Rituximab for the Treatment of Refractory Cardiac Sarcoidosis: A Single-Center Experience

Elwazir, Mohamed Y.; Krause, Megan L.; Bois, John P.; Christopoulos, Georgios; Kendi, Ayse T.; Cooper, Jr. Leslie T.; Jouni, Hayan; AbouEzzeddine, Omar F.; Chareonthaitawee, Panithaya; Abdel-Shafee, M; Amin, Shreyasee

doi:10.1016/j.cardfail.2021.07.008

Cited by 22 publications

(7 citation statements)

References 37 publications

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“…74 Several immunosuppressive agents can reduce the lowest effective corticosteroid dose, including methotrexate, mycophenolate, azathioprine, infliximab, adalimumab, and rituximab. [75][76][77][78] Initial combination therapy of corticosteroids with a steroid-sparing agent can be considered in severe clinical presentations or in individuals intolerant of moderate to high doses of corticosteroids. Although there is emerging interest in the routine upfront use of combination corticosteroids with steroid-sparing agents, data are lacking, and a recent survey of CS experts found no consensus on the combination approach.…”

Section: Immunomodulating Agentsmentioning

confidence: 99%

Diagnosis and Management of Cardiac Sarcoidosis: A Scientific Statement From the American Heart Association

Cheng,

Kittleson,

Beavers

et al. 2024

Circulation

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Cardiac sarcoidosis is an infiltrative cardiomyopathy that results from granulomatous inflammation of the myocardium and may present with high-grade conduction disease, ventricular arrhythmias, and right or left ventricular dysfunction. Over the past several decades, the prevalence of cardiac sarcoidosis has increased. Definitive histological confirmation is often not possible, so clinicians frequently face uncertainty about the accuracy of diagnosis. Hence, the likelihood of cardiac sarcoidosis should be thought of as a continuum (definite, highly probable, probable, possible, low probability, unlikely) rather than in a binary fashion. Treatment should be initiated in individuals with clinical manifestations and active inflammation in a tiered approach, with corticosteroids as first-line treatment. The lack of randomized clinical trials in cardiac sarcoidosis has led to treatment decisions based on cohort studies and consensus opinions, with substantial variation observed across centers. This scientific statement is intended to guide clinical practice and to facilitate management conformity by providing a framework for the diagnosis and management of cardiac sarcoidosis.

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Section: Immunomodulating Agentsmentioning

confidence: 99%

Diagnosis and Management of Cardiac Sarcoidosis: A Scientific Statement From the American Heart Association

Cheng,

Kittleson,

Beavers

et al. 2024

Circulation

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“…In support of the common genetic basis are the family reports [ 1 , 65 , 66 , 67 ]. Willoughby et al [ 1 ] identified a family cluster in three siblings.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Crohn’s Disease or Crohn’s Disease with Lung Sarcoidosis? A Case Report and Literature Review

et al. 2022

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Background: Crohn’s disease and ulcerative hemorrhagic colitis are forms of granulomatous inflammatory intestinal disease, which usually affects the gastrointestinal tract. There are also reported rare localizations at the skin, kidney, joints, liver and eye level. Pulmonary involvement is relatively rare, and it is most commonly reported in suppuration with bronchiectasis. On the other hand, sarcoidosis is, in principle, a thoracic localization of a granulomatosis disease, although bowel, skin and intestinal disorders are described. There is not a clear line to separate Crohn’s disease from sarcoidosis with, possibly because they are, in fact, considered to have the same inflammatory granulomatosis disease pathology. The diagnoses of the two entities, sarcoidosis and Crohn’s disease, are based on non-pathognomonic, inclusive clinical and paraclinical criteria, without elements of the mutual exclusion of typical locations. Case Report: We present a very rare case of a young male, already diagnosed with small-bowel Crohn’s disease. Granulomatous lung disease with major hemoptysis requires emergency surgery. An intraoperative assessment revealed a necrotic hemorrhagic lesion located in the left lower lobe and a lobectomy was performed. The final pathological report showed the presence of non-caseous granulomatous inflammation, with the identification of specific multinucleated giant cells. Conclusions: The identical diagnostic principles of Crohn’s disease and sarcoidosis, Crohn’s disease as a predecessor to pulmonary lesions, the clinical picture and the necrotico-hemorrhagic appearance of the unilateral pulmonary lesion, which are similar to aggressive necrotico-hemorrhagic or perforating intestinal forms, are arguments in favor of the diagnosis of pulmonary Crohn’s disease and not pulmonary sarcoidosis. At the same time, in general, the two diseases have overlapping elements, suggesting they are, in fact, not the same disease with different facets.

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“…This study suggests that corticosteroid-sparing regimens containing methotrexate with or without adalimumab is an effective maintenance therapy in patients after an initial response and that recurrence may occur in patients on and off immunosuppression supporting the need for ongoing radiologic surveillance regardless of immunosuppression regimen. Whether other IT such as rituximab, which has been studied in seven patients with refractory CS, or anakinra should be considered in patients with refractory CS remains to be determined [68 ▪ ,69].…”

Section: Immunosuppressive Therapy – Steroids Sparing Agentsmentioning

confidence: 99%

What cardiologists should know about cardiac sarcoidosis in 2022?

Lemay

Marchand

Sénéchal

2022

Current Opinion in Cardiology

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Purpose of reviewCardiac sarcoidosis (CS) is a potentially fatal condition when unrecognized or not treated adequately. The purpose of this review is to provide new strategies to increase clinical recognition of CS and to present an updated overview of the immunosuppressive treatments using most recent data published in the last 18 months. Recent findingsCS is an increasingly recognized pathology, and its diagnostic is made 20 times more often in the last two decades. Recent studies have shown that imaging alone usually lacks specificity to distinguish CS from other inflammatory cardiomyopathies. However, imaging can be used to increase significantly diagnostic yield of extracardiac and cardiac biopsy. Recent reviews have also demonstrated that nearly 25% of patients will be refractory to standard treatment with prednisone and that combined treatment with a corticosteroid-sparing agent is often necessary for a period that remains undetermined. SummaryCS is a complex pathology that should always require a biopsy attempt to have a histological proven diagnosis before starting immunosuppressive therapy consisting of corticosteroids with or without a corticosteroid-sparing agent.

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Rituximab for the Treatment of Refractory Cardiac Sarcoidosis: A Single-Center Experience

Cited by 22 publications

References 37 publications

Diagnosis and Management of Cardiac Sarcoidosis: A Scientific Statement From the American Heart Association

Diagnosis and Management of Cardiac Sarcoidosis: A Scientific Statement From the American Heart Association

Pulmonary Crohn’s Disease or Crohn’s Disease with Lung Sarcoidosis? A Case Report and Literature Review

What cardiologists should know about cardiac sarcoidosis in 2022?

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