Clinical Rheumatology

, Volume 35, Issue 3, pp 825–828 | Cite as

Pulmonary Langerhans Histiocytosis: an uncommon cause of interstitial pneumonia in a patient with Sjögren syndrome

  • Andrés González García
  • José Luis Callejas Rubio
  • Raquel Ríos Fernández
  • Norberto Ortego Centeno
Case Based Review


Sjögren syndrome is a chronic, systemic, and autoimmune disorder that targets exocrine glands by remarkable B cell hyperactivity. Eventually, it is associated with extra-glandular clinical manifestations that affect essentially any organ system, including pulmonary involvement. Interstitial lung disease is one of the most serious pulmonary complications, and the early diagnosis is essential to initiate a prompt therapy. On the other hand, Sjögren syndrome could present concomitantly with several rheumatologic diseases such as systemic lupus erythematosus or rheumatoid arthritis. Pulmonary Langerhans Histiocytosis is a rare clonal proliferative disease characterized by pulmonary involvement by cells phenotypically similar to Langerhans cells. We describe the case of a nonsmoker 62-year-old woman with Sjögren syndrome who presented concomitantly a Pulmonary Langerhans Histiocytosis mimicking a pulmonary complication of its Sjögren. Fortunately, she had a well response to corticosteroids and azathioprine regimen. The aim of the paper is to emphasize the importance of the good differential diagnosis related to the pulmonary involvement. To the best of our knowledge, this is the first description of these two entities in the literature.


Azathioprine CD1a-stained cells Interstitial lung diseases Pulmonary Langerhans Histiocytosis Sarcoidosis Sjögren syndrome 





  1. 1.
    Ramos-Casals M, Brito-Zeron P, Siso-Almirall A, Bosch X (2012) Primary Sjogren syndrome. BMJ 344:e3821. doi: 10.1136/bmj.e3821 CrossRefPubMedGoogle Scholar
  2. 2.
    Parambil JG, Myers JL, Lindell RM, Matteson EL, Ryu JH (2006) Interstitial lung disease in primary Sjogren syndrome. Chest 130(5):1489–1495. doi: 10.1378/chest.130.5.1489 CrossRefPubMedGoogle Scholar
  3. 3.
    Vij R, Strek ME (2013) Diagnosis and treatment of connective tissue disease-associated interstitial lung disease. Chest 143(3):814–824. doi: 10.1378/chest. 12-0741 CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Schonfeld N, Frank W, Wenig S, Uhrmeister P, Allica E, Preussler H, Grassot A, Loddenkemper R (1993) Clinical and radiologic features, lung function and therapeutic results in pulmonary histiocytosis X. Respiration 60(1):38–44CrossRefPubMedGoogle Scholar
  5. 5.
    Watanabe M, Naniwa T, Hara M, Arakawa T, Maeda T (2010) Pulmonary manifestations in Sjogren’s syndrome: correlation analysis between chest computed tomographic findings and clinical subsets with poor prognosis in 80 patients. JRheumatol 37(2):365–373. doi: 10.3899/jrheum.090507 CrossRefGoogle Scholar
  6. 6.
    Koyama M, Johkoh T, Honda O, Mihara N, Kozuka T, Tomiyama N, Hamada S, Nakamura H (2001) Pulmonary involvement in primary Sjogren’s syndrome: spectrum of pulmonary abnormalities and computed tomography findings in 60 patients. J Thorac Imaging 16(4):290–296CrossRefPubMedGoogle Scholar
  7. 7.
    Vassallo R, Ryu JH, Colby TV, Hartman T, Limper AH (2000) Pulmonary Langerhans’ cell histiocytosis. N Engl J Med 342(26):1969–1978. doi: 10.1056/NEJM200006293422607 CrossRefPubMedGoogle Scholar
  8. 8.
    Auerswald U, Barth J, Magnussen H (1991) Value of CD-1-positive cells in bronchoalveolar lavage fluid for the diagnosis of pulmonary histiocytosis X. Lung 169(6):305–309CrossRefPubMedGoogle Scholar
  9. 9.
    Rees J, Paterson AW (2009) Langerhans cell histiocytosis in an adult. Br J Oral Maxillofac Surg 47(1):52–53. doi: 10.1016/j.bjoms.2008.04.010 CrossRefPubMedGoogle Scholar
  10. 10.
    Castillo D, Martin-Arroyo I, Moreno A, Balcells E, Villar A, Vicens-Zygmunt V, Vendrell M, Belda S, Portillo K, Kettou D, Aparicio F, Molina-Molina M, en nombre del grupo C (2014) Adult pulmonary Langerhans’ cell histiocytosis: approach to the reality of the Spanish population. Med Clin 143(10):433–439. doi: 10.1016/j.medcli.2013.11.039 CrossRefGoogle Scholar
  11. 11.
    Hoyles RK, Ellis RW, Wellsbury J, Lees B, Newlands P, Goh NS, Roberts C, Desai S, Herrick AL, McHugh NJ, Foley NM, Pearson SB, Emery P, Veale DJ, Denton CP, Wells AU, Black CM, du Bois RM (2006) A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum 54(12):3962–3970. doi: 10.1002/art.22204 CrossRefPubMedGoogle Scholar

Copyright information

© International League of Associations for Rheumatology (ILAR) 2015

Authors and Affiliations

  • Andrés González García
    • 1
  • José Luis Callejas Rubio
    • 2
  • Raquel Ríos Fernández
    • 2
  • Norberto Ortego Centeno
    • 2
  1. 1.Department of Internal MedicineUniversity Hospital Ramón y CajalMadridSpain
  2. 2.Systemic Autoimmune Diseases UnitHospital San CecilioGranadaSpain

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