Cardiac Sarcoidosis: When and How to Treat Inflammation

Abstract

Sarcoidosis is a complex, multisystem inflammatory disease with a heterogeneous clinical spectrum. Approximately 25% of patients with systemic sarcoidosis will have cardiac involvement that portends a poorer outcome. The diagnosis, particularly of isolated cardiac sarcoidosis, can be challenging. A paucity of randomised data exist on who, when and how to treat myocardial inflammation in cardiac sarcoidosis. Despite this, corticosteroids continue to be the mainstay of therapy for the inflammatory phase, with an evolving role for steroid-sparing and biological agents. This review explores the immunopathogenesis of inflammation in sarcoidosis, current evidence-based treatment indications and commonly used immunosuppression agents. It explores a multidisciplinary treatment and monitoring approach to myocardial inflammation and outlines current gaps in our understanding of this condition, emerging research and future directions in this field.

The effect of immunosuppressive therapy on cardiac involvements in anti‐mitochondrial antibody‐positive myositis

“…It is notable that 27% of patients were hospitalized after their initial treatment due to cardiac involvement, indicating different reactions to treatment between cardiac involvement and myositis. Moreover, various immunosuppressive treatment responses among cardiac involvements are similar to those of CS, an inflammatory cardiomyopathy 29,30 . Although it is unclear whether arrhythmias were improved by immunosuppressive therapy rather than medication and device implantation, the response to immunosuppressive therapy might depend on the type of cardiac involvement or the phase of inflammation, as we previously reported 31 …”

“…Moreover, various immunosuppressive treatment responses among cardiac involvements are similar to those of CS, an inflammatory cardiomyopathy. 29,30 Although it is unclear whether arrhythmias were improved by immunosuppressive therapy rather than medication and device implantation, the response to immunosuppressive therapy might depend on the type of cardiac involvement or the phase of inflammation, as we previously reported. In summary, there is a growing evidence that AMA-positive myositis is frequently associated with cardiac involvements.…”

Cardiac sarcoidosis: phenotypes, diagnosis, treatment, and prognosis

“…Initiating immunosuppression for CS presupposes proof of inflammatory activity, and repeat scans may help identify response to and relapse after therapy. 60 , 61 The prognostic value of PET was confirmed in a recent meta-analysis of pertinent studies, 62 though not all works are supportive. 63 , 64 A ‘mismatch pattern’ (see Figure 2 ) and RV uptake are the key predictors of cardiac events.…”

Pragmatic electrocardiogram tracings in non-ischaemic dilated cardiomyopathy: diagnostic and prognostic role

“…Non-necrotizing granulomas are the histopathological hallmark of sarcoidosis, and therapy is principally based on immunosuppression and the management of cardiac involvement (conduction disorders, ventricular arrhythmia and heart failure). 14 The ECG is fundamental in the management of these patients, in order to identify the cardiac involvement and the severity of arrhythmias and conduction disorders. A common manifestation of cardiac sarcoidosis is the first and high degree of atrio-ventricular block.…”