Main Article Content


Interstitial lung diseases (ILD) are known as a debilitating pulmonary complications that may be occured in almost all systemic connective tissue diseases (CTD), including mixed connective tissue disease (MCTD). ILD is usually found in more than half of MCTD patients after 2-4
years after the diagnosis made. A-47-years-old female initially diagnosed as systemic lupus erythematosus (SLE) developed a severe progressive dyspnea. She has recently diagnosed as MCTD with ILD after 9 months of initial symptoms. She was giving with Cyclophosphamide 500 mg IV pulse dose. However, after 1 months she developed severe pneumonia and
pronounced demise due to intractable septic shock. The debilitating course of ILD is commonly seen in most systemic CTD. Therefore, it is important to perform initial screening and prevention. Systemic corticosteroid with or without immunosupressor agent(s) are indicated in
ILD-MCTD. Patients with progressive diseases will have poor prognosis.
Keywords : ILD, MCTD, Corticosteroid

Article Details

How to Cite
Tendean, M., Nuriawan, S. A., & Nugroho, P. (2017). Interstitial lung disease in mixed connective tissue disease. Indonesian Journal of Rheumatology, 9(1).


  1. Capobianco J, Grimberg A, Thompson BM, et al. Thoracic Manifestations of Collagen Vascular Diseases. RadioGraphics 2011; 32: 33–50.
  2. American Thoracic Society, European Respiratory Society. American Thoracic Society / European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med. 2002;165 (2): 277–304.
  3. Castelino FV, Varga J. Interstitial lung disease in connective tissue diseases : evolving concepts of pathogenesis and management. Arthritis Res Ther. 2010; 12(4): 213–224.
  4. Mathai SC, Danoff SK. Management of interstitial lung disease associated with connective tissue disease. BMJ. 2016; 352: h6819.
  5. Fischer A, Antoniou KM, Brown KK, et al. An official European Respiratory Society / American Thoracic Society research statement : interstitial pneumonia with autoimmune features. Eur Respir J. 2015; 46(4):976–987.
  6. Travis WD, Costabel U, Hansell DM, et al. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013; 188(6): 733–748.
  7. Narsimulu G. Mixed connective tissue disorders (mctd). Med Updat 2010;17:842–845.
  8. Bennet R. Overlap syndromes. In: Kelley’s Textbook of Rheumatology Garry S F, Ralph C B, Harris Jr ED, et al. (eds). USA: Saunders; 2017: 1489–1511.
  9. Bodolay E, Szekanecz Z, Vegh J, et al. Mixed connective tissue disease: should the diagnosis be more restrictive?: reply. Rheumatol 2005; 44: 1466–1467.
  10. Bull TM, Fagan KA, Badesch DB. Pulmonary Vascular Manifestations of Mixed Connective Tissue Disease. Rheum Dis Clin 2017; 31: 451–464.
  11. Vegh J, Szilasi M, Soos G, et al. Interstitial lung disease in mixed connective tissue disease. Orv Hetil 2005; 146: 2435–2443.
  12. Colin G, Nunes H, Hatron PY, et al. Étude Des Pneumopathies Interstitielles Diffuses De La Connectivite Mixte. Rev Mal Respir 2010; 27: 238–246.
  13. Gunnarsson R, Aaløkken TM, Molberg Ø, et al. Prevalence and severity of interstitial lung disease in mixed connective tissue disease : a nationwide, cross-sectional study. Ann Rheum Dis 2012; 1966–1972.
  14. Jindal SK, Agarwal R. Autoimmunity and interstitial lung disease. Curr Opin Pulm Med 2005; 11: 438–446.
  15. Kozuka T, Johkoh T, Honda O, et al. Pulmonary involvement in mixed connective tissue disease: high-resolution CT findings in 41 patients. J ThoracImaging 2001;16:94–98.
  16. Meyer KC. Diagnosis and management of interstitial lung disease. Transl Respir Med 2014; 2: 1–13.
  17. Bodolay E, Szekanecz Z, Dévényi K, et al. Evaluation of interstitial lung disease in mixed connective tissue disease (MCTD). Rheumatol 2005; 44: 656–661.
  18. Kim EA, Lee KS, Johkoh T, et al. Interstitial Lung Diseases Associated with Collagen Vascular Diseases: Radiologic and Histopathologic Findings. RadioGraphics 2002; 22: S151–S165.