Sarcoidosis is a systemic inflammatory disease for which the etiology is unclear, that manifests noncaseating granulomas, and is usually restricted to the lungs and self-limiting. If treatment is necessary, corticosteroids remain the primary therapy. Unfortunately, sarcoidosis may progress into an interstitial lung disease process leading to respiratory failure, which may be further complicated by the development of pulmonary hypertension and right heart failure. These may become significant comorbidities that the anesthesia care team must address when the patient with complicated sarcoidosis is undergoing surgery (either associated with or independent of the sarcoidosis). The anesthesia care team needs a working understanding of sarcoidosis, its complications, and its treatment, with the aim of provicing excellent patient outcomes.
Keywordschronic granulomas, corticosteroids, interstitial lung disease, respiratory failure, systemic disease
A 50-year-old African American woman with known pulmonary sarcoidosis, stage III, is to undergo a thoracotomy for resection of a right upper lobe aspergilloma. Chronically, she has a hoarse voice, dyspnea at rest, and swollen ankles. She is receiving corticosteroids and ambulatory oxygen therapy at a rate of 2 L/min. She complains of palpitations and fainting spells.
Sarcoidosis is a systemic granulomatous disease, and although described 140 years ago, its etiology is still unknown. A complex interaction of genetic, environmental, and/or infectious antigens triggers a type 1 T-lymphocyte response that is characterized by chronic inflammation, monocyte recruitment, and granuloma formation. Pathology consists of noncaseating granulomas that are discrete and compact. Granulomas are composed of mononuclear phagocytes, such as epithelioid cells and multinucleated giant cells and lymphocytes. The central cells are surrounded by fibroblasts and mast cells ( Fig. 23.1 ).
Sarcoidosis occurs mainly between the ages of 20 and 40 years, with prevalence in the United States of approximately 20 per 100,000. It has a slightly greater female-to-male ratio, and an African American–to–Caucasian ratio of 15:1.
Sarcoidosis may present acutely, subacutely, or insidiously. Between 30% and 60% of cases are asymptomatic and incidentally detected through an abnormal chest radiograph. Constitutional symptoms may occur and consist of fever, fatigue, anorexia, cough, dyspnea, and vague retrosternal discomfort. Syndromes including erythema nodosum, anterior uveitis, arthritis, parotid enlargement, and facial nerve palsy are associated with acute sarcoidosis. In patients with an insidious presentation, respiratory symptoms usually predominate.
No specific infectious agent has been defined as an etiologic antigen. However, exposure to combustible wood products has been implicated. Interestingly, an increased incidence of a sarcoid-like granulomatous disease has been documented in the firefighters who participated in the rescue effort following the World Trade Towers 9/11 terrorist attack.
Diagnosis requires relevant clinical features and a tissue biopsy demonstrating noncaseating granulomas.
Degree and variability of disease activity together with the organ involved determine the variation in clinical presentation. These factors contribute to the delay in diagnosis seen observed in sarcoid patients.
Beryllium and hypersensitivity pneumonitis should be excluded before the diagnosis of sarcoidosis.
Death from sarcoidosis results usually from progressive pulmonary, cardiac, or neurologic involvement. Over the past 20 years the sarcoid mortality rate has increased by 3% per year.
In up to 95% cases of sarcoidosis the lung is involved.
Upper airway. Although laryngeal sarcoid may be an isolated finding, it is usually associated with systemic manifestations. Occurring in up to 5% of patients with sarcoidosis, symptoms and signs of laryngeal sarcoidosis include dysphagia, hoarseness, throat pain, dyspnea, and stridor.
Granulomas or nodules involving the supraglottic larynx or the entire larynx often are found ( Fig. 23.2 ). Airway obstruction can occur and a tracheostomy may become necessary in some cases. Recurrent laryngeal nerve involvement can result in unilateral vocal cord paralysis.
Lower airway. Enlarged intrathoracic lymph nodes can compress large airways, potentially causing tracheal and bronchial stenosis, airflow obstruction, and pulmonary atelectasis.
Pulmonary parenchyma. The lung and intrathoracic lymph nodes are involved in 90% of cases. Approximately 50% of patients develop permanent pulmonary abnormalities, with 5% to 15% developing pulmonary fibrosis. Chronic hypoxemic respiratory failure and cor pulmonale may result from pulmonary sarcoidosis.
Chest radiographic and laboratory findings. Ninety percent of patients with sarcoidosis have an abnormal chest radiograph at some time. Three classic chest radiographic patterns have been described, with increasing stages correlating with an increased frequency of dyspnea ( Table 23.1 ). An increase in the serum concentrations of angiotensin-converting enzyme (sACE), an enzyme secreted by sarcoid granulomas, can assist in making the diagnosis. Unfortunately other chronic inflammatory conditions are associated with increased sACE. Recent findings demonstrate that sACE has not been consistently correlating with disease activity or treatment response.
Chest Radiograph Pattern
Incidence of ACE Elevation (%)
Hilar and mediastinal abnormality without pulmonary parenchymal abnormality
Hilar and mediastinal abnormality associated with pulmonary parenchymal abnormality
Diffuse pulmonary disease without node enlargement
Computed tomography (CT). High-resolution computed tomography (HRCT) is helpful if the chest radiograph is equivocal and in up to 75% of cases a diagnosis can be made. HRCT can be used to diagnose small, well-defined nodes and peribronchial interstitial thickening, whereas conventional CT is better for the evaluation of diffuse parenchymal sarcoidosis ( Fig. 23.3 ).