RESUMEN
Cardiac sarcoidosis (CS) is a relapsing-remitting disease, and immune suppression (IS) is the mainstay of therapy. Predictors of relapse for patients with CS in remission are not well characterized. We assessed incidence of relapse in consecutive patients with CS treated with high-dose steroids and/or steroid-sparing agents (SSA) in our center from 2000 to 2020. Remission was defined as reaching maintenance therapy (no IS, SSA, and/or prednisone ≤5 mg/d) for ≥1 month. Relapse was defined as recurrence of CS syndrome requiring IS intensification: heart failure, ventricular arrhythmia, decrease in left ventricular ejection fraction, or increased disease burden on imaging. Among 68 patients, the mean age was 50.7±9.0 years; 25 (37%) were women, and 32 (47%) were Black. In total, 59 patients (87%) reached remission. Over a median follow-up of 39.5 months (interquartile range 17.6, 92.5), 28 (48%) relapsed. Greater percentage of late gadolinium enhancement (LGE) on pretreatment magnetic resonance imaging corresponded with increased likelihood of relapse (odds ratio 1.396 per 5% increase [95% confidence interval (CI) 1.04 to 1.88]; p = 0.028). LGE ≥11% predicted elevated risk of relapse (adjusted odds ratio 4.998 [1.34 to 18.64]; p = 0.017). Shorter time to relapse was observed with isolated CS (adjusted hazard ratio 4.084 [1.44,11.56]; p = 0.008) and LGE ≥11% (adjusted hazard ratio 3.007 [1.01, 8.98]; p = 0.049). Approximately 1 in 2 patients with CS in remission experienced relapse. Greater burden of LGE on cardiac magnetic resonance imaging and isolated CS are associated with greater risk of relapse. Future work is needed to refine risk stratification for relapse and to optimize surveillance strategies on the basis of the burden of disease.
Asunto(s)
Cardiomiopatías , Miocarditis , Sarcoidosis , Humanos , Femenino , Adulto , Persona de Mediana Edad , Masculino , Cardiomiopatías/complicaciones , Medios de Contraste , Volumen Sistólico , Incidencia , Destete , Función Ventricular Izquierda , Gadolinio , Sarcoidosis/tratamiento farmacológico , Sarcoidosis/epidemiología , Sarcoidosis/complicaciones , Imagen por Resonancia Magnética/métodos , Miocarditis/complicaciones , Enfermedad Crónica , Imagen por Resonancia Cinemagnética/métodosRESUMEN
CASE DESCRIPTION: A 2.5-year-old 12.4-kg (27.3-lb) castrated male Scottish Terrier was evaluated because of episodes of hypertonia and kyphosis for which a presumptive diagnosis of so-called Scottie cramp had been made when the dog was a puppy. CLINICAL FINDINGS: Findings of general physical, orthopedic, and neurologic examinations were within reference limits. Pelvic limb hypertonicity and kyphosis without signs of pain were induced with minimal exercise; ambulation returned to normal after a period of rest. TREATMENT AND OUTCOME: Fluoxetine, a selective serotonin reuptake inhibitor, was administered orally at a dosage of 1.2 mg/kg (0.55 mg/lb) once daily for 1 month. After this period of treatment, clinical signs of the disease were greatly reduced; the dosage of fluoxetine was changed to 0.8 mg/kg (0.36 mg/lb) twice daily, and response to treatment continued. CLINICAL RELEVANCE: Administration of benzodiazepines, vitamin E, or phenothiazines has been recommended for treatment of episodes of hypertonicity, but often does not result in control of clinical signs. It has been suggested that the pathogenesis of this disease is related to deficiencies in concentration or function of serotonin in the CNS; thus, a logical choice for treatment is administration of a serotonin reuptake inhibitor. In the dog of this report, fluoxetine resulted in good control of clinical signs. The use of an effective medication (other than a controlled substance) that is administered once or twice daily, has minimal adverse effects on the patient's mental status, and is inexpensive may lead to better owner compliance and an improved quality of life for affected dogs.