Case Reports in Rheumatology

Case Reports in Rheumatology / 2012 / Article

Case Report | Open Access

Volume 2012 |Article ID 640353 |

Go Makimoto, Michiko Asano, Nobukazu Fujimoto, Yasuko Fuchimoto, Katsuichiro Ono, Shinji Ozaki, Koji Taguchi, Takumi Kishimoto, "Bilateral Pleural Effusions as an Initial Presentation in Primary Sjögren’s Syndrome", Case Reports in Rheumatology, vol. 2012, Article ID 640353, 3 pages, 2012.

Bilateral Pleural Effusions as an Initial Presentation in Primary Sjögren’s Syndrome

Academic Editor: M. Salazar-Paramo
Received23 Aug 2012
Accepted09 Oct 2012
Published01 Nov 2012


Sjögren’s syndrome (SS) is a systemic autoimmune disease characterized by sicca symptoms. Interstitial pulmonary fibrosis and tracheobronchial sicca are the most common symptoms of pulmonary involvement in primary SjS, and they are rarely accompanied by serositis such as pleuritis or pericarditis. We report a case of SS presenting initially with bilateral pleural effusions. A 63-year old man was admitted to our hospital with a one-month history of cough, dyspnea, and right chest pain. Chest-computed tomography revealed bilateral pleural effusions. Serum anti-SS-A antibody titer was 1 : 256. Ophthalmological examination revealed a positive Schirmer test. Lip biopsy showed atrophy and plasmacytic infiltration of the salivary gland. Corticosteroid treatment was initiated. Pleural effusions were almost completely resolved by day 30. The patient has not experienced any recurrence.

1. Introduction

Sjögren’s syndrome (SS) is a systemic autoimmune disease characterized by sicca symptoms. Pathologically, chronic inflammation is seen in the lacrimal glands and small salivary glands. Interstitial pulmonary fibrosis and tracheobronchial sicca are the most common symptoms of pulmonary involvement in primary SjS, and some cases are also complicated by pulmonary arterial hypertension, pseudolymphoma, pulmonary lymphoma, lymphocytic interstitial pneumonitis, and amyloidosis [1, 2]. However, they are rarely accompanied by serositis such as pleuritis or pericarditis. We report a case of SS presenting initially with bilateral pleural effusions (Table 2).

2. Case Report

A 63-year-old man was admitted to our hospital in December 2011. He had a history of diabetes mellitus, prostate enlargement, and brain infarction. He reported being in his usual state of health until approximately one month earlier, when he developed cough, dyspnea, and right chest pain. He went to a local clinic where computed tomography (CT) of the chest revealed bilateral pleural effusions.

The patient had no fever, rash, joint swelling, or pain. Chest X-ray (Figure 1(a)) and CT (Figure 1(b)) showed bilateral pleural effusions. Laboratory findings on admission were as follows: white blood cells (WBCs) 5700/μL, C-reactive protein (CRP) 9.7 mg/dL, and erythrocyte sedimentation rate (ESR) 100 mm/hr. In addition, the patient was found to be hypothyroid: free thyroxine 4 was 0.59 ng/dL, thyroid stimulating hormone was 48.71 μIU/mL, and antithyroglobulin antibody was over 4000 IU/mL. Pleurocentesis revealed an exudative pleural fluid with no malignant cells but increased proportion of lymphocytes. Protein/albumin concentration and lactase dehydrogenase value in the fluid were 5.6/2.3 g/dL and 315 IU/L, respectively. The smear tests for Mycobacterium tuberculosis and bacterial culture were negative.

Antibiotic therapy (tazobactam/piperacillin 4.5 g × 3/day) was initiated and serum CRP decreased to 2.8 mg/dL on the third hospital day. However, the patient developed high fever on the fifth day. Laboratory evaluation for lupus erythematosus was negative, and rheumatoid factor (RF) was 15 IU/L. Serum antinuclear antibody titer was positive at 1 : 320, anti-SS-A antibody titer was positive at 1 : 256, but anti-SS-B antibody was negative. Other antibodies and immunological profile are shown in Table 1. Pleurocentesis was performed; on pleural fluid analysis, antinuclear antibody titer was 1 : 320 and anti-SS-A antibody titer was 1 : 256. Ophthalmological examination revealed a positive Schirmer test. Lip biopsy showed atrophy of the salivary gland and plasmacytic infiltration around the salivary gland ducts (Figure 2), consistent with SS. Ultimately, we diagnosed the patient with SS due to the presence of sicca symptoms. Corticosteroid treatment (prednisolone 40 mg/day) was initiated and produced a drastic decrease in the pleural effusions. Daily prednisolone dose was gradually reduced from 40 mg to 25 mg over three weeks. Pleural effusion was almost completely resolved by day 30. There is no evidence of recurrence thus far.

ValueNormal valueUnit

Rheumatoid factor150–10IU/L
Antinuclear antibodyX320<40
Anti-ds-DNA antibody10<12IU/mL
Anticardiolipin antibody (IgM)1.2<3.5IU/mL
Anticardiolipin antibody (IgG)8<10IU/mL
Lupus erythematosus testNegative
Lupus anticoagalant1.05<1.3sec
Anti-Sm antibodyNegative
Anti-Sjögren’s syndrome-A antibodyX256
Anti-Sjögren’s syndrome-B antibodyNegative
Proteinase-3 antineutrophil cytoplasmic antibody<10<10EU
Myeloperoxidase antineutrophil cytoplasmic antibody<10<20EU
Serum complement level40.825–48CH50/mL
Soluble interleukin 2 receptor1550145–519IU/mL

AuthorsAgeGenderChief symptomsANAAnti-SS-AAnti-SS-B

Alvarez-Sala et al.64FChest pain+NDND+ND
Ogihara et al.62MFever1 : 40ND1 : 41 : 41 : 81 : 8
Suzuki et al.53FCough1 : 1601 : 80+ND+ND
Kawamata et al.70MCough1 : 1280ND++
Horita et al.73MDyspnea1 : 320ND25.9 U/mL22.3 U/mL59.1 U/mL76.4 U/mL
Teshigawara et al.65MCough, dyspnea1 : 3201 : 80>500 U/mL89.9 U/mL49 U/mL34.3 U/mL

ANA: antinuclear antibody, SS: Sjögren’s syndrome, PE: pleural effusion, ND: not done.

3. Discussion

In 1989 a European research group proposed classification criterion for SS; this was revised by an American-European consensus group in 2002 [9, 10]. It comprises two subjective criteria, ocular and oral symptoms, four objective criteria including ocular and oral signs, and histopathological and serological findings including antinuclear, anti-SS-A, or anti-SS-B antibodies. The diagnosis of SS requires at least four of the six criteria including histopathological or serological finding, or three of the four objective criteria. Our patient had ocular and oral symptoms and a positive Schirmer test. Furthermore, lip biopsy showed atrophy of the salivary gland with plasmacytic infiltration, and positive antinuclear and anti-SS-A antibodies were detected.

SS is classified into two types: primary and secondary, with the secondary form being complicated by other collagen disorders such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and scleroderma. In this case, the patient had no clinical signs of RA or scleroderma. There were some laboratory findings compatible with SLE, but only two criteria of SLE were met: serositis and high titer antinuclear antibody; thus we could not make the diagnosis of SLE. Eventually, a diagnosis of primary SS was reached.

SS is rarely accompanied by pleural effusion. Papathanasiou et al. [11] reported that pleural effusion was observed in no cases of primary SS and in 2 of 26 cases of secondary SS. There have been only six reports of primary SjS complicated by pleural effusion [38] (Table 1). Among them, pleural effusion was an initial manifestation only in two cases [6, 8]. Anti-SS-A and/or SS-B antibody was detected in the pleural effusion of these cases. Physicians should take notice to examine these antibodies in undiagnosed pleural effusion.

Corticosteroid therapy, started at 30 or 40 mg/day of prednisolone if not complicated by interstitial pneumonitis, is a common treatment for SS [4]. A good response is expected, but recurrence is also reported during dose reduction [8]. There has been no evidence of recurrence in our patient so far, but careful followup is warranted.

In conclusion, SS should be considered as one of the collagen diseases potentially presenting with pleuritis.

Conflict of Interests

The authors declare that they have no conflict of interests.


  1. C. V. Strimlan, E. C. Rosenow III, M. B. Divertie, and E. G. Harrison Jr., “Pulmonary manifestations of Sjogren's syndrome,” Chest, vol. 70, no. 3, pp. 354–361, 1976. View at: Google Scholar
  2. S. H. Constantopoulos, C. S. Papadimitriou, and H. M. Moutsopoulos, “Respiratory manifestations in primary Sjogren's syndrome. A clinical, functional, and histologic study,” Chest, vol. 88, no. 2, pp. 226–229, 1985. View at: Google Scholar
  3. R. Alvarez-Sala, P. F. Sanchez-Toril, J. Garcia-Martinez, A. Zaera, and J. F. Masa, “Primary Sjogren syndrome and pleural effusion,” Chest, vol. 96, no. 6, pp. 1440–1441, 1989. View at: Google Scholar
  4. T. Ogihara, A. Nakatani, H. Ito et al., “Sjögren's syndrome with pleural effusion,” Internal Medicine, vol. 34, no. 8, pp. 811–814, 1995. View at: Google Scholar
  5. H. Suzuki, P. Hickling, and C. B. A. Lyons, “A case of primary Sjögren's syndrome, complicated by cryoglobulinaemic glomerulonephritis, pericardial and pleural effusions,” Revmatologiia, vol. 35, no. 1, pp. 72–75, 1996. View at: Google Scholar
  6. K. Kawamata, H. Haraoka, S. Hirohata, T. Hashimoto, R. N. Jenktns, and P. E. Lipsky, “Pleurisy in primary Sjogren's syndrome: T cell receptor β-chain variable region gene bias and local autoantibody production in the pleural effusion,” Clinical and Experimental Rheumatology, vol. 15, no. 2, pp. 193–196, 1997. View at: Google Scholar
  7. Y. Horita, M. Miyazaki, J. I. Kadota et al., “Type II diabetes mellitus and primary Sjögren's syndrome complicated by pleural effusion,” Internal Medicine, vol. 39, no. 11, pp. 979–984, 2000. View at: Google Scholar
  8. K. Teshigawara, S. Kakizaki, M. Horiya et al., “Primary Sjogren's syndrome complicated by bilateral pleural effusion,” Respirology, vol. 13, no. 1, pp. 155–158, 2008. View at: Publisher Site | Google Scholar
  9. C. Vitali, S. Bombardieri, R. Jonsson et al., “Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group,” Annals of the Rheumatic Diseases, vol. 61, no. 6, pp. 554–558, 2002. View at: Publisher Site | Google Scholar
  10. C. Baldini, R. Talarico, A. G. Tzioufas, and S. Bombardieri, “Classification criteria for Sjögren’s syndrome: a critical review,” Journal of Autoimmunity, vol. 39, no. 1-2, pp. 9–14, 2012. View at: Publisher Site | Google Scholar
  11. M. P. Papathanasiou, S. H. Constantopoulos, and C. Tsampoulas, “Reappraisal of respiratory abnormalities in primary and secondary Sjogren's syndrome. A controlled study,” Chest, vol. 90, no. 3, pp. 370–374, 1986. View at: Google Scholar

Copyright © 2012 Go Makimoto et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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