There is a large spectrum of extrapulmonary sarcoidosis; non-pulmonary, non-cardiac specific visceral involvements with potential risks, impaired functionality and quality of life (QoL). Virtually all organs can be involved. Its presentation can be initial (pulmonary, or extrapulmonary) or delayed. The most frequent is the initial presentation with the implication of parotids, and less frequently with nasosinusal, nervous system, kidney and peripheral adenopathy implications. The diagnosis at presentation should include a minimum of physical examination, blood analysis, ECG and routine ophthalmologic examination. A biopsy is needed to confirm whether there is renal, muscle, liver, bone marrow or ENT implications. Treatment has to be considered in situations of risk and/or when there is significant QoL impairment. It’s important to consider endpoint measurements and take into consideration adverse events (particularly those from corticosteroids). Collegial decisions between organ specialists should be optimised, ideally experts in sarcoidosis. It’s important to focus on the treatment of the patient rather than within organs, sharing decisions with the individual.
There are no definitive diagnostic criteria for cardiac sarcoidosis. At present cardiac magnetic resonance (CMR) and cardiac PET show advanced imaging techniques, PET being superior to Gallium scintigraphy, and to the Japanese Ministry of Health and Welfare (JMHW) criteria for diagnosis and prognosis. Clinical diagnosis of cardiac sarcoidosis includes the evaluation of cardiac rhythm and function and myocardial tissue. An accurate diagnosis of cardiac sarcoidosis needs a multidisciplinary approach, including respiratory physicians, cardiologists, echocardiography, nuclear medicine and a cardiac MRI. There is limited availability of controlled data of treatments. Early treatment should be definitive in cases with mayor organ involvement. Initially, the level of inflammation should be brought under control. Secondly, the objective is to taper the treatment dose to maintain efficacy and avoid side effects. Different approaches have been studied with good results: prednisone followed by maintenance therapy with methotrexate or either adalimumab or infliximab in refractory CS patients.