Chest
Volume 129, Issue 5, May 2006, Pages 1234-1245
Journal home page for Chest

Original Research: Sarcoidosis
A New Tool To Assess Sarcoidosis Severity

https://doi.org/10.1378/chest.129.5.1234Get rights and content

Study objectives

Sarcoidosis is a granulomatous disorder primarily affecting the lung, but with frequent extrapulmonary organ involvement. There are no comprehensive scoring systems for sarcoidosis disease severity. Our goal was to develop and validate an objective and comprehensive sarcoidosis disease severity scoring system.

Design

Three sarcoidosis experts reviewed clinical data on 104 patients with biopsy-confirmed sarcoidosis. Each expert independently scored disease severity using a visual analog scale. Interrater agreement was assessed. Univariate analysis was performed, and those variables with p values ≤ 0.25 were used in backward regression multivariable analysis. A model was obtained including variables with a p value of ≤ 0.15 to predict severity scores. This model was subsequently validated using an independent panel of three additional international experts.

Setting

Granuloma clinic at National Jewish Medical and Research Center.

Patients

A total of 104 patients with biopsy-confirmed sarcoidosis.

Interventions

None.

Measurements and results

Pairwise assessment of interrater agreement yielded high degrees of correlation with Spearman correlation coefficients of 0.86 to 0.89 and an intraclass correlation coefficient of 0.87. Univariate analysis showed that smoking status, immunosuppressive therapy, percent predicted for diffusing capacity of the lung for carbon monoxide (Dlco), FEV1, FVC, and total lung capacity, FEV1/FVC ratio, disease duration, sites of organ involvement, and African-American race were associated with mean severity score. The multivariable model included cardiac and neurologic involvement, current therapy with noncorticosteroid immunosuppressive agents, Dlco percent predicted, FEV1/FVC ratio, African-American race, FVC percent predicted, and skin involvement. This model was validated using additional reviewer scores yielding Spearman correlation coefficients of 0.66 to 0.76 and an intraclass correlation coefficient of 0.74.

Conclusions

We derived an objective disease severity scoring system that incorporates data on demographics, pulmonary function, and organ involvement to produce a whole-body sarcoidosis assessment. This preliminary tool has potential applicability in the assessment of disease severity in sarcoidosis research.

Section snippets

Study Population

Sarcoidosis patients (n = 104) were recruited from the granuloma clinic at National Jewish Medical and Research Center between January 2002 and August 2004. All patients met the diagnostic criteria for sarcoidosis established by the American Thoracic Society consensus panel.2 All study subjects were adults between the ages of 18 and 70 years, and provided their informed consent. The consent and the protocol were approved by the National Jewish Medical and Research Center Institutional Review

Characterization of Study Population

The study population consisted of 39 men and 65 women. The demographic features of this group are presented in Table 1. Of note, the race distribution of sarcoidosis patients in this population reflects the local demographics for Colorado. The clinical characteristics of the patient population are summarized in Table 1, Table 2, and reflect a wide range of chest radiograph Scadding stage and organ involvement. All but one subject had pulmonary involvement, with a variety of lung function

Summary

Sarcoidosis is a disease with great phenotypic diversity, with the potential for numerous sites of organ involvement, varying levels of disease activity, and varying degrees of long-term organ dysfunction resulting from periods of active inflammation and, potentially, fibrosis. Our purpose in this study was to create a research tool that would incorporate these many aspects of the disease into a single measure of severity that could be derived from readily available clinical information from a

Acknowledgment

The authors thank Bevin Luna, Juliana Barnard, Trudi Madigan, Deborah Corliss, and Janice Herrell for their role in enrolling patients; Michele Cooper for technical assistance; Lee Newman for insightful discussion and careful review of the manuscript; Mary Solida for her tireless care of the patients; and the patients for their willingness to participate in this work.

References (45)

  • NevilleE et al.

    Prognostic factors predicting the outcome of sarcoidosis: an analysis of 818 patients

    Q J Med

    (1983)
  • KeoghBA et al.

    The alveolitis of pulmonary sarcoidosis: evaluation of natural history and alveolitis-dependent changes in lung function

    Am Rev Respir Dis

    (1983)
  • HunninghakeGW et al.

    Outcome of the treatment for sarcoidosis

    Am J Respir Crit Care Med

    (1994)
  • MuersMF 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)
  • GibsonGJ et al.

    British Thoracic Society Sarcoidosis study: effects of long term corticosteroid treatment

    Thorax

    (1996)
  • FerrisBG

    Epidemiology Standardization Project (American Thoracic Society)

    Am Rev Respir Dis

    (1978)
  • JudsonMA et al.

    Defining organ involvement in sarcoidosis: the ACCESS proposed instrument; ACCESS Research Group—a case control etiologic study of sarcoidosis

    Sarcoidosis Vasc Diffuse Lung Dis

    (1999)
  • WattersLC et al.

    A clinical, radiographic, and physiologic scoring system for the longitudinal assessment of patients with idiopathic pulmonary fibrosis

    Am Rev Respir Dis

    (1986)
  • KingTE et al.

    Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model

    Am J Respir Crit Care Med

    (2001)
  • WellsAU et al.

    Idiopathic pulmonary fibrosis: a composite physiologic index derived from disease extent observed by computed tomography

    Am J Respir Crit Care Med

    (2003)
  • MatoukE et al.

    Internal consistency reliability and predictive validity of a modified N. Huang clinical scoring system in adult cystic fibrosis patients

    Eur Respir J

    (1997)
  • MatoukE et al.

    Construct and longitudinal validity of a modified Huang clinical scoring system in adult cystic fibrosis patients

    Eur Respir J

    (1999)
  • Cited by (56)

    • Pulmonary Sarcoidosis: Prognostic Factors at Diagnosis in Patients from North of Portugal

      2020, Reumatologia Clinica
      Citation Excerpt :

      However, a minority of patients have a chronic course of the disease, sometimes requiring persistent immunosuppressive therapy. It has been suggested that the immunological environment may change to a T-helper 2 response that is associated with a chronic course.3,8,12,13 At diagnosis, besides Löfgren syndrome (LS), we still do not have any reliable and recognized clinical or serum markers that can predict the outcome of a particular patient.

    • Reprint of: The pathology of pulmonary sarcoidosis: update

      2018, Seminars in Diagnostic Pathology
      Citation Excerpt :

      In this recent study at the National Jewish Medical and Research Center of 104 sarcoidosis patients, 70% had extra-pulmonary organ involvement. The involved sites included, in decreasing frequency, skin, endocrine organs, extra-thoracic lymph nodes, neurologic sites, eyes, liver, spleen, bone marrow, cardiac, ear/nose/throat, parotid/salivary, muscles, bones/joint, and kidney.37,38 Necrotizing sarcoid granulomatosis (NSG) is both controversial and rare.

    • Treatment of Sarcoidosis

      2015, Clinics in Chest Medicine
    View all citing articles on Scopus

    Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).

    This research was supported by the Parker B. Francis Fellowship and by National Institutes of Health grant M01 RR00051. None of the authors are involved in any organization with a direct financial interest in the subject of the manuscript.

    View full text