Advanced Diagnostic and Therapeutic Strategies for Sarcoidosis

Dear Colleagues,

Although almost 150 years have passed since sarcoidosis was first described, it remains an enigmatic disease that poses diagnostic and therapeutic challenges. Despite the development of molecular diagnostic techniques, researchers have not identified the aetiological agent of this rare disease, the histopathological hallmark of which is non-caseating granulomas in affected organs. It is now thought that sarcoidosis is the result of a complex immune response to an unidentified antigen in genetically predisposed individuals. The most common form is respiratory sarcoidosis, although any organ can be affected, including vital organs such as the heart or central nervous system. In the majority of patients, the granulomatous inflammation resolves spontaneously or with treatment, but in some patients, it progresses to a chronic inflammatory process, eventually leading to fibrosis or the failure of the affected organ. The diagnosis of sarcoidosis is a complex process requiring the consideration of the typical clinical and radiological picture, demonstration of the presence of non-caseating granulomas in the affected organ, and exclusion of other causes of granulomatous inflammation. The diagnosis of cardiac or CNS sarcoidosis is a real challenge and requires the collaboration between specialists with expertise in the field. Cardiac sarcoidosis is the second most common cause of death in sarcoidosis patients after pulmonary sarcoidosis. Current recommendations include active screening for cardiac sarcoidosis in all patients with newly diagnosed disease based on clinical history, physical examination and ECG. If screening is positive, the patient should undergo cardiac MRI or cardiac PET-CT to confirm cardiac involvement based on characteristic changes detected via these tests. The first-line treatment of sarcoidosis continues to be systemic corticosteroids, now used at lower doses than in the past, supported by other immunosuppressive drugs if they are ineffective or need to be prolonged, and then by biologic drugs (anti-TNF, anti-CD20). There are no clear treatment recommendations for fibrotic pulmonary sarcoidosis, which occurs in about 5 to 20 per cent of patients as an initial manifestation of the disease and in up to 25 per cent of sarcoidosis patients later in the course of a chronic inflammatory process. Only a small number of sarcoidosis patients have been included in trials of antifibrotic drugs.

The treatment of sarcoidosis patients should also address systemic symptoms such as fatigue, decreased exercise tolerance or muscle weakness, which occur due to the systemic nature of the disease and respond poorly to immunosuppressive therapy.

This Special Issue will present advanced approaches for the diagnosis and management of sarcoidosis, particularly cardiac sarcoidosis and fibrotic pulmonary sarcoidosis. Clinicians and researchers are encouraged to submit both original and review articles that address the current diagnosis of sarcoidosis using advanced imaging modalities, as well as a wide range of therapeutic approaches in patients with different sarcoidosis manifestations.

Dr. Małgorzata Sobiecka
Prof. Dr. Monika Szturmowicz
Guest Editors

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• sarcoidosis
• cardiac sarcoidosis
• pulmonary fibrosis
• magnetic resonance imaging
• FDG-PET/CT
• echocardiography
• neurosarcoidosis
• differential diagnosis
• hypercalcaemia
• IGRA
• sarcoidosis-associated pulmonary hypertension
• fatigue
• corticosteroids
• metotrexat
• infliximab
• antifibrotic treatment
• lung transplantation
• artificial intelligence
• broncho-alveolar lavage
• cytokines