Lupus myocarditis: review of current diagnostic modalities and their application in clinical practice

Abstract

Lupus myocarditis (LM) is a potentially fatal manifestation of systemic lupus erythematosus (SLE), occurring in 5–10% of patients. Clinical manifestations may vary from an unexplained tachycardia to fulminant congestive cardiac failure (CCF). With no single clinical or imaging modality being diagnostic, a rational and practical approach to the patient presenting with possible LM is essential. Markers of myocyte injury (including troponin-I and creatine kinase) may be unelevated and do not exclude a diagnosis of LM. Findings on electrocardiogram are non-specific but remain essential to exclude other causes of CCF such as an acute coronary syndrome or conduction disorders. Echocardiographic modalities including wall motion abnormalities and speckle tracking echocardiography may demonstrate regional and/or global left ventricular dysfunction and is more sensitive than conventional echocardiography, especially early in the course of LM. Cardiac magnetic resonance imaging (CMR) is regarded as the non-invasive diagnostic modality of choice in myocarditis. Whilst more sensitive and specific than echocardiography, CMR has certain limitations in the context of SLE including technical challenges in acutely unwell and uncooperative patients, contra-indications to gadolinium use in the context of renal impairment (including lupus nephritis) and limited literature regarding the application of recommended diagnostic CMR criteria in SLE. Both echocardiography as well as CMR may detect subclinical myocardial dysfunction and/or injury of which the clinical significance remains uncertain. Considering these challenges, a combined decision-making approach by rheumatologist and cardiologist, interpreting diagnostic test results within the clinical context of the patient is essential to ensure an accurate, early diagnosis of LM.

Pulmonary Hypertension in Connective Tissue Diseases Other than Systemic Sclerosis

“…Patients with non-SSc PAH-CTD are also at higher risk of PH via Group 2, 3, and 4 mechanisms. For example, Group 2 PH can arise from CTD-specific cardiac involvement, which includes both valvular disease (particularly in the setting of SLE-associated antiphospholipid antibodies (aPLs), 39,40 with or without antiphospholipid syndrome [APS] 41,42 ) and either systolic or diastolic left-heart dysfunction related to myocardial involvement, particularly in SLE, 43,44 MCTD, 45,46 and the IIMs. 47 Group 3 PH is typically related to interstitial lung disease (ILD), which is a common manifestation of non-SSc CTD, particularly SLE, 48,49 MCTD, 50 pSS, 51 and the IIMs.…”