Skip to main content

Advertisement

SpringerLink
Log in

Pulmonary alveolar proteinosis as an unusual pattern of lung involvement in Sjögren syndrome

  • Short Communication
  • Published:
Rheumatology International Aims and scope Submit manuscript

Abstract

Primary Sjögren syndrome (pSS) is an autoimmune disease that is characterized by infiltration of lymphocytes in exocrine glands, followed by hypofunction of the glands. Dry mouth and eyes are main symptoms, but there are various extraglandular manifestations in pSS. Nine to 75% of patients have pulmonary involvement such as interstitial lung diseases. Pulmonary alveolar proteinosis (PAP) is also a kind of autoimmune disorder, characterized by the presence of anti-granulocyte/macrophage colony-stimulating factor antibody. A 45-year-old nonsmoking woman was admitted with dyspnea and dry cough. She was diagnosed as having PAP, based on characteristic computed tomography, bronchoalveolar lavage, and lung biopsy findings. At the same time, she was diagnosed with pSS, based on dry mouth, xerophthalmia, positive anti-Ro/La antibodies, and scintigraphic finding of salivary glands. Whole lung lavages and monthly intravenous cyclophosphamide had slight improvement in PAP.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

References

  1. Rosen SH, Castleman B, Liebow AA (1958) Pulmonary alveolar proteinosis. N Engl J Med 258(23):1123–1142

    Article  PubMed  CAS  Google Scholar 

  2. Carnovale R, Zornoza J, Goldman AM et al (1977) Pulmonary alveolar proteinosis: its association with hematologic malignancy and lymphoma. Radiology 122(2):303–306

    PubMed  CAS  Google Scholar 

  3. Oerlemans WG, Jansen EN, Prevo RL et al (1998) Primary cerebellar nocardiosis and alveolar proteinosis. Acta Neurol Scand 97(2):138–141

    Article  PubMed  CAS  Google Scholar 

  4. Patiroglu T, Akyildiz B, Patiroglu TE et al (2008) Recurrent pulmonary alveolar proteinosis secondary to agammaglobulinemia. Pediatr Pulmonol 43(7):710–713

    Article  PubMed  Google Scholar 

  5. Ranchod M, Bissell M (1979) Pulmonary alveolar proteinosis and cytomegalovirus infection. Arch Pathol Lab Med 103(3):139–142

    PubMed  CAS  Google Scholar 

  6. Stanley E, Lieschke GJ, Grail D et al (1994) Granulocyte/macrophage colony-stimulating factor-deficient mice show no major perturbation of hematopoiesis but develop a characteristic pulmonary pathology. Proc Natl Acad Sci USA 91(12):5592–5596

    Article  PubMed  CAS  Google Scholar 

  7. Reed JA, Ikegami M, Cianciolo ER et al (1999) Aerosolized GM-CSF ameliorates pulmonary alveolar proteinosis in GM-CSF-deficient mice. Am J Physiol 276(4 Pt 1):L556–L563

    PubMed  CAS  Google Scholar 

  8. Huffman Reed JA, Rice WR, Zsengeller ZK et al (1997) GM-CSF enhances lung growth and causes alveolar type II epithelial cell hyperplasia in transgenic mice. Am J Physiol 273(4 Pt 1):L715–L725

    PubMed  CAS  Google Scholar 

  9. Golde DW, Territo M, Finley TN et al (1976) Defective lung macrophages in pulmonary alveolar proteinosis. Ann Intern Med 85(3):304–309

    PubMed  CAS  Google Scholar 

  10. Harris JO (1979) Pulmonary alveolar proteinosis: abnormal in vitro function of alveolar macrophages. Chest 76(2):156–159

    Article  PubMed  CAS  Google Scholar 

  11. Gonzalez-Rothi RJ, Harris JO (1986) Pulmonary alveolar proteinosis. Further evaluation of abnormal alveolar macrophages. Chest 90(5):656–661

    Article  PubMed  CAS  Google Scholar 

  12. Pertovaara M, Korpela M, Uusitalo H et al (1999) Clinical follow up study of 87 patients with sicca symptoms (dryness of eyes or mouth, or both). Ann Rheum Dis 58(7):423–427

    Article  PubMed  CAS  Google Scholar 

  13. Oxholm P (1992) Primary Sjögren’s syndrome–clinical and laboratory markers of disease activity. Semin Arthritis Rheum 22(2):114–126

    Article  PubMed  CAS  Google Scholar 

  14. Strimlan CV, Rosenow EC III, Divertie MB et al (1976) Pulmonary manifestations of Sjögren’s syndrome. Chest 70(03):354–361

    Article  PubMed  CAS  Google Scholar 

  15. Constantopoulos SH, Papadimitriou CS, Moutsopoulos HM (1985) Respiratory manifestations in primary Sjögren’s syndrome. A clinical, functional, and histologic study. Chest 88(2):226–229

    Article  PubMed  CAS  Google Scholar 

  16. Gardiner P, Ward C, Allison A et al (1993) Pleuropulmonary abnormalities in primary Sjögren’s syndrome. J Rheumatol 20(5):831–837

    PubMed  CAS  Google Scholar 

  17. Trapnell BC, Whitsett JA, Nakata K (2003) Pulmonary alveolar proteinosis. N Engl J Med 349(26):2527–2539

    Article  PubMed  CAS  Google Scholar 

  18. Uchiyama M, Nagao T, Hattori A et al (2009) Pulmonary alveolar proteinosis in a patient with Behcet’s disease. Respirology 14(2):305–308

    Article  PubMed  Google Scholar 

  19. Seymour JF, Presneill JJ (2002) Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir Crit Care Med 166(2):215–235

    Article  PubMed  Google Scholar 

  20. Borie R, Debray MP, Laine C et al (2009) Rituximab therapy in autoimmune pulmonary alveolar proteinosis. Eur Respir J 33(6):1503–1506

    Article  PubMed  CAS  Google Scholar 

  21. Danel C, Israel-Biet D, Costabel U et al (1992) Therapeutic applications of bronchoalveolar lavage. Eur Respir J 5(10):1173–1175

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Rheumatology, Department of Internal Medicine, Konkuk University School of Medicine, 265 Guuiro, Gwangjin-gu, Seoul, 143-729, Korea

    Eugene Park, Hae-Rim Kim & Sang-Heon Lee

  2. Division of Pulmonology, Department of Internal Medicine, Konkuk University School of Medicine, Seoul, Korea

    Hee Joung Kim

Authors
  1. Eugene Park
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Hae-Rim Kim
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Hee Joung Kim
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Sang-Heon Lee
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Hae-Rim Kim.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Park, E., Kim, HR., Kim, H.J. et al. Pulmonary alveolar proteinosis as an unusual pattern of lung involvement in Sjögren syndrome. Rheumatol Int 32, 2945–2948 (2012). https://doi.org/10.1007/s00296-011-2001-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-011-2001-0

Keywords

Search

Navigation