Advertisement
Mayo Clinic Proceedings Home

Pulmonary Lymphoma in Sjögren's Syndrome

      Sjögren's syndrome (SS) is an immunologie disease characterized by progressive destruction of the exocrine glands that causes mucosal and conjunctival dryness. In addition to the common pulmonary complications of diffuse interstitial lung disease, airways obstruction, desiccation of the upper respiratory tract, localized parenchymal nodules, recurrent tracheobronchitis, bronchiectasis, interstitial pneumonitis, and pleural effusion, patients with SS have a high risk of developing non-Hodgkin's lymphoma that may affect the lungs. Among 50 patients with SS and associated lymphoma, 10 had pulmonary involvement by lymphoma. The mean age of these 10 patients was 59.7 years, and 8 were women. The mean duration of SS was 7.2 years, and the mean interval between the onset of SS and lymphoma was 5.4 years. Cough and slowly progressive dyspnea were the most common pulmonary symptoms, and chest roentgenographic findings varied. Lung biopsies revealed a spectrum of low- to high-grade lymphomas, and high-grade lymphomas were associated with increased mortality. Of the 10 patients, 4 died from 8 to 48 months after lymphoma was diagnosed. We conclude that pulmonary involvement is common in patients with lymphoma associated with SS; thus, lymphoma should be considered in the differential diagnosis of pulmonary lesions in patients with SS.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Mayo Clinic Proceedings
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      REFERENCES

        • Lane HC
        • Fauci AS
        Sjögren's syndrome.
        in: Braunwald E Isselbacher KJ Petersdorf RG Wilson JD Martin JB Fauci AS Harrison's Principles of Internal Medicine. Eleventh edition. McGraw-Hill Book Company, New York1987: 1433-1434
        • Talal N
        Sjögren's syndrome and connective tissue disease with other immunologic disorders.
        in: McCarty DJ Arthritis and Allied Conditions. Tenth edition. Lea & Febiger, Philadelphia1985: 1037-1051
        • Talal N
        Sjögren's syndrome.
        in: Wyngaarden JB Smith Jr, LH Cecil Textbook of Medicine. Eighteenth edition. WB Saunders Company, Philadelphia1988: 2024-2025
        • Fox RI
        • Howell FV
        • Bone RC
        • Michelson P
        Primary Sjogren syndrome: clinical and immunopathologic features.
        Semin Arthritis Rheum. 1984; 14: 77-105
        • Fox RI
        • Robinson CA
        • Curd JG
        • Kozin F
        • Howell FV
        Sjögren's syndrome: proposed criteria for classification.
        Arthritis Rheum. 1986; 29: 577-585
        • Kassan SS
        • Thomas TL
        • Moutsopoulos HM
        • Hoover R
        • Kimberly RP
        • Budman DR
        • Costa J
        • Decker JL
        • Chused TM
        Increased risk of lymphoma in sicca syndrome.
        Ann Intern Med. 1978; 89: 888-892
        • Moutsopoulos HM
        • Chused TM
        • Mann DL
        • Klippel JH
        • Fauci AS
        • Frank MM
        • Lawley TJ
        • Hamburger MI
        Sjögren's syndrome (sicca syndrome): current issues.
        Ann Intern Med. 1980; 92: 212-226
        • Alexander M
        Clinical aspects of Sjögren's syndrome.
        South Med J. 1986; 79: 857-862
        • Bloch KJ
        • Buchanan WW
        • Wohl MG
        • Bunim JJ
        Sjögren's syndrome: a clinical, pathological, and serological study of sixty-two cases.
        Medicine. 1965; 44: 187-231
        • Papathanasiou MI
        • Constantopoulos SH
        • Tsampoulas C
        • Drosos AA
        • Moutsopoulos HM
        Reappraisal of respiratory abnormalities in primary and secondary Sjögren's syndrome: a controlled study.
        Chest. 1986; 90: 370-374
        • Constantopoulos SH
        • Drosos AA
        • Maddison PJ
        • Moutsopoulos HM
        Xerotrachea and interstitial lung disease in primary Sjogren's disease.
        Respiration. 1984; 46: 310-314
        • Hunninghake GW
        • Fauci AS
        Pulmonary involvement in the collagen vascular diseases.
        Am Rev Respir Dis. 1979; 119: 471-503
        • Strimlan CV
        • Rosenow III, EC
        • Divertie MB
        • Harrison Jr, EG
        Pulmonary manifestations of Sjögren's syndrome.
        Chest. 1976; 70: 354-361
        • Constantopoulos SH
        • Papadimitriou CS
        • Moutsopoulos HM
        Respiratory manifestations in primary Sjögren's syndrome: a clinical, functional, and histologic study.
        Chest. 1985; 88: 226-229
        • Karlish AJ
        Lung changes in Sjögren's syndrome.
        Proc R Soc Med. 1969; 62: 1042-1043
        • Newball HH
        • Brahim SA
        Chronic obstructive airway, disease in patients with Sjögren's syndrome.
        Am Rev Respir Dis. 1977; 115: 295-304
        • Karsh J
        • Moutsopoulos HM
        • Vergalla J
        • Jones EA
        Protease inhibitor phenotypes and pulmonary disease in patients with Sjögren's syndrome.
        Respiration. 1981; 41: 60-65
        • Fairfax AJ
        • Haslam PL
        • Pavia D
        • Sheahan NF
        • Bateman JRM
        • Agnew JE
        • Clarke SW
        • Turner-Warwick M
        Pulmonary disorders associated with Sjögren's syndrome.
        Q J Med. 1981; 50: 279-295
        • Oxholm P
        • Bundgaard A
        • Madsen EB
        • Manthorpe R
        • Rasmussen FV
        Pulmonary function in patients with primary Sjögren's syndrome.
        Rheumatol Int. 1982; 2: 179-181
        • Forman MB
        • Zwi S
        • Gear AJ
        • Kallenbach J
        • Wing J
        Severe airway obstruction associated with rheumatoid arthritis and Sjögren's syndrome.
        S Afr Med J. 1982; 61: 674-676
        • Constantopoulos SH
        • Moutsopoulos HM
        Respiratory involvement in patients with Sjögren's syndrome: is it a problem?.
        Scand J Rheumatol Suppl. 1986; 61: 146-150
        • Molina R
        • Provost TT
        • Arnett FC
        • Bias WB
        • Hochberg MC
        • Wilson RW
        • Alexander EL
        Primary Sjögren's syndrome in men: clinical, serologic, and immunogenetic features.
        Am J Med. 1986; 80: 23-31
        • Martinez-Lavin M
        • Vaughan JH
        • Tan EM
        Autoantibodies and the spectrum of Sjögren's syndrome.
        Ann Intern Med. 1979; 91: 185-190
        • Hamburger MI
        • Moutsopoulos HM
        • Lawley TJ
        • Frank MM
        Sjögren's syndrome: a defect in reticuloendothelial system Fc-receptor-specific clearance.
        Ann Intern Med. 1979; 91: 534-538
        • Bonner Jr, H
        • Ennis RS
        • Geelhoed GW
        • Tarpley Jr, TM
        Lymphoid infiltration and amyloidosis of lung in Sjögren's syndrome.
        Arch Pathol. 1973; 95: 42-44
        • Ferreiro JE
        • Robalino BD
        • Saldana MJ
        Primary Sjögren's syndrome with diffuse cerebral vasculitis and lymphocytic interstitial pneumonitis.
        Am J Med. 1987; 82: 1227-1232
        • Fox RI
        • Adamson III, TC
        • Fong S
        • Robinson CA
        • Morgan EL
        • Robb JA
        • Howell FV
        Lymphocyte phenotype and function in pseudolymphoma associated with Sjögren's syndrome.
        J Clin Invest. 1983; 72: 52-62
        • Shearn MA
        Sjögren's syndrome.
        Major Probl Intern Med. 1971; 2: 1-255
        • Mason AMS
        • Gumpel JM
        • Golding PL
        Sjögren's syndrome—a clinical review.
        Semin Arthritis Rheum. 1973; 2: 301-331
        • Anderson LG
        • Talal N
        The spectrum of benign to malignant lymphoproliferation in Sjögren's syndrome.
        Clin Exp Immunol. 1972; 10: 199-221
        • Liebow AA
        • Carrington CB
        Diffuse pulmonary lymphoreticular infiltrations associated with dysproteinemia.
        Med Clin North Am. May 1973; 57: 809-843
        • Strimlan CV
        • Rosenow III, EC
        • Weiland LH
        • Brown LR
        Lymphocytic interstitial pneumonitis: review of 13 cases.
        Ann Intern Med. 1978; 88: 616-621
        • Faguet GB
        • Webb HH
        • Agee JF
        • Ricks WB
        • Sharbaugh AH
        Immunologically diagnosed malignancy in Sjögren's pseudolymphoma.
        Am J Med. 1978; 65: 424-429
        • Adamson III, TC
        • Fox RI
        • Frisman DM
        • Howell FV
        Immunohistologic analysis of lymphoid infiltrates in primary Sjögren's syndrome using monoclonal antibodies.
        J Immunol. 1983; 130: 203-208
        • 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. 1979; 299: 1215-1220
      1. Case records of the Massachusetts General Hospital (Case 29-1981).
        N Engl J Med. 1981; 305: 153-160
        • Sugai S
        • Konda S
        • Shirasaki Y
        • Murayama T
        • Nishikawa T
        Non-IgM monoclonal gammopathy in patients with Sjögren's syndrome.
        Am J Med. 1980; 68: 861-866
        • Shearn MA
        • Moutsopoulos HM
        • Sawada S
        • Gak C
        Sjögren's syndrome with light chain myeloma (letter to the editor).
        West J Med. 1975; 123: 496-497
        • Wilke WS
        • Tubbs RR
        • Bukowski RM
        • Currie TE
        • Calabrese LH
        • Weiss RA
        • Savage RA
        • Sebek BA
        T cell lymphoma occurring in Sjögren's syndrome.
        Arthritis Rheum. 1984; 27: 951-954
        • Pagani JJ
        • Collins JD
        • Reza MJ
        Sjogren's syndrome presenting as pulmonary pseudolymphoma: report of a case.
        J Natl Med Assoc. 1979; 71: 677-678
        • Greenberg SD
        • Heisler JG
        • Gyorkey F
        • Jenkins DE
        Pulmonary lymphoma versus pseudolymphoma: a perplexing problem.
        South Med J. 1972; 65: 775-784
        • Saltzstein SL
        Pulmonary malignant lymphomas and pseudolymphomas: classification, therapy, and prognosis.
        Cancer. 1963; 16: 928-955
        • Talal N
        • Sokoloff L
        • Barth WF
        Extrasalivary lymphoid abnormalities in Sjögren's syndrome (reticulum cell sarcoma, “pseudolymphoma,” macroglobulinemia).
        Am J Med. 1967; 43: 50-65
        • Talal N
        • Bunim JJ
        The development of malignant lymphoma in the course of Sjögren's syndrome.
        Am J Med. 1964; 36: 529-540
      2. Case records of the Massachusetts General Hospital (Case 28-1975).
        N Engl J Med. 1975; 293: 136-144
        • Sutinen S
        • Sutinen S
        • Huhti E
        Ultrastructure of lymphoid interstitial pneumonia: virus-like particles in bronchiolar epithelium of patient with Sjögren's syndrome.
        Am J Clin Pathol. 1977; 67: 328-333
        • Cummings NA
        • Schall GL
        • Asofsky R
        • Anderson LG
        • Talal N
        Sjögren's syndrome—newer aspects of research, diagnosis, and therapy.
        Ann Intern Med. 1971; 75: 937-950
        • Goldenberg GJ
        • Parakevas F
        • Israels LG
        The association of rheumatoid arthritis with plasma cell and lymphocytic neoplasms.
        Arthritis Rheum. 1969; 12: 569-579
        • Colby TV
        • Carrington CB
        Lymphoreticular tumors and infiltrates of the lung.
        Pathol Annu. 1983; 18: 27-70
        • Herbert A
        • Wright DH
        • Isaacson PG
        • Smith JL
        Primary malignant lymphoma of the lung: histopathologic and immunologic evaluation of nine cases.
        Hum Pathol. 1984; 15: 415-422
        • Sugai S
        • Takiguchi T
        • Hirose Y
        • Shimizu S
        • Tachibana J
        • Konda S
        • Konishi F
        B cell malignant lymphoma in a patient with progressive systemic sclerosis and Sjögren's syndrome: report of a case and review of the literature.
        Jpn J Med. 1985; 24: 155-163
        • Capron F
        • Audouin J
        • Diebold J
        • Ameille J
        • Lebeau B
        • Rochemaure J
        Pulmonary polymorphic centroblastic type malignant lymphoma in a patient with lymphomatoid granulomatosis, Sjögren's syndrome and other manifestations of a dysimmune state.
        Pathol Res Pract. 1985; 179: 656-661
        • Herbert A
        • Walters MT
        • Cawley MID
        • Godfrey RC
        Lymphocytic interstitial pneumonia identified as lymphoma of mucosa associated lymphoid tissue.
        J Pathol. 1985; 146: 129-138
        • Walters MT
        • Stevenson FK
        • Herbert A
        • Cawley MID
        • Smith JL
        Lymphoma in Sjögren's syndrome: urinary monoclonal free light chains as a diagnostic aid and a means of tumour monitoring.
        Scand J Rheumatol Suppl. 1986; 61: 114-117
        • Spencer H
        Third edition. Pathology of the Lung. Vol 2. Pergamon Press, Oxford1977: 937-938
        • Gibbs AR
        • Seal RME
        Primary lymphoproliferative conditions of lung.
        Thorax. 1978; 33: 140-152
        • Colby TV
        • Carrington CB
        Pulmonary lymphomas: current concepts.
        Hum Pathol. 1983; 14: 884-887
        • Isaacson P
        • Wright DH
        Extranodal malignant lymphoma arising from mucosa-associated lymphoid tissue.
        Cancer. 1984; 53: 2515-2524
        • Isaacson PG
        • Spencer J
        Malignant lymphoma of mucosa-associated lymphoid tissue.
        Histopathology. 1987; 11: 445-462