Semin Respir Crit Care Med 2010; 31(4): 409-418
DOI: 10.1055/s-0030-1262209
© Thieme Medical Publishers

Assessing Pulmonary Disease and Response to Therapy: Which Test?

Greg Keir1 , Athol U. Wells1
  • 1Interstitial Lung Disease Unit, Royal Brompton Hospital, London, United Kingdom
Further Information

Publication History

Publication Date:
27 July 2010 (online)

ABSTRACT

Because 75% of deaths attributable to sarcoidosis occur due to progressive respiratory failure, the staging of pulmonary disease and the accurate identification of changes in disease severity with time are both an essential part of clinical management. Historically, pulmonary function tests (PFTs) and chest radiographic appearances have been applied to both goals. Several additional investigations have been proposed as markers of active disease, including gallium scanning, positron emission tomographic (PET) scanning, high-resolution computed tomographic (HRCT) scanning, bronchoalveolar lavage, and candidate biomarkers such as serum angiotensin-converting enzyme (ACE) levels and serum interleukin (IL)-2 receptor levels. However, none of these tests has been shown to add value to PFTs and chest radiography, either in staging disease at baseline or in detecting change, although PET scanning merits further evaluation with particular reference to the suppression of activity with treatment in irreversible disease. Furthermore, no single pulmonary function or chest radiographic variable in isolation is accurate in all cases in this heterogeneous disease. Thus the evaluation of pulmonary disease in sarcoidosis is a multidisciplinary exercise, with the integration of PFTs (including measures of gas transfer) and chest radiographic findings (best assessed using simple user-friendly grading systems) with symptomatic severity and change.

REFERENCES

  • 1 Turner-Warwick M, McAllister W, Lawrence R, Britten A, Haslam P L. Corticosteroid treatment in pulmonary sarcoidosis: do serial lavage lymphocyte counts, serum angiotensin converting enzyme measurements, and gallium-67 scans help management?.  Thorax. 1986;  41 903-913
  • 2 Keogh B A, Crystal R G. Clinical significance of pulmonary function tests. Pulmonary function testing in interstitial pulmonary disease. What does it tell us?.  Chest. 1980;  78 856-865
  • 3 Neville E, Walker A, James D G. Prognostic factors predicting outcome of sarcoidosis: an analysis of 818 patients.  Q J Med. 1983;  52 525-533
  • 4 Rømer F K. Presentation of sarcoidosis and outcome of pulmonary changes.  Dan Med Bull. 1982;  29 27-32
  • 5 Alhamad E H, Lynch III J P, Martinez F J. Pulmonary function tests in interstitial lung disease: what role do they have?.  Clin Chest Med. 2001;  22 715-750 ix
  • 6 Harrison B D, Shaylor J M, Stokes T C, Wilkes A R. Airflow limitation in sarcoidosis—a study of pulmonary function in 107 patients with newly diagnosed disease.  Respir Med. 1991;  85 59-64
  • 7 Lynch III J P, Kazerooni E A, Gay S E. Pulmonary sarcoidosis.  Clin Chest Med. 1997;  18 755-785
  • 8 Gleeson F V, Traill Z C, Hansell D M. Evidence of expiratory CT scans of small-airway obstruction in sarcoidosis.  AJR Am J Roentgenol. 1996;  166 1052-1054
  • 9 Hansell D M, Milne D G, Wilsher M L, Wells A U. Pulmonary sarcoidosis: morphologic associations of airflow obstruction at thin section CT.  Radiology. 1998;  209 697-704
  • 10 Chambellan A, Turbie P, Nunes H, Brauner M, Battesti J P, Valeyre D. Endoluminal stenosis of proximal bronchi in sarcoidosis: bronchoscopy, function, and evolution.  Chest. 2005;  127 472-481
  • 11 Carrington C B. Structure and function in sarcoidosis.  Ann N Y Acad Sci. 1976;  278 265-283
  • 12 Levinson R S, Metzger L F, Stanley N N et al.. Airway function in sarcoidosis.  Am J Med. 1977;  62 51-59
  • 13 Shorr A F, Davies D B, Nathan S D. Predicting mortality in patients with sarcoidosis awaiting lung transplantation.  Chest. 2003;  124 922-928
  • 14 Baughman R P, Engel P J, Meyer C A, Barrett A B, Lower E E. Pulmonary hypertension in sarcoidosis.  Sarcoidosis Vasc Diffuse Lung Dis. 2006;  23 108-116
  • 15 Scadding J G. Prognosis of intrathoracic sarcoidosis in England.  BMJ. 1961;  2 1165-1172
  • 16 Israel H L, Lenchner G, Steiner R M. Late development of mediastinal calcification in sarcoidosis.  Am Rev Respir Dis. 1981;  124 302-305
  • 17 Gawne-Cain M L, Hansell D M. The pattern and distribution of calcified mediastinal lymph nodes in sarcoidosis and tuberculosis: a CT study.  Clin Radiol. 1996;  51 263-267
  • 18 Smellie H, Hoyle C. The natural history of pulmonary sarcoidosis.  Q J Med. 1960;  29 539-558
  • 19 Ellis K, Renthal G. Pulmonary sarcoidosis. Roentgenographic observations on course of disease.  AJR Am J Roentgenol. 1962;  88 1070-1083
  • 20 Costabel U, Guzman J. Bronchoalveolar lavage in interstitial lung disease.  Curr Opin Pulm Med. 2001;  7 255-261
  • 21 Remy-Jardin M, Giraud F, Remy J, Wattinne L, Wallaert B, Duhamel A. Pulmonary sarcoidosis: role of CT in the evaluation of disease activity and functional impairment and in prognosis assessment.  Radiology. 1994;  191 675-680
  • 22 Hansell D M, Armstrong P, Lynch D A, McAdams H P. Imaging of Diseases of the Chest. 4th ed. Philadelphia; Elsevier Mosby 2005: 635-636
  • 23 Drent M, De Vries J, Lenters M et al.. Sarcoidosis: assessment of disease severity using HRCT.  Eur Radiol. 2003;  13 2462-2471
  • 24 Oberstein A, von Zitzewitz H, Schweden F, Müller-Quernheim J. Non invasive evaluation of the inflammatory activity in sarcoidosis with high-resolution computed tomography.  Sarcoidosis Vasc Diffuse Lung Dis. 1997;  14 65-72
  • 25 Köhn H, Klech H, Mostbeck A, Kummer F. 67Ga scanning for assessment of disease activity and therapy decisions in pulmonary sarcoidosis in comparison to chest radiography, serum ACE and blood T-lymphocytes.  Eur J Nucl Med. 1982;  7 413-416
  • 26 Nishiyama Y, Yamamoto Y, Fukunaga K et al.. Comparative evaluation of 18F-FDG PET and 67Ga scintigraphy in patients with sarcoidosis.  J Nucl Med. 2006;  47 1571-1576
  • 27 Braun J J, Kessler R, Constantinesco A, Imperiale A. 18F-FDG PET/CT in sarcoidosis management: review and report of 20 cases.  Eur J Nucl Med Mol Imaging. 2008;  35 1537-1543
  • 28 Teirstein A S, Machac J, Almeida O, Lu P, Padilla M L, Iannuzzi M C. Results of 188 whole-body fluorodeoxyglucose positron emission tomography scans in 137 patients with sarcoidosis.  Chest. 2007;  132 1949-1953
  • 29 Keijsers R GM, Verzijlbergen J F, van Diepen D M, van den Bosch J MM, Grutters J C. 18F-FDG PET in sarcoidosis: an observational study in 12 patients treated with infliximab.  Sarcoidosis Vasc Diffuse Lung Dis. 2008;  25 143-149
  • 30 Ziegenhagen M W, Rothe M E, Schlaak M, Müller-Quernheim J. Bronchoalveolar and serological parameters reflecting the severity of sarcoidosis.  Eur Respir J. 2003;  21 407-413
  • 31 Drent M, Jacobs J A, de Vries J, Lamers R JS, Liem I H, Wouters E FM. Does the cellular bronchoalveolar lavage fluid profile reflect the severity of sarcoidosis?.  Eur Respir J. 1999;  13 1338-1344
  • 32 Shorr A F, Torrington K G, Parker J M. Serum angiotensin converting enzyme does not correlate with radiographic stage at initial diagnosis of sarcoidosis.  Respir Med. 1997;  91 399-401
  • 33 Leung A N, Brauner M W, Caillat-Vigneron N, Valeyre D, Grenier P. Sarcoidosis activity: correlation of HRCT findings with those of 67Ga scanning, bronchoalveolar lavage, and serum angiotensin-converting enzyme assay.  J Comput Assist Tomogr. 1998;  22 229-234
  • 34 Stokes G S, Monaghan J C, Schrader A P, Glenn C L, Ryan M, Morris B J. Influence of angiotensin converting enzyme (ACE) genotype on interpretation of diagnostic tests for serum ACE activity.  Aust N Z J Med. 1999;  29 315-318
  • 35 Bradley B, Branley H M, Egan J J British Thoracic Society Interstitial Lung Disease Guideline Group, British Thoracic Society Standards of Care Committee et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society.  Thorax. 2008;  63(Suppl 5) v1-v58
  • 36 Ziegenhagen M W, Benner U K, Zissel G, Zabel P, Schlaak M, Müller-Quernheim J. Sarcoidosis: TNF-alpha release from alveolar macrophages and serum level of sIL-2R are prognostic markers.  Am J Respir Crit Care Med. 1997;  156 1586-1592
  • 37 Keicho N, Kitamura K, Takaku F, Yotsumoto H. Serum concentration of soluble interleukin-2 receptor as a sensitive parameter of disease activity in sarcoidosis.  Chest. 1990;  98 1125-1129
  • 38 Grutters J C, Fellrath J M, Mulder L, Janssen R, van den Bosch J M, van Velzen-Blad H. Serum soluble interleukin-2 receptor measurement in patients with sarcoidosis: a clinical evaluation.  Chest. 2003;  124 186-195
  • 39 Müller-Quernheim J, Pfeifer S, Strausz J, Ferlinz R. Correlation of clinical and immunologic parameters of the inflammatory activity of pulmonary sarcoidosis.  Am Rev Respir Dis. 1991;  144 1322-1329
  • 40 ILO Guidelines for the Use of ILO International Classification of Radiographs of Pneumoconioses. Geneva; International Labor Office 1980 Occupational Safety Series No. 22
  • 41 Judson M A, Gilbert G E, Rodgers J K, Greer C F, Schabel S I. The utility of the chest radiograph in diagnosing exacerbations of pulmonary sarcoidosis.  Respirology. 2008;  13 97-102
  • 42 Gibson G J, Prescott R J, Muers M F et al. British Thoracic Society Sarcoidosis study: effects of long term corticosteroid treatment.  Thorax. 1996;  51 238-247
  • 43 Muers M F, Middleton W G, Gibson G J et al.. A simple radiographic scoring method for monitoring pulmonary sarcoidosis: relations between radiographic scores, dyspnoea grade and respiratory function in the British Thoracic Society Study of Long-Term Corticosteroid Treatment.  Sarcoidosis Vasc Diffuse Lung Dis. 1997;  14 46-56
  • 44 Baughman R P, Shipley R, Desai S Sarcoidosis Investigators et al. Changes in chest roentgenogram of sarcoidosis patients during a clinical trial of infliximab therapy: comparison of different methods of evaluation.  Chest. 2009;  136 526-535
  • 45 American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and Other Granulomatous Disorders . Statement on Sarcoidosis.  Am J Respir Crit Care Med. 1999;  160 736-755

Athol U WellsM.D. 

Interstitial Lung Disease Unit, Royal Brompton Hospital, Emmanuel Kaye Bldg., Manresa Rd., Chelsea, London

SW3 6LR UK

Email: A.wells@rbh.nthames.nhs.uk

    >