Quarterly medical review
Pulmonary manifestations of sarcoidosis

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Summary

Sarcoidosis affects the lungs in more than 90% of cases. Symptoms include cough, dyspnea, and chest pain. The entire respiratory tract can be involved. The most common areas of involvement are the airways and interstitium. Airway disease can lead to airway obstruction while interstitial lung disease can lead to restrictive disease. Patients may have a mix of these areas of involvement. For the symptomatic patient, the identification of disease involvement can usually be determined by pulmonary function testing and chest imaging. The chest X-ray staging system has been widely used in sarcoidosis, high-resolution computer tomography (HRCT) can provide detailed information regarding lung involvement. Unfortunately the various patterns seen on HRCT have limited the ability to develop a simple scoring system. Special studies such as bronchoscopy can be useful for detecting large airway disease. Other chest manifestations include adenopathy, pulmonary hypertension, and pulmonary muscle weakness. Fibrotic lung disease can lead to bronchiectasis, which can become infected.

In this issue

Everything you always wanted to know about sarcoidosis… but were afraid to ask

D. Valeyre, Bobigny, France and M. Humbert, Kremlin-Bicêtre, France

Pathogenesis of Sarcoidosis

J. Müller-Quernheim et al. Freiburg, Germany

Pulmonary Manifestations of Sarcoidosis

R.P. Baughman et al. Cincinnati, USA

Pulmonary hypertension complicating sarcoidosis

H. Nunes et al. Bobigny, France

Cardiac sarcoidosis

C. Chapelon-Abric, Paris, France

Neurosarcoidosis: clinical manifestations, diagnosis and treatment

K. Nozaki, Charleston, USA and M.A. Judson, Albany, USA

Ocular sarcoidosis

B. Bodaghi et al., Paris, France

Skin manifestations of sarcoidosis

J. Mañá and J. Marcoval, Barcelona, Spain

Section snippets

Upper and large airway involvement

Sarcoidosis of the upper respiratory tract (SURT) includes sinus, pharyngeal, vocal cord, tracheal, and bronchial involvement [12], [13]. Its overall incidence is about 5% of sarcoidosis patients [14]. In this chapter, we will focus on tracheal and bronchial involvement. The symptoms of tracheal and endobronchial sarcoidosis include cough, dyspnea, wheezing, and hemoptysis [15]. Bronchoscopy may reveal an endobronchial lesion (figure 1). The more common features are erythema, mucousal

Bronchiole

Airflow obstruction occurs in a significant number of patients with sarcoidosis [2], [23]. In one study, 14% of patients had a forced expiratory volume in one second (FEV-1) to forced vital capacity (FVC) ratio of less than 70% at time of diagnosis [2]. Airflow obstruction is often not responsive to bronchodilators [20], [24]. In some series, there was an association between airflow obstruction and cigarette smoking [24], but airflow obstruction has clearly been observed in sarcoidosis patients

Adenopathy

Presence of adenopathy alone (stage 1, figure 7) or with parenchymal disease (stage 2, figure 8) has been found in more than half of sarcoidosis patients at the time of diagnosis [1], [2]. While adenopathy can often be appreciated on chest roentgenogram, CT scan is far more sensitive for detecting adenopathy. Various interstitial lung disease can cause some adenopathy, but the enlargement is usually only to a mild extent. Adenopathy of greater than 15 mm in maximum diameter can help

Interstitium

Parenchymal lung involvement occurs in a significant number of patients with sarcoidosis. The most common symptom of parenchymal disease is dyspnea. Dyspnea can be assessed in several ways, including the Medical Research Council (MRC) scale [38]. However, a significant number of patients with parenchymal lung disease have no symptoms or a normal chest roentgenogram [4]. One of the striking features about sarcoidosis is the lack of correlation between chest roengenogram or pulmonary function

Pulmonary complications

Hospitalization for sarcoidosis is usually related to comorbid respiratory conditions, such as pneumonia. In the United States, African American women have a higher rate of hospitalization [69]. Infection can be a result of pulmonary sarcoidosis itself or of the use of immunosuppressive therapy to treat the disease. In a prospective 18-month study of 753 patients seen at one sarcoidosis clinic, seven (0.9%) fungal infections were diagnosed (histoplasmosis, blastomycosis, and cryptococcosis) [70]

Conclusion

There are a wide variety of pulmonary manifestations from sarcoidosis. The most commonly used tools to detect these are pulmonary function testing and chest imaging. Treatment decisions in sarcoidosis are usually based on symptoms [79]. Dyspnea and cough are the most common symptoms leading to treatment for sarcoidosis. The most commonly used tools to detect lung involvement are pulmonary function testing and chest imaging. However, these tests only mildly correlate with dyspnea [4] and are not

Disclosure of interest

the authors declare that they have no conflicts of interest concerning this article.

Glossary

CPET
cardiopulmonary exercise testing
CT
computer tomography
FEV-1
forced expiratory volume in one second
FVC
forced vital capacity
HRCT
high-resolution CT
MRC
Medical Research Council
SURT
sarcoidosis of the upper respiratory tract

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