Skip to main content

Advertisement

SpringerLink
Log in

Lung involvement in “stable” undifferentiated connective tissue diseases: a rheumatology perspective

  • Original Article
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Previous studies of the occurrence of interstitial lung disease (ILD) in undifferentiated connective tissue diseases (UCTD) were conducted in patients admitted to Respiratory Medicine Units. The aim of the present prospective study was to investigate lung involvement in UCTD patients admitted to a Rheumatology Unit. Eighty-one consecutive UCTD patients were enrolled in the study. Each patient underwent history and physical examination, routine laboratory investigations, antinuclear antibody (ANA) profiling, B-mode echocardiography, and lung function study according to previously reported methods. Lung high resolution computed tomography (HRCT) was performed in patients who provided informed consent. Six patients (7.4%) had a history of grade II dyspnea. Three of them had a DLCO ranging from 42 to 55% of the predicted value; and a HRCT-documented ILD with a non-specific interstitial pneumonia (NSIP) pattern. Symptoms in the other three patients were due to cardiac disease. None of the 75 asymptomatic patients, had relevant findings at physical examination, 26/75 had a DLCO <80% (<70% in 10 cases). Of these, 3 of the 30 patients who underwent lung HRCT were affected by NSIP-ILD. Six of the 81 enrolled were affected by ILD, which was symptomatic in three patients. A higher percentage of patients had a reduced DLCO. The latter finding may reflect a preradiographic ILD or a preechocardiographic pulmonary vascular disease.

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

Similar content being viewed by others

References

  1. Fischer A, du Bois R (2012) Interstitial lung disease in connective tissue in connective tissue disorders. Lancet 280:689–698

    Article  Google Scholar 

  2. Wells AU, Denton CP (2014) Interstitial lung disease in connective tissue disease: mechanisms and management. Nat Rev Rheumatol 10:728–739

    Article  CAS  PubMed  Google Scholar 

  3. Kinder BW, Collard HR, Koth L, Daikh DI, Wolters PJ, Elicker B et al (2007) Idiopathic nonspecific interstitial pneumonia lung manifestation of undifferentiated connective tissue disease? Am J Respir Crit Care Med 176:691–697

    Article  PubMed  PubMed Central  Google Scholar 

  4. Kinder BW, Shariat C, Collard HR, Koth LL, Wolters PJ, Golden JA et al (2010) Undifferentiated connective tissue disease-associated interstitial lung disease: changes in lung function. Lung 188:143–149

    Article  PubMed  PubMed Central  Google Scholar 

  5. Fischer A, West SG, Swigris JJ, Brown KK, du Bois RM (2010) Connective tissue disease associated interstitial lung disease: a call for clarification. Chest 138:251–256

    Article  PubMed  PubMed Central  Google Scholar 

  6. Vij R, Noth I, Strek ME (2011) Autoimmune-featured interstitial lung disease: a distinct entity. Chest 140:1292–1299

    Article  PubMed  PubMed Central  Google Scholar 

  7. Fischer A, Antoniou KM, Brown KK, Cadranel J, Corte TJ, du Bois RM, ERS/ATS Task Force on Undifferentiated Forms of CTD-ILD et al (2015) An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J 46:976–987

    Article  CAS  PubMed  Google Scholar 

  8. Mosca M, Neri R, Bombardieri S (1999) Undifferentiated connective tissue diseases (UCTD): review of the literature and proposal for preliminary classification criteria. Clin Exp Rheumatol 17:615–620

    CAS  PubMed  Google Scholar 

  9. Doria A, Mosca M, Gambari PF, Bombardieri S (2005) Defining unclassifiable connective tissue diseases: incomplete, undifferentiated, or both? J Rheumatol 32:213–215

    PubMed  Google Scholar 

  10. Mosca M, Tani C, Vagnani S, Carli L, Bombardieri S (2005) The diagnosis and classification of undifferentiated connective tissue diseases. J Autoimmun 48-49:50–52

    Article  Google Scholar 

  11. Valentini G, Marcoccia A, Cuomo G, Vettori S, Iudici M, Bondanini F, Santoriello C, Ciani A, Cozzolino D, De Matteis GM, Cappabianca S, Vitelli F, Spanò A (2014) Early systemic sclerosis: analysis of the disease course in patients with marker autoantibody and/or capillaroscopic positivity. Arthritis Care Res (Hoboken) 66(10):1520–1527

    Article  Google Scholar 

  12. Maricq HR (1981) Widefield capillary microscopy: technique and rating scale for abnormalities seen in scleroderma and related disorders. Arthritis Rheum 24:1159–1165

    Article  CAS  PubMed  Google Scholar 

  13. Maione S, Cuomo G, Giunta A et al (2005) Echocardiographic alterations in systemic sclerosis. A longitudinal study. Semin Arthritis Rheum 34:721–727

    Article  PubMed  Google Scholar 

  14. American Thoracic Society (1995) Single-breath carbon monoxide diffusing capacity (transfer factor). Recommendations for a standard technique-1995 update. Am J Respir Crit Care Med 152:2185–2198

    Article  Google Scholar 

  15. American Thoracic Society (1995) Standardization of spirometry—1995 update. Am J Respir Crit Care Med 152:1107–1136

    Article  Google Scholar 

  16. Steen VD, Owens GR, Fino GJ, Rodnan GP, Medsger TA (1985) Pulmonary involvement in systemic sclerosis (scleroderma). Arthritis Rheum 28:759–767

    Article  CAS  PubMed  Google Scholar 

  17. Pellegrino R, Viegi G, Brusasco V, Crapo RO, Burgos F, Casaburi R et al (2005) Interpretative strategies for lung function tests. Eur Respir J 26:948–968

    Article  CAS  PubMed  Google Scholar 

  18. Staitieh BS, Ioachimescu O (2017) Interpretation of pulmonary function tests: beyond the basics. J Investig med 65:301–310

    Article  PubMed  Google Scholar 

  19. Fischer A, Brown KK (2015) Interstitial lung disease in undifferentiated forms of connective tissue disease. Arthritis Care Res (Hoboken) 67:4–11

    Article  Google Scholar 

  20. Suda T, Kono M, Nakamura Y, Enomoto N, Kaida Y, Fujisawa T et al (2010) Distinct prognosis of idiopathic nonspecific interstitial pneumonia (NSIP) fulfilling criteria for undifferentiated connective tissue disease (UCTD). Respir Med 104:1527–1534

    Article  PubMed  Google Scholar 

  21. Steen VD, Graham G, Conte C, Owens G, Medsger TA Jr (1992) Isolated diffusing capacity reduction in systemic sclerosis. Arthritis Rheum 35:765–770

    Article  CAS  PubMed  Google Scholar 

  22. Iudici M, Cuomo G, Vettori S, Bocchino M, Sanduzzi Zamparelli A, Cappabianca S et al (2015) Low-dose pulse cyclophosphamide in interstitial lung disease associated with systemic sclerosis (SSc-ILD): efficacy of maintenance immunosuppression in responders and non-responders. Semin Arthritis Rheum 44:437–444

    Article  CAS  PubMed  Google Scholar 

  23. Tashkin DP, Volkmann ER, Tseng CH, Kim HJ, Goldin J, Clements P et al (2016) Relationship between quantitative radiographic assessments of interstitial lung disease and physiological and clinical features of systemic sclerosis. Ann Rheum Dis 75:374–381

    Article  CAS  PubMed  Google Scholar 

  24. Khanna D, Seibold JR, Wells A, Distler O, Allanore Y, Denton C et al (2010) Systemic sclerosis-associated interstitial lung disease: lessons from clinical trials, outcome measures, and future study design. Curr Rheumatol Rev 6:138–144

    Article  PubMed  PubMed Central  Google Scholar 

  25. Ferri C, Manfredi A, Sebastiani M, Colaci M, Giuggioli D, Vacchi C et al (2016) Interstitial pneumonia with autoimmune features and undifferentiated connective tissue disease: our interdisciplinary rheumatology-pneumology experience, and review of the literature. Autoimmun Rev 15:61–70

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

The authors thank Jean Ann Gilder (Scientific Communication srl., Naples, Italy) for editing the manuscript.

Author information

Authors and Affiliations

  1. Rheumatology Unit, Department of Internal and Experimental Medicine, University of Campania “Luigi Vanvitelli”, Naples, Italy

    Antonella Riccardi, Rosaria Irace, Ilaria Di Stefano, Michele Iudici, Serena Fasano & Gabriele Valentini

  2. Department of Clinical Medicine and Surgery, University “Federico II”, Medical School, Naples, Italy

    Marialuisa Bocchino, Annalisa Capaccio & Alessandro Sanduzzi

Authors
  1. Antonella Riccardi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Rosaria Irace
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ilaria Di Stefano
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Michele Iudici
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Serena Fasano
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Marialuisa Bocchino
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Annalisa Capaccio
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Alessandro Sanduzzi
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Gabriele Valentini
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Gabriele Valentini.

Ethics declarations

Financial support

None.

Disclosures

None.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Riccardi, A., Irace, R., Di Stefano, I. et al. Lung involvement in “stable” undifferentiated connective tissue diseases: a rheumatology perspective. Clin Rheumatol 36, 1833–1837 (2017). https://doi.org/10.1007/s10067-017-3704-8

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-017-3704-8

Keywords

Search

Navigation