Management of sarcoidosis in clinical practice

Abstract

Sarcoidosis is a systemic disease of unknown cause with very diverse presentation, outcome, severity and need for treatments. While some presentations may be very typical, for many patients, the presentation is nonspecific, with shared associations with other diseases at times being by far more frequent or misleading, which can be a cause of significant delay and often several consultations before a diagnosis of sarcoidosis can be confirmed. This is particularly the case when pulmonary manifestations are in the forefront. The diagnosis relies on three well-known criteria. In clinical practice, these criteria are not easily implemented, particularly by physicians without expertise in sarcoidosis, which can lead to a risk of either under- or over-diagnosis. Qualifying the presentation according to sarcoidosis diagnosis is essential. However, it is often not easy to classify the presentation as typical versus compatible or compatible versus inconsistent. Further investigations are needed before any other hypothesis is to be considered. It is important to detect events and to determine whether or not they are indicative of a flare of sarcoidosis. Eventually, treatment needs to be related to the correct indications. The evaluation of the efficacy and safety of treatments is crucial. To address such issues, we present five emblematic cases that illustrate this.

FIGURE 1

Thoracic high-resolution computed tomography: a) mediastinal lymphadenopathy b) without hilar lymphadenopathy (case 1).

FIGURE 2

Thoracic high-resolution computed tomography: predominant diffuse ground-glass opacities associated with a lymphatic distribution of micronodules with fissural spreading and bilateral hilar lymphadenopathy (case 2).

FIGURE 3

Chest radiography: a) association of pulmonary infiltration and bilateral hilar lymphadenopathy; b) increased infiltration; c) improvement (case 4).

FIGURE 4

Evolution of pulmonary function tests (case 4). FVC: forced vital capacity; D_LCO: diffusing capacity of the lung for carbon monoxide.

FIGURE 5

Evolution of pulmonary function tests (case 5). Arrow: diagnosis of mycobacterial infection and initiation of antimycobacterial treatment. FVC: forced vital capacity; FEV₁: forced expiratory volume in 1 s; D_LCO: diffusing capacity of the lung for carbon monoxide.

FIGURE 6

Algorithm for diagnosis and treatment of reported cases. HRCT: high-resolution computed tomography; BHL: bilateral hilar lymphadenopathy; PFT: pulmonary function test; MRI: magnetic resonance imaging; PET: positron emission tomography. ^#: blood cell counts, blood creatinine, blood calcium, 24-h urinary calcium, aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, γ-glutamyltransferase, serum protein electrophoresis and angiotensin-converting enzyme; ^¶: indications for HRCT were 1) atypical clinical and/or radiographic findings, 2) normal chest radiography but a clinical suspicion of sarcoidosis, and 3) detection of pulmonary complications.