Spontaneous Remission Without Steroid Therapy In Isolated Cardiac Sarcoidosis With Severe Left Ventricular Systolic Dysfunction
Ozawa, T., Goto, K., Miura, K., Kobayashi, K., Kikuta, Y., Sato, K., Taniguchi, M., Hiramatsu, S., Takebayashi, H., & Haruta, S. (2024). Spontaneous Remission Without Steroid Therapy in Isolated Cardiac Sarcoidosis with Severe Left Ventricular Systolic Dysfunction. International heart journal, 65(2), 359–362. https://doi.org/10.1536/ihj.23-399
View Original Source →Abstract
Spontaneous remission is often observed in extracardiac cases of sarcoidosis, such as skin sarcoidosis. However, for cardiac sarcoidosis (CS), the prognosis is unfavorable. Although corticosteroids are the first-line treatment for CS, data regarding the natural history of isolated CS are limited. We describe a rare case of isolated CS with severe left ventricular systolic dysfunction that improved without steroid therapy.
Case Details
Disease Location
Heart
Personal Characteristics
42-year-old female, history of atopic dermatitis
Clinical Characteristics
Referred to our hospital with shortness of breath and lower extremity edema. She had been (nyha iii). Chest x-ray showed cardiomegaly without hilar lymphadenopathy. Ecg showed a sinus rhythm without conduction abnormalities. Transthoracic echocardiography showed basal thinning of the interventricular septum and severe global lv hypokinesis (lvef 20%). Laboratory revealed elevations in brain natriuretic peptide and soluble interleukin-2 receptor. Dyspnea promptly resolved after administration of intravenous diuretic. She was started on enalapril, carvedilol, and spironolactone for heart failure. Cardiac catheterization showed normal coronary arteries but severely impaired biventricular function. Right ventricular endomyocardial biopsy showed interstitial fibrosis but no apparent granuloma. PET showed abnormal focal uptake at the basal septum and posteromedial papillary muscle. Clinical diagnosis of active isolated cardiac sarcoidosis. Corticosteroid therapy was initiated. She developed obstructive pyelonephritis secondary to upper urinary calculi and underwent transurethral ureterolithotripsy after urethral drainage
Remission Characteristics
During the 2-year follow-up period without steroid therapy both the abnormal myocardial fdg uptake and lge almost completely disappeared and lv function had improved (lvef 56%).
Treatment & Mechanisms
Clinical Treatment
Steroid therapy enalapril, carvedilol and spironolactone. Biopsy