Elsevier

Clinics in Chest Medicine

Volume 29, Issue 3, September 2008, Pages 459-473
Clinics in Chest Medicine

Pulmonary Sarcoidosis

https://doi.org/10.1016/j.ccm.2008.03.002Get rights and content

Sarcoidosis is an inflammatory granulomatous disease that is characterized by diverse organ system manifestations, a variable clinical course, and a predilection for affecting relatively young adults worldwide. Abnormalities on chest radiographs are detected in 85% to 95% of patients who have sarcoidosis. Approximately 20% to 50% of patients who have sarcoidosis present with respiratory symptoms, including dyspnea, cough, chest pain, and tightness of the chest. The clinical course and manifestations of pulmonary sarcoidosis are protean: spontaneous remission occurs in approximately two thirds of patients; up to 30% of patients have chronic course of the lung disease, resulting in progressive, (sometimes life-threatening) loss of lung function. Morbidity that correlates to sarcoidosis occurs in 1% to 4% of patients.

Section snippets

Clinical symptoms of pulmonary sarcoidosis

The clinical manifestations of sarcoidosis are heterogeneous and overlap with many infectious and noninfectious granulomatous disorders. Although the lung is involved in more than 90% of patients, multisystemic involvement is characteristic of the disease, and virtually any organ can be affected [1], [2], [3], [4]. In some patients, extrapulmonary manifestations are the presenting and predominant features. Recognition of extrapulmonary features of sarcoidosis is critical to ensure prompt

Fever

Sarcoidosis is an important cause of “fever of unknown origin” [11], [12], although fever is more common in tuberculosis, fungal infection, and some other granulomatous infections. Fever may occur in the early course of sarcoidosis, but fever that lasts longer than 6 weeks occurs in less than 5% of patients who have sarcoidosis [11], [12], [13], [14], [15], [16]. The fever of an early stage of sarcoidosis spontaneously remits within a few weeks in most patients [10]. Fever can be combined with

Clinical appearance of sarcoidosis

Clinical manifestations of sarcoidosis show various symptoms and signs depending on the organ involved. Commonly, sarcoidosis is the disease with an acute onset. In some patients, sarcoidosis is a chronic disease with an atypical, unusual onset, which clinically may represent a broad spectrum of any other infectious or noninfectious disease. Possible clinical manifestations of sarcoidosis are shown in Box 1.

Sarcoidosis of the airways: endobronchial sarcoidosis

The bronchial mucosa is often involved in sarcoidosis. In one study, 40% of patients with stage I and approximately 70% of patients with stages II and III had noncaseating granulomas in bronchial biopsy specimens [3]. Endobronchial sarcoidosis is commonly found as nodular elevation on the bronchial mucosa, with the nodules 2 to 3 mm in diameter. Uncommonly, there are gross mucosal abnormalities [19]. Rarely, granulomatous involvement may produce narrowing of bronchi with resulting atelectasis

Influence of clinical findings on prognosis of pulmonary sarcoidosis

Clinical manifestations of sarcoidosis may have some prognostic value. Löfgren's syndrome (manifested as BHL, erythema nodosum polyarthritis, and fever) in acute onset of sarcoidosis has excellent prognosis [6], [29], [30]. Patients have high rates of spontaneous remission. Clinical manifestations associated with poor prognosis include age at onset older than 40 years [29], [31], hypercalcemia [29], extrapulmonary involvement (ie, chronic eye, bone, or sarcoidosis of the upper respiratory tract

Influence of chest radiographic findings on prognosis

Although the chest radiographic staging system was developed approximately 50 years ago, currently it still has a certain prognostic value [4], [9]. Scandinavian authors emphasized the predominance of stages I and II with good prognosis among patients who have sarcoidosis in that part of the world [4]. The prognosis is best with radiographic stage I in patients who have sarcoidosis all over the world. In a study by Scadding [9], patients who had sarcoidosis were followed for 5 years. At the end

CT scans

A routine CT chest scan is not necessary in managing sarcoidosis [38]. Thoracic CT scans may be helpful in patients who have sarcoidosis and atypical chest radiographic findings or atypical clinical manifestations of the disease or in patients with normal chest radiographic findings but clinical suspicion for sarcoidosis [1], [22]. A thoracic CT scan is indicated to detect specific complications of sarcoidosis, such as bronchiectasis, aspergilloma, pulmonary fibrosis with honeycombing, and

Lung function impairments in sarcoidosis

“With no other disease did pulmonary physiologists have so much fun as with sarcoidosis. More is yet to come, because so much remains unexplained” [19], [40], [41], [42], [43]. This observation was written 20 years ago to introduce the topic on functional impairments in sarcoidosis at the Meeting of Sarcoidosis in Milan, Italy in 1988. Considering lung function in sarcoidosis, this introduction remains appropriate two decades later.

Respiratory tract involvement occurs at some time during the

Lung function impairments and prognosis of sarcoidosis

Criteria of assessing the response to therapy or the actual improvement of the lung function parameters have not been validated. Most authors define the increase of forced vital capacity of more than 10% to 15% and diffusing capacity for carbon monoxide of more than 20% as significant [4], [64], [65]. Several studies on lung function impairments in sarcoidosis showed the importance of lung function testing in following the course and assessing the response to the treatment. In these studies,

Diagnosis of pulmonary sarcoidosis

The diagnosis of pulmonary sarcoidosis is based on (1) recognizing the possible clinical manifestations of sarcoidosis, (2) recognizing the chest radiographic abnormalities, and (3) finding the histologic evidence of noncaseating granuloma. Finding the histologic evidence of noncaseating granuloma, the hallmark of sarcoidosis, requires lung biopsy procedures in patients with suspected pulmonary sarcoidosis. The initial procedure is flexible fiberoptic bronchoscopy with transbronchial lung

Pleural sarcoidosis

Pleural involvement is an uncommon manifestation of sarcoidosis. It may manifest as a pleural effusion, pneumothorax, pleural thickening and nodules, hydropneumothorax, trapped lung, hemothorax, or chylothorax [4], [70], [71], [72], [73], [74], [75], [76]. Clinically significant pleural manifestations (eg, pneumothorax, pleural effusions or chylothorax) occur in 2% to 4% of patients who have sarcoidosis [4], [70], [72], [77], [78], [79], [80], [81]. With the introduction of CT scan, especially

Pleural effusions

Pleural effusions complicate sarcoidosis in less than 3% of patients; when present, they are usually asymptomatic [4], [72]. The incidence of pleural effusion with sarcoidosis ranges from 0 to 5% [71], [72], [78] but has been reported to be as high as 7.5% [81]. An analysis of the published references up to 1985 that included reports of pleural involvement with sarcoidosis [14], [77], [78], [81], [82], [83], [84], [85], [86], [87], [88], [89], [90], [91], [92], [93], [94] showed that out of a

Pneumothorax

Pneumothorax occurs in 2% to 4% of patients who have sarcoidosis (Fig. 10) [4], [118], [119]. The necrosis of subpleural granuloma or the rupture of bullae or both seem to be the mechanism of this rare manifestation of sarcoidosis [71], [82]. Since the first description by Freiman in 1948, numerous reports have described the association between pneumothorax and sarcoidosis [14], [84], [85], [92], [93], [118], [119], [120], [121], [122], [123], [124], [125]. Pneumothorax may be the presenting

Pleural thickening and nodules

Pleural thickening is much more common in sarcoidosis than has been emphasized in the literature [4], [71], [72], [81], [82], [88], [106], [111]. It has been found often at thoracotomy [106] and autopsy. It is rarely extensive enough to produce clinical or physiologic consequences [105]. Pleural thickening is usually not associated with clinical symptoms [4], [72], [82]. Pleural thickening and abnormalities of the subpleural region have been recognized increasingly in sarcoidosis by use of

Summary

Sarcoidosis, an enigmatic multisystemic disease, has many different faces. Every patient has his or her own story, and every sarcoidologist has a unique but complex approach to maintaining the disease. Although multisystemic involvement is characteristic of the disease and virtually any organ can be affected, lungs are involved in more than 90% of patients who have sarcoidosis. Radiographic features and lung function impairments are supportive in managing how serious the disease is in an

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