Case report: Systemic lupus erythematosus combined with myocardial hypertrophy

TL;DR

This case report describes a rare instance of heart muscle thickening caused by lupus, which improved with high-dose steroid treatment.

Contribution

The paper presents a rare case of SLE-induced cardiomyopathy with reversible myocardial hypertrophy.

Findings

Severe diffuse myocardial hypertrophy was diagnosed as SLE-specific cardiomyopathy after excluding other causes.

High-dose corticosteroid therapy led to significant and reversible improvement in myocardial hypertrophy.

Myocardial biopsy showed cardiomyocyte hypertrophy and degeneration without signs of amyloidosis or myocarditis.

Abstract

Systemic lupus erythematosus (SLE) is a multisystem‐involved, highly heterogeneous autoimmune disease with diverse clinical manifestations. We report an extremely rare case of SLE with severe diffuse myocardial hypertrophy.

The patientʼs echocardiography and cardiac magnetic resonance imaging (CMR) results indicated diffuse myocardial hypertrophy. After excluding coronary atherosclerosis, hypertensive cardiomyopathy, drug toxicity, and other causes, the patient was diagnosed with SLE‐specific cardiomyopathy. Medications such as hormones, antimalarials, immunosuppressants, and biologics were administered.

Ancillary test results were as follows: hs‐cTnI: 0.054 ng/mL (0–0.016); NTproBNP: 1594.0 pg/mL (<150); A contrast‐enhanced CMR revealed the diffuse thickening of the left ventricular wall with multiple abnormal enhancements, reduced left ventricular systolic and diastolic function, and moderate amount of pericardial effusion. Endomyocardial myocardial biopsy was performed, showing cardiomyocyte hypertrophy and degeneration, and no changes in myocarditis or amyloidosis. The pathology viewed by electron microscopy showed increased intracellular glycogen in the myocardium, and no hydroxychloroquine‐associated damage in the myocardium. The 24‐h ambulatory blood pressure and contrast‐enhanced computed tomography of coronary arteries were normal. The diagnosis of SLE‐specific cardiomyopathy was clear. The myocardial hypertrophy showed reversible alleviation following treatment with high‐dose corticosteroids. CMR results before and after treatment were as follows: interventricular septum, pretreatment (28) versus post‐treatment (22) mm; left ventricular inferior wall, pretreatment (18–21) versus post‐treatment (12–14) mm; left ventricular lateral wall, pretreatment (17–18) versus post‐treatment (10–12) mm; pericardial effusion (left ventricular lateral wall), pretreatment (25) versus post‐treatment (12) mm; left ventricular ejection fraction, pretreatment (38.9%) versus post‐treatment (66%).

Myocardial hypertrophy may be an important sign of active and prognostic assessment in SLE diagnosis and management. Similarly, when encountering cases of myocardial hypertrophy, the possibility of autoimmune disease should be considered in addition to common causes.

We report a rare case of severe diffuse myocardial hypertrophy directly caused by systemic lupus erythematosus (SLE), secondary to severe heart failure.Reversible improvement in myocardial hypertrophy occurred after high‐dose corticosteroid therapy.SLE is associated with a reversible form of cardiomyopathy.Myocardial hypertrophy may be an important sign of active and prognostic assessment of SLE.When encountering cases of myocardial hypertrophy, the possibility of autoimmune disease should be considered in addition to the common causes.

We report a rare case of severe diffuse myocardial hypertrophy directly caused by systemic lupus erythematosus (SLE), secondary to severe heart failure.

Reversible improvement in myocardial hypertrophy occurred after high‐dose corticosteroid therapy.

SLE is associated with a reversible form of cardiomyopathy.

Myocardial hypertrophy may be an important sign of active and prognostic assessment of SLE.

When encountering cases of myocardial hypertrophy, the possibility of autoimmune disease should be considered in addition to the common causes.

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