmegen, PO Box 9101, showed no formation. The findings lymph node enlargement. The Netherlands in the sublabial salivary gland strongly supported Laboratory investigations showed raised C Bodeutsch a diagnosis of Siogren's syndrome. erythrocyte sedimentation rate (ESR) (112 P C M de Wilde mm/h), a normochromic, normocytic anaemia Division of , with a haemoglobin 106 g/l (normal 130-160). University Hospital Case report The other haematological indices were normal. Nijmegen, PO Box 9101, In July 1986 a 45 year old man was referred to The gammaglobulins were 25 g/l (normal 7-15). The Netherlands internal medicine by A M Th Boerbooms the department of general Serum immunoelectrophoresis showed a hyper- the department of surgery. He had undergone a gammaglobulinaemia and no paraprotein. No Correspondence to: Dr Koopmans. diagnostic laparatomy because of a suspected cryoglobulins were found. Serological tests for Accepted for publication pancreatic malignancy. An enlargement of the rheumatoid factor, the antinuclear antibody 4 August 1989 pancreatic head was found. Microscopic test, and circulating immune complexes were 408 Koopmans, Bodeutsch, de Wilde, Boerbooms

strongly positive. SS-A and SS-B were not detected. Serum was slightly raised

(166 ,umol/1) (normal 60-100) and there was a Ann Rheum Dis: first published as 10.1136/ard.49.6.407 on 1 June 1990. Downloaded from of 0-82 g/l. Tests for liver function, serum amylase, and calcium were normal. Angiotensin converting enzyme concentration was increased 20-2 mE/ml (normal 12-15). A Schirmer test (right 6 mm, left 8 mm) showed diminished tear production. Rose bengal stain- ing of the conjunctiva was positive. Routine chest x rays showed diffuse fibrotic lesions and bilateral hilar enlargement, the latter confirmed by tomography. Submandibulary gland sialo- graphy was performed, showing diffuse narrow- ing and widening of the ducts, suggesting t 4t;%.J 6'If~~~~~~~~~f chronic . As clinically the differential diagnosis lay between sarcoidosis or Sjogren's syndrome, fibreoptic and a sublabial salivary gland biopsy were carried out. Fibrosis and an Figure 1: Part ofthe second lung biopsy specimen withfibrosis and some mononuclear inflammatory cells. No evidence ofnon-caseating granulomatous process. (Haematoxylin and infiltration of histiocytes and lymphocytes was eosin.) found in the lung biopsy specimen (fig 1) but no granulomata, multinucleate giant cells, or epithelioid cells were seen. Microscopy of the sublabial salivary gland biopsy specimen (fig 2) showed large focal lymphocytic infiltrations (focus score 215 with confluent foci) and ductal hyperplasia. No granulomata or multinucleate giant cells were found. Immunohistochemical A~~~ ~ ~ staining showed a polyclonal plasmacytic infil- trate and a shift in the relative number of IgA bearing plasma cells in favour of IgG and IgM :~~~~~~~~~~bearing plasma cells. The plasmacytic infiltrate consisted of 40 1% IgA bearing cells (normal (SD) 919 (8 6)%, 47-1% IgG bearing cells Ij-ft (normal 3-4 (4 8)%), and 12-8% IgM bearing di plasma cells (normal 4-8 (4-8)%). Both the .4W findings http://ard.bmj.com/ - histological and immunohistochemical JOr".-I in the sublabial salivary gland biopsy specimen were strongly suggestive of Sjogren's syndrome. When revision of the early lung biopsy was 4'r performed a non-specific fibrosing inflammatory process was found. Some structures were sug- gestive of sarcoidosis, but no granulomata, Figure 2: Sublabial salivary gland biopsy specimen with large confluent lymphocytic foci and epitheloid cells, or multinucleate giant cells on September 28, 2021 by guest. Protected copyright. destruction ofparenchyma. (Haematoxylin and eosin.) could be shown (fig 3).

Discussion Primary Sjogren's syndrome is a chronic 4S A, - inflammatory disorder characterised by i'45D lymphoid cell infitration and destruction of salivary and lachrymal glands, resulting in keratoconjunctivitis sicca and . Sarcoidosis may present with keratoconjuncti- vitis and sialoadenitis resembling Sjogren's syn- drome.4 In addition, the pulmonary manifesta- tions of Sjogren's syndrome may resemble those of sarcoidosis.5 6 Bilateral hilar lymph node enlargement strongly suggested sarcoidosis in our patient, and this diagnosis was supported by the general symptoms and the raised angiotensin converting enzyme concentration in plasma. The clinical diagnosis of primary Sjogren's syndrome was supported by the typical ocular and salivary symptoms, the sialoadenitis seen on sialography, and the biochemical indices, in- cluding raised ESR, gammaglobulin value, and Figure 3: Part ofthefirst lung biopsy specimen withfibrosis and diffuse infiltration with mononuclear inflammatory cells. No evidence ofnon-caseatinggranulomatous process. the positive rheumatoid factor and antinuclear (Haematoxylin and eosin.) antibody test.7 Antibodies to SS-A and SS-B Primary Sjogren's syndrome presenting aspancreatic malignancy 409

were negative, but although often found in ably) the kidney, which may occur in primary Sjogren's syndrome, they are not always pre- Sjogren's syndrome; (b) how difficult differen- sent.8 Furthermore, the patient had chronic tial diagnosis from sarcoidosis can be; and (c) Ann Rheum Dis: first published as 10.1136/ard.49.6.407 on 1 June 1990. Downloaded from pancreatitis, proteinuria, and raised serum that a sublabial salivary gland biopsy may be creatinine, which have been reported in primary helpful for the diagnosis. Sjogren's syndrome.7 91o Thus as none of these findings is specific a diagnosis on clinical grounds was not possible. 1 De Wilde P C M, Slootweg P J, Hene R J, Baak J P A, Kater L. Multinucleate giant cells in sublabial salivary gland tissue Therefore, a sublabial salivary gland biopsy in Siogren's syndrome. A diagnostic pitfall. Virchows Arch and a second lung biopsy were carried out to [A] 1984; 403: 247-56. 2 Melsom R D, Speight P M, Ryan J, Perry J D. Sarcoidosis in differentiate between sarcoidosis and Sjogren's a patient presenting with clinical and histological features of syndrome. Furthermore, when the previous primary Siogren's syndrome. Ann Rheum Dis 1988; 47: 166-8. transbronchial lung biopsy specimen was 3 De Wilde P C M, Kater L, Baak J P A, Van Houwelingen reviewed, no definite granulomata, multi- J C, Hene R J, Slootweg P J. A new and highly sensitive immunohistological diagnostic criterion for Siogren's syn- nucleate giant cells, or epitheloid cells could be drome. Rheum (in press). shown, but fibrosis and infiltrations of histio- 4 Nessan V J, Jacoway J R. Biopsy of minor salivary gland biopsy in the diagnosis of sarcoidosis. N Engl J Med 1979; cytes and lymphocytes were present. The first 301: 922-4. biopsy specimen did, however, show some 5 Strimlan V C, Rosenow E C, Divertie M B, Harrison E G Jr. Pulmonary manifestations of Sjogren's syndrome. Chest structures faintly resembling granulomata such 1976; 70: 354-61. as may be found in sarcoidosis. 6 Constantopoulos S H, Papadimitriou C S, Moutsopoulos H M. Respiratory manifestations in primary Sjogren's The histological and immunohistological syndrome. Chest 1985; 88: 226-9. findings in the sublabial salivary gland biopsy 7 Talal N, Moutsopoulos H M, Kassan S S. Sjogren's syndrome. Berlin, Heidelberg: Springer, 1987. specimen strongly indicated Sjogren's syn- 8 Smeenk R, Westgeest T, Swaak T. Antinuclear antibody drome.' 1 1 The absence of granulomata was determination: the present state of diagnostic and clinical relevance. ScandJa Rheumatol [Suppl] 1985; 56: S78-92. also against a diagnosis of sarcoidosis. 9 Montefusco P P, Geiss A C, Bronzo R L, Randall S, Kahn Non-specific infiltrations by mononuclear E, McKinley M J. Sclerosing cholangitis, chronic pan- cells and fibrosis in the biopsy specimens of the creatitis, and Siogren's syndrome: a syndrome complex. Am J Surg 1984; 147: 822-6. liver, lymph node, and pancreas as seen in this 10 Versapuech J M, Labayle D, Grange D, Fischer D, Kemeny case have been often in F, D'Hubert E. Cholangite sclerosante, pancreatite chroni- reported primary que et syndrome de Sjogren. Ann Med Interne (Paris) 1986; Sj6gren's syndrome,7 and as no granulomata 137: 147-54. could be shown in the 11 Lane H C, Callihan T R, Jaffe E S, Fauci A S, Moutsopoulos these biopsy specimens H M. Presence of intra-cytoplasmic IgG in the lymphocytic diagnosis of primary Sj6gren's syndrome was infiltrates of the minor salivary glands of patients with primary Sjogren's syndrome. Clin Exp Rheumatol 1983; 1: supported indirectly. 237-9. In conclusion, this case report shows (a) the 12 Greenspan J S, Daniels T E, Talal N, Sylvester R A. The histopathology of Siogren's syndrome in labial salivary severe systemic manifestations, including gland biopsies. Oral Surg Oral Med Oral Pathol 1974; 37: involvement of the lung, pancreas, and (prob- 217-29. http://ard.bmj.com/ on September 28, 2021 by guest. Protected copyright.