RESUMEN
Sarcoidosis is a multi-factorial inflammatory disease characterised by the formation of non-caseating granulomas in the affected organs. Cardiac involvement can be the first, and occasionally the only, manifestation of sarcoidosis. The prevalence of cardiac sarcoidosis (CS) is higher than previously suspected. CS is associated with increased morbidity and mortality. Thus, early diagnosis is critical to introducing immunosuppressive therapy that could prevent an adverse outcome. Endomyocardial biopsy (EMB) has limited utility in the diagnostic pathway of patients with suspected CS. As a result, advanced imaging modalities, i.e. cardiac magnetic resonance imaging (MRI) and positron emission tomography with 18F-Fluorodeoxyglucose/computed tomography scan (18F-FDG-PET/CT), have emerged as alternative tools for diagnosing CS and might be considered the new 'gold standard'. This focused review will discuss the epidemiology and pathology of CS, when to suspect and evaluate CS, highlight the complementary roles of cardiac MRI and 18F-FDG-PET/CT, and their diagnostic and prognostic values in CS, in the current content of guidelines for the diagnostic workflow of CS.
RESUMEN
BACKGROUND: Sarcoidosis is a granulomatous inflammatory disease, possibly of infectious aetiology. We aimed to investigate whether the degree of functional polarization of alveolar macrophages (AMs), or Toll-like receptor (TLR) expression, is associated with sarcoidosis or with distinct clinical manifestations of this disease. METHODS: Total BAL cells (cultured four or 24 h in medium, or stimulated 24 h with LPS) from 14 patients and six healthy subjects, sorted AMs from 22 patients (Löfgren's syndrome n = 11) and 11 healthy subjects, and sorted CD4+ T cells from 26 patients (Löfgren's syndrome n = 13) and seven healthy subjects, were included. Using real-time PCR, the relative gene expression of IL-10, IL-12p35, IL-12p40, IL-23p19, CCR2, CCR7, iNOS, CXCL10, CXCL11, CXCL16, CCL18, CCL20, CD80, and CD86, and innate immune receptors TLR2, TLR4, and TLR9, was quantified in sorted AMs, and for selected genes in total BAL cells, while IL-17A was quantified in T cells. RESULTS: We did not find evidence of a difference with regard to alveolar macrophage M1/M2 polarization between sarcoidosis patients and healthy controls. TLR2 gene expression was significantly lower in sorted AMs from patients, particular in Löfgren's patients. CCL18 gene expression in AMs was significantly higher in patients compared to controls. Additionally, the IL-17A expression was lower in Löfgren's patients' CD4+ T cells. CONCLUSIONS: Overall, there was no evidence for alveolar macrophage polarization in sarcoidosis. However, there was a reduced TLR2 mRNA expression in patients with Löfgren's syndrome, which may be of relevance for macrophage interactions with a postulated sarcoidosis pathogen, and for the characteristics of the ensuing T cell response.