Sjögren's Syndrome

ABSTRACT

Sjögren's syndrome (SS) is a chronic, slowly progressive, inflammatory, autoimmune disease characterized by (1) lymphocytic infiltration of the exocrine glands leading to diminished or absent glandular secretion, and (2) marked B-lymphocytic cell hyperreactivity manifested initially by a variety of serum autoantibodies, including those against the Ro(SSA) and La(SSB) ribonucleoproteins, ending in the development of B cell non-Hodgkin's lymphoma in a substantial number of patients. Most patients with SS present only with keratoconjunctivitis sicca and xerostomia. However, ~40% develop extraglandular manifestations that present in two ways: (1) the development of lymphoepithelial lesions in several extra-exocrine gland tissues (i.e., bronchi, renal tubules, or biliary ducts), and (2) vasculitis related to the deposition of immune complexes due to B cell hyperreactivity. Pulmonary manifestations develop in some patients and may present as (1) bronchitis sicca; (2) a wide spectrum of lymphoproliferative diseases, ranging from bronchus-associated lymphoid tissue (BALT) hyperplasia, lymphoid interstitial pneumonia, and B cell non-Hodgkin's lymphoma mainly of the extranodal marginal zone B-cell lymphoma of BALT-type or rarely of higher-grade malignancy; and (3) other interstitial pneumonias. Pleuritis can be seen in SS patients with associated systemic lupus erythematosus or rheumatoid arthritis.

Lymphoepithelial lesions - bronchus-associated lymphoid tissue hyperplasia - lymphoid interstitial pneumonia - marginal zone B cell lymphoma - nonspecific interstitial pneumonia

REFERENCES

• 1 Moutsopoulos H. Sjögren's syndrome. In: Braunwald E, Kasper DL, Hauser SL, Longo DL, Jameson JL Harrison's Principles of Internal Medicine. 14th ed. New York; McGraw-Hill 2004: 1990-1993
• 2 Moutsopoulos H M. Sjögren's syndrome: autoimmune epithelitis.  Clin Immunol Immunopathol. 1994;  72 162-165
• 3 Moutsopoulos H M, Chused T M, Mann D L et al.. Sjögren's syndrome (Sicca syndrome): current issues.  Ann Intern Med. 1980;  92 212-226
• 4 Talal N, Moutsopoulos H, Kassan S S. Sjögren's Syndrome: Clinical and Immunological Aspects. Heidelberg; Springer-Verlag 1987
• 5 Kassan S S, Thomas T L, Moutsopoulos H M et al.. Increased risk of lymphoma in sicca syndrome.  Ann Intern Med. 1978;  89 888-892
• 6 Royer B, Cazals-Hatem D, Sibilia J et al.. Lymphomas in patients with Sjögren's syndrome are marginal zone B-cell neoplasms, arise in diverse extranodal and nodal sites, and are not associated with viruses.  Blood. 1997;  90 766-775
• 7 Talal N, Bunim J J. The development of malignant lymphoma in the course of Sjoegren's syndrome.  Am J Med. 1964;  36 529-540
• 8 Voulgarelis M, Dafni U G, Isenberg D A, Moutsopoulos H M. Malignant lymphoma in primary Sjögren's syndrome: a multicenter, retrospective, clinical study by the European Concerted Action on Sjögren's Syndrome.  Arthritis Rheum. 1999;  42 1765-1772
• 9 Skopouli F N, Moutsopoulos H M. Autoimmune epithelitis: Sjögren's syndrome.  Clin Exp Rheumatol. 1994;  12 (suppl 11) S9-S11
• 10 Cairns D, Shelley L, Burke W M et al.. The differing patterns of interstitial lung involvement in connective tissue diseases.  J Rheumatol. 1992;  19 1089-1095
• 11 Kelly C, Gardiner P, Pal B, Griffiths I. Lung function in primary Sjögren's syndrome: a cross sectional and longitudinal study.  Thorax. 1991;  46 180-183
• 12 Papiris S A, Kassan S S, Moutsopoulos H M. Lung involvement in primary Sjögren's syndrome.  Semin Resp Crit Care Med. 1999;  20 179-184
• 13 Vitali C, Bombardieri S, Jonsson R et al.. Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group.  Ann Rheum Dis. 2002;  61 554-558
• 14 Manoussakis M N, Moutsopoulos H M. Sjögren's syndrome: autoimmune epithelitis.  Baillieres Best Pract Res Clin Rheumatol. 2000;  14 73-95
• 15 Sjögren H. Zur Kenntnis de Keraconjunctivitis sicca (Keratitis filiformis bei Hypofunction der Tranendrusen).  Acta Opthalmologica. 1933;  2 1
• 16 Cha S I, Fessler M B, Cool C D, Schwarz M I, Brown K K. Lymphoid interstitial pneumonia: clinical features, associations and prognosis.  Eur Respir J. 2006;  28 364-369
• 17 Masaki Y, Sugai S. Lymphoproliferative disorders in Sjögren's syndrome.  Autoimmun Rev. 2004;  3 175-182
• 18 Papiris S A, Kalomenidis I, Malagari K et al.. Extranodal marginal zone B-cell lymphoma of the lung in Sjögren's syndrome patients: reappraisal of clinical, radiological, and pathology findings.  Respir Med. 2007;  101 84-92
• 19 Ito I, Nagai S, Kitaichi M et al.. Pulmonary manifestations of primary Sjögren's syndrome: a clinical, radiologic, and pathologic study.  Am J Respir Crit Care Med. 2005;  171 632-638
• 20 Parambil J G, Myers J L, Lindell R M, Matteson E L, Ryu J H. Interstitial lung disease in primary Sjögren syndrome.  Chest. 2006;  130 1489-1495
• 21 Cain H C, Noble P W, Matthay R A. Pulmonary manifestations of Sjögren's syndrome.  Clin Chest Med. 1998;  19 687-699 , viii
• 22 Constantopoulos S H, Papadimitriou C S, Moutsopoulos H M. Respiratory manifestations in primary Sjögren's syndrome: a clinical, functional, and histologic study.  Chest. 1985;  88 226-229
• 23 Davidson B K, Kelly C A, Griffiths I D. Ten-year follow-up of pulmonary function in patients with primary Sjögren's syndrome.  Ann Rheum Dis. 2000;  59 709-712
• 24 Hunninghake G W, Fauci A S. Pulmonary involvement in the collagen vascular diseases.  Am Rev Respir Dis. 1979;  119 471-503
• 25 Papathanasiou M P, Constantopoulos S H, Tsampoulas C, Drosos A A, Moutsopoulos H M. Reappraisal of respiratory abnormalities in primary and secondary Sjögren's syndrome: a controlled study.  Chest. 1986;  90 370-374
• 26 Segal I, Fink G, Machtey I, Gura V, Spitzer S A. Pulmonary function abnormalities in Sjögren's syndrome and the sicca complex.  Thorax. 1981;  36 286-289
• 27 Gardiner P. Primary Sjögren's syndrome.  Baillieres Clin Rheumatol. 1993;  7 59-77
• 28 Mialon P, Barthelemy L, Sebert P et al.. A longitudinal study of lung impairment in patients with primary Sjögren's syndrome.  Clin Exp Rheumatol. 1997;  15 349-354
• 29 Franquet T, Gimenez A, Monill J M, Diaz C, Geli C. Primary Sjögren's syndrome and associated lung disease: CT findings in 50 patients.  AJR Am J Roentgenol. 1997;  169 655-658
• 30 Uffmann M, Kiener H P, Bankier A A et al.. Lung manifestation in asymptomatic patients with primary Sjögren syndrome: assessment with high resolution CT and pulmonary function tests.  J Thorac Imaging. 2001;  16 282-289
• 31 Dalavanga Y A, Constantopoulos S H, Galanopoulou V, Zerva L, Moutsopoulos H M. Alveolitis correlates with clinical pulmonary involvement in primary Sjögren's syndrome.  Chest. 1991;  99 1394-1397
• 32 Hatron P Y, Wallaert B, Gosset D et al.. Subclinical lung inflammation in primary Sjögren's syndrome: relationship between bronchoalveolar lavage cellular analysis findings and characteristics of the disease.  Arthritis Rheum. 1987;  30 1226-1231
• 33 Adamson III T C, Fox R I, Frisman D M, Howell F V. Immunohistologic analysis of lymphoid infiltrates in primary Sjögren's syndrome using monoclonal antibodies.  J Immunol. 1983;  130 203-208
• 34 Brookes S M, Price E J, Venables P J, Maini R N. Interferon-gamma and epithelial cell activation in Sjögren's syndrome.  Br J Rheumatol. 1995;  34 226-231
• 35 Fox R I, Theofilopoulos A N, Altman A. Production of interleukin 2 (IL 2) by salivary gland lymphocytes in Sjögren's syndrome: detection of reactive cells by using antibody directed to synthetic peptides of IL 2.  J Immunol. 1985;  135 3109-3115
• 36 Moutsopoulos H M, Hooks J J, Chan C C et al.. HLA-DR expression by labial minor salivary gland tissues in Sjögren's syndrome.  Ann Rheum Dis. 1986;  45 677-683
• 37 Papiris S A, Saetta M, Turato G et al.. CD4-positive T-lymphocytes infiltrate the bronchial mucosa of patients with Sjögren's syndrome.  Am J Respir Crit Care Med. 1997;  156 637-641
• 38 Xanthou G, Polihronis M, Tzioufas A G et al.. “Lymphoid” chemokine messenger RNA expression by epithelial cells in the chronic inflammatory lesion of the salivary glands of Sjögren's syndrome patients: possible participation in lymphoid structure formation.  Arthritis Rheum. 2001;  44 408-418
• 39 Xanthou G, Tapinos N I, Polihronis M et al.. CD4 cytotoxic and dendritic cells in the immunopathologic lesion of Sjögren's syndrome.  Clin Exp Immunol. 1999;  118 154-163
• 40 Manoussakis M N. Immunological and clinical predictors for lymphoma in Sjögren's syndrome [abstract SP0013].  Ann Rheum Dis. 2006;  65(Suppl 2) 6
• 41 Moutsopoulos H M, Tzioufas A G, Bai M K, Papadopoulos N M, Papadimitriou C S. Association of serum IgM kappa monoclonicity in patients with Sjögren's syndrome with an increased proportion of kappa positive plasma cells infiltrating the labial minor salivary glands.  Ann Rheum Dis. 1990;  49 929-931
• 42 Tengner P, Halse A K, Haga H J, Jonsson R, Wahren-Herlenius M. Detection of anti-Ro/SSA and anti-La/SSB autoantibody-producing cells in salivary glands from patients with Sjögren's syndrome.  Arthritis Rheum. 1998;  41 2238-2248
• 43 Casciola-Rosen L A, Anhalt G, Rosen A. Autoantigens targeted in systemic lupus erythematosus are clustered in two populations of surface structures on apoptotic keratinocytes.  J Exp Med. 1994;  179 1317-1330
• 44 Kapsogeorgou E K, Abu-Helu R F, Moutsopoulos H M, Manoussakis M N. Salivary gland epithelial cell exosomes: a source of autoantigenic ribonucleoproteins.  Arthritis Rheum. 2005;  52 1517-1521
• 45 Skopouli F N, Kousvelari E E, Mertz P et al.. c-myc mRNA expression in minor salivary glands of patients with Sjögren's syndrome.  J Rheumatol. 1992;  19 693-699
• 46 Tapinos N I, Polihronis M, Moutsopoulos H M. Lymphoma development in Sjögren's syndrome: novel p53 mutations.  Arthritis Rheum. 1999;  42 1466-1472
• 47 de Wilde P C, Kater L, Bodeutsch C et al.. Aberrant expression pattern of the SS-B/La antigen in the labial salivary glands of patients with Sjögren's syndrome.  Arthritis Rheum. 1996;  39 783-791
• 48 Yannopoulos D I, Roncin S, Lamour A et al.. Conjunctival epithelial cells from patients with Sjögren's syndrome inappropriately express major histocompatibility complex molecules, La(SSB) antigen, and heat-shock proteins.  J Clin Immunol. 1992;  12 259-265
• 49 Kapsogeorgou E K, Dimitriou I D, Abu-Helu R F, Moutsopoulos H M, Manoussakis M N. Activation of epithelial and myoepithelial cells in the salivary glands of patients with Sjögren's syndrome: high expression of intercellular adhesion molecule-1 (ICAM.1) in biopsy specimens and cultured cells.  Clin Exp Immunol. 2001;  124 126-133
• 50 Boumba D, Skopouli F N, Moutsopoulos H M. Cytokine mRNA expression in the labial salivary gland tissues from patients with primary Sjögren's syndrome.  Br J Rheumatol. 1995;  34 326-333
• 51 Fox R I, Bumol T, Fantozzi R, Bone R, Schreiber R. Expression of histocompatibility antigen HLA-DR by salivary gland epithelial cells in Sjögren's syndrome.  Arthritis Rheum. 1986;  29 1105-1111
• 52 Lindahl G, Hedfors E, Klareskog L, Forsum U. Epithelial HLA-DR expression and T lymphocyte subsets in salivary glands in Sjögren's syndrome.  Clin Exp Immunol. 1985;  61 475-482
• 53 Kapsogeorgou E K, Moutsopoulos H M, Manoussakis M N. Functional expression of a costimulatory B7.2 (CD86) protein on human salivary gland epithelial cells that interacts with the CD28 receptor, but has reduced binding to CTLA4.  J Immunol. 2001;  166 3107-3113
• 54 Manoussakis M N, Dimitriou I D, Kapsogeorgou E K et al.. Expression of B7 costimulatory molecules by salivary gland epithelial cells in patients with Sjögren's syndrome.  Arthritis Rheum. 1999;  42 229-239
• 55 Dimitriou I D, Kapsogeorgou E K, Moutsopoulos H M, Manoussakis M N. CD40 on salivary gland epithelial cells: high constitutive expression by cultured cells from Sjögren's syndrome patients indicating their intrinsic activation.  Clin Exp Immunol. 2002;  127 386-392
• 56 Spachidou M P, Bourazopoulou E, Kapsogeorgou E K et al.. Functional Toll-like receptors (TLRs) are expressed by cultured salivary gland epithelial cells: increased mRNA expression of TLR -1, -2 and -4 in cells derived from patients with primary Sjögren's syndrome [abstract 684].  Arthritis Rheum. 2005;  52 278
• 57 Triantafyllopoulou A, Tapinos N, Moutsopoulos H M. Evidence for coxsackievirus infection in primary Sjögren's syndrome.  Arthritis Rheum. 2004;  50 2897-2902
• 58 Harris S R, Mark E J. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises: case 17-2001: a 42-year-old man with multiple pulmonary cysts and recurrent respiratory infections.  N Engl J Med. 2001;  344 1701-1708
• 59 Constantopoulos S H, Drosos A A, Maddison P J, Moutsopoulos H M. Xerotrachea and interstitial lung disease in primary Sjögren's syndrome.  Respiration. 1984;  46 310-314
• 60 Ellman P, Parkes Weber F. Sjögren's disease with dryness of the bronchial mucosa and uncertain lung lesion.  BMJ. 1949;  1 304-305
• 61 Ellman P, Parkes Weber F, Goddier T EW. A contribution to the pathology of Sjögren's syndrome.  Q J Med. 1951;  77 33-43
• 62 Andoh Y, Shimura S, Sawai T et al.. Morphometric analysis of airways in Sjögren's syndrome.  Am Rev Respir Dis. 1993;  148 1358-1362
• 63 Strimlan C V, Rosenow III E C, Divertie M B, Harrison Jr E G. Pulmonary manifestations of Sjögren's syndrome.  Chest. 1976;  70 354-361
• 64 Newball H H, Brahim S A. Chronic obstructive airway disease in patients with Sjögren's syndrome.  Am Rev Respir Dis. 1977;  115 295-304
• 65 Oxholm P, Bundgaard A, Birk Madsen E, Manthorpe R, Vejlo Rasmussen F. Pulmonary function in patients with primary Sjögren's syndrome.  Rheumatol Int. 1982;  2 179-181
• 66 Bariffi F, Pesci A, Bertorelli G, Manganelli P, Ambanelli U. Pulmonary involvement in Sjögren's syndrome.  Respiration. 1984;  46 82-87
• 67 Potena A, La Corte R, Fabbri L M et al.. Increased bronchial responsiveness in primary and secondary Sjögren's syndrome.  Eur Respir J. 1990;  3 548-553
• 68 Gudbjornsson B, Hedenstrom H, Stalenheim G, Hallgren R. Bronchial hyperresponsiveness to methacholine in patients with primary Sjögren's syndrome.  Ann Rheum Dis. 1991;  50 36-40
• 69 Gardiner P, Ward C, Allison A et al.. Pleuropulmonary abnormalities in primary Sjögren's syndrome.  J Rheumatol. 1993;  20 831-837
• 70 Lahdensuo A, Korpela M. Pulmonary findings in patients with primary Sjögren's syndrome.  Chest. 1995;  108 316-319
• 71 Salaffi F, Manganelli P, Carotti M et al.. A longitudinal study of pulmonary involvement in primary Sjögren's syndrome: relationship between alveolitis and subsequent lung changes on high-resolution computed tomography.  Br J Rheumatol. 1998;  37 263-269
• 72 Franquet T, Diaz C, Domingo P, Gimenez A, Geli C. Air trapping in primary Sjögren syndrome: correlation of expiratory CT with pulmonary function tests.  J Comput Assist Tomogr. 1999;  23 169-173
• 73 Ludviksdottir D, Janson C, Hogman M et al.. Increased nitric oxide in expired air in patients with Sjögren's syndrome. BHR study group: bronchial hyperresponsiveness.  Eur Respir J. 1999;  13 739-743
• 74 Papiris S A, Maniati M, Constantopoulos S H et al.. Lung involvement in primary Sjögren's syndrome is mainly related to the small airway disease.  Ann Rheum Dis. 1999;  58 61-64
• 75 Taouli B, Brauner M W, Mourey I, Lemouchi D, Grenier P A. Thin-section chest CT findings of primary Sjögren's syndrome: correlation with pulmonary function.  Eur Radiol. 2002;  12 1504-1511
• 76 Pertovaara M, Korpela M, Saarelainen S et al.. Long-term follow-up study of pulmonary findings in patients with primary Sjögren's syndrome.  Scand J Rheumatol. 2004;  33 343-348
• 77 Dalavanga Y A, Voulgari P V, Georgiadis A N et al.. Lymphocytic alveolitis: a surprising index of poor prognosis in patients with primary Sjögren's syndrome.  Rheumatol Int. 2006;  26 799-804
• 78 Whaley K, Webb J, McAvoy B A et al.. Sjögren's syndrome, II: Clinical associations and immunological phenomena.  Q J Med. 1973;  42 513-548
• 79 Silbiger M L, Peterson Jr C C. Sjögren's syndrome: its roentgenographic features.  Am J Roentgenol Radium Ther Nucl Med. 1967;  100 554-558
• 80 Meyer C A, Pina J S, Taillon D, Godwin J D. Inspiratory and expiratory high-resolution CT findings in a patient with Sjögren's syndrome and cystic lung disease.  AJR Am J Roentgenol. 1997;  168 101-103
• 81 Johkoh T, Muller N L, Pickford H A et al.. Lymphocytic interstitial pneumonia: thin-section CT findings in 22 patients.  Radiology. 1999;  212 567-572
• 82 Sutinen S, Sutinen S, Huhti E. Ultrastructure of lymphoid interstitial pneumonia: virus-like particles in bronchiolar epithelium of a patient with Sjögren's syndrome.  Am J Clin Pathol. 1977;  67 328-333
• 83 Yousem S A, Colby T V, Carrington C B. Follicular bronchitis/bronchiolitis.  Hum Pathol. 1985;  16 700-706
• 84 Colby T V. Pulmonary pathology in patients with systemic autoimmune diseases.  Clin Chest Med. 1998;  19 587-612 , vii
• 85 Zulman J, Jaffe R, Talal N. Evidence that the malignant lymphoma of Sjögren's syndrome is a monoclonal B-cell neoplasm.  N Engl J Med. 1978;  299 1215-1220
• 86 Colby T V, Carrington C B. Lymphoreticular tumors and infiltrates of the lung.  Pathol Annu. 1983;  18(Pt 1) 27-70
• 87 Anderson L G, Talal N. The spectrum of benign to malignant lymphoproliferation in Sjögren's syndrome.  Clin Exp Immunol. 1972;  10 199-221
• 88 Bonner Jr H, Ennis R S, Geelhoed G W, Tarpley Jr T M. Lymphoid infiltration and amyloidosis of lung in Sjögren's syndrome.  Arch Pathol. 1973;  95 42-44
• 89 Liebow A A, Carrington C B. Diffuse pulmonary lymphoreticular infiltrations associated with dysproteinemia.  Med Clin North Am. 1973;  57 809-843
• 90 Fortoul T I, Cano-Valle F, Oliva E, Barrios R. Follicular bronchiolitis in association with connective tissue diseases.  Lung. 1985;  163 305-314
• 91 Katzenstein A L. Primary lymphoid lung lesions. In: Katzenstein AL Katzenstein and Askin's surgical pathology of nonneoplastic lung disorders. 3rd ed. Philadelphia; Saunders 1997: 223-246
• 92 Kobayashi H, Matsuoka R, Kitamura S, Tsunoda N, Saito K. Sjögren's syndrome with multiple bullae and pulmonary nodular amyloidosis.  Chest. 1988;  94 438-440
• 93 Mariette X. Lymphomas in patients with Sjögren's syndrome: review of the literature and physiopathologic hypothesis.  Leuk Lymphoma. 1999;  33 93-99
• 94 Agostini C, Chilosi M, Zambello R, Trentin L, Semenzato G. Pulmonary immune cells in health and disease: lymphocytes.  Eur Respir J. 1993;  6 1378-1401
• 95 Sakamoto O, Saita N, Ando M et al.. Two cases of Sjögren's syndrome with multiple bullae.  Intern Med. 2002;  41 124-128
• 96 Hubscher O, Re R, Iotti R. Cystic lung disease in Sjögren's syndrome.  J Rheumatol. 2002;  29 2235-2236
• 97 Wells A U, du Bois R M. Bronchiolitis in association with connective tissue disorders.  Clin Chest Med. 1993;  14 655-666
• 98 Swigris J J, Berry G J, Raffin T A, Kuschner W G. Lymphoid interstitial pneumonia: a narrative review.  Chest. 2002;  122 2150-2164
• 99 Saltzstein S L. Pulmonary malignant lymphomas and pseudolymphomas: classification, therapy, and prognosis.  Cancer. 1963;  16 928-955
• 100 Liebow A A, Carrington C R, Friedman P J. Lymphomatoid granulomatosis.  Hum Pathol. 1972;  3 457-558
• 101 Katzenstein A L, Carrington C B, Liebow A A. Lymphomatoid granulomatosis: a clinicopathologic study of 152 cases.  Cancer. 1979;  43 360-373
• 102 Weisbrot I M. Lymphomatoid granulomatosis of the lung, associated with a long history of benign lymphoepithelial lesions of the salivary glands and lymphoid interstitial pneumonitis: report of a case.  Am J Clin Pathol. 1976;  66 792-801
• 103 Ioannidis J P, Vassiliou V A, Moutsopoulos H M. Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjögren's syndrome.  Arthritis Rheum. 2002;  46 741-747
• 104 Kamholz S, Sher A, Barland P et al.. Sjögren's syndrome: severe upper airways obstruction due to primary malignant tracheal lymphoma developing during successful treatment of lymphocytic interstitial pneumonitis.  J Rheumatol. 1987;  14 588-594
• 105 Theander E, Henriksson G, Ljungberg O et al.. Lymphoma and other malignancies in primary Sjögren's syndrome: a cohort study on cancer incidence and lymphoma predictors.  Ann Rheum Dis. 2006;  65 796-803
• 106 Honda O, Johkoh T, Ichikado K et al.. Differential diagnosis of lymphocytic interstitial pneumonia and malignant lymphoma on high-resolution CT.  AJR Am J Roentgenol. 1999;  173 71-74
• 107 Deheinzelin D, Capelozzi V L, Kairalla R A et al.. Interstitial lung disease in primary Sjögren's syndrome: clinical-pathological evaluation and response to treatment.  Am J Respir Crit Care Med. 1996;  154 794-799
• 108 Yamadori I, Fujita J, Bandoh S et al.. Nonspecific interstitial pneumonia as pulmonary involvement of primary Sjögren's syndrome.  Rheumatol Int. 2002;  22 89-92
• 109 American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias . This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001.  Am J Respir Crit Care Med. 2002;  165 277-304
• 110 Epler G R, Colby T V, McLoud T C, Carrington C B, Gaensler E A. Bronchiolitis obliterans organizing pneumonia.  N Engl J Med. 1985;  312 152-158
• 111 Matteson E L, Ike R W. Bronchiolitis obliterans organizing pneumonia and Sjögren's syndrome.  J Rheumatol. 1990;  17 676-679
• 112 Usui Y, Kimula Y, Miura H et al.. A case of bronchiolitis obliterans organizing pneumonia associated with primary Sjögren's syndrome who died of superimposed diffuse alveolar damage.  Respiration. 1992;  59 122-124
• 113 Homma H, Yamanaka A, Tanimoto S et al.. Diffuse panbronchiolitis: a disease of the transitional zone of the lung.  Chest. 1983;  83 63-69
• 114 Okano A, Sato A, Suda T et al.. A case of diffuse panbronchiolitis complicated by malignant thymoma and Sjögren's syndrome.  Nihon Kyobu Shikkan Gakkai Zasshi. 1991;  29 263-268
• 115 Ubukata M, Sugiyama S, Ohno S, Sugama Y, Kitamura S. A case of Sjögren's syndrome with a clinical course similar to diffuse panbronchiolitis.  Nihon Kyobu Shikkan Gakkai Zasshi. 1991;  29 387-391
• 116 Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises: case 23-1975.  N Engl J Med. 1975;  292 1285-1290
• 117 Sugai S. Mucosa-associated lymphoid tissue (MALT) lymphoma and primary amyloidosis in the lung in Sjögren's syndrome.  Intern Med. 2002;  41 251-252
• 118 Alvarez-Sala R, Sanchez-Toril F, Garcia-Martinez J, Zaera A, Masa J F. Primary Sjögren syndrome and pleural effusion.  Chest. 1989;  96 1440-1441
• 119 Ogihara T, Nakatani A, Ito H et al.. Sjögren's syndrome with pleural effusion.  Intern Med. 1995;  34 811-814
• 120 Skopouli F N, Dafni U, Ioannidis J P, Moutsopoulos H M. Clinical evolution, and morbidity and mortality of primary Sjögren's syndrome.  Semin Arthritis Rheum. 2000;  29 296-304
• 121 Manoussakis M N, Moutsopoulos H M. Sjögren's syndrome: current concepts.  Adv Intern Med. 2001;  47 191-217
• 122 Edwards J C, Cambridge G. B-cell targeting in rheumatoid arthritis and other autoimmune diseases.  Nat Rev Immunol. 2006;  6 394-403
• 123 Pijpe J, van Imhoff G W, Spijkervet F K et al.. Rituximab treatment in patients with primary Sjögren's syndrome: an open-label phase II study.  Arthritis Rheum. 2005;  52 2740-2750
• 124 Seror R, Sordet C, Guillevin L et al.. Tolerance and efficacy of rituximab and changes in serum B cell biomarkers in patients with systemic complications of primary Sjögren's syndrome.  Ann Rheum Dis. 2007;  66 351-357
• 125 Steinfeld S D, Tant L, Burmester G R et al.. Epratuzumab (humanised anti-CD22 antibody) in primary Sjögren's syndrome: an open-label phase I/II study.  Arthritis Res Ther. 2006;  8 R129-R139
• 126 Swords R, Power D, Fay M, O'Donnell R, Murphy P T. Interstitial pneumonitis following rituximab therapy for immune thrombocytopenic purpura (ITP).  Am J Hematol. 2004;  77 103-104
• 127 Brown S L, Miller R A, Horning S J et al.. Treatment of B-cell lymphomas with anti-idiotype antibodies alone and in combination with alpha interferon.  Blood. 1989;  73 651-661
• 128 Voulgarelis M, Giannouli S, Tzioufas A G, Moutsopoulos H M. Long term remission of Sjögren's syndrome associated aggressive B cell non-Hodgkin's lymphomas following combined B cell depletion therapy and CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone).  Ann Rheum Dis. 2006;  65 1033-1037