Acute severe mitral and aortic regurgitation associated with adult-onset Still’s disease

Authors

• Wafa Baya Internal Medicine Department, Sahloul University Hospital, Sousse, Tunisia; Faculty of Medicine Ibn El Jazzar Sousse, University of Sousse, Sousse, Tunisia
• Wiem Romdhane Faculty of Medicine Ibn El Jazzar Sousse, University of Sousse, Sousse, Tunisia
• Najeh Adaily Internal Medicine Department, Sahloul University Hospital, Sousse, Tunisia; Faculty of Medicine Ibn El Jazzar Sousse, University of Sousse, Sousse, Tunisia
• Imen Ben Hassine Internal Medicine Department, Sahloul University Hospital, Sousse, Tunisia; Faculty of Medicine Ibn El Jazzar Sousse, University of Sousse, Sousse, Tunisia
• Jihed Anoun Internal Medicine Department, Sahloul University Hospital, Sousse, Tunisia; Faculty of Medicine Ibn El Jazzar Sousse, University of Sousse, Sousse, Tunisia
• Amel Rezgui Internal Medicine Department, Sahloul University Hospital, Sousse, Tunisia; Faculty of Medicine Ibn El Jazzar Sousse, University of Sousse, Sousse, Tunisia
• Fatma Ben Fredj Ismail Internal Medicine Department, Sahloul University Hospital, Sousse, Tunisia; Faculty of Medicine Ibn El Jazzar Sousse, University of Sousse, Sousse, Tunisia

bayawafa@famso.u-sousse.tn

Keywords

Abstract

Background: Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disease of unknown etiology usually affecting young adults. The primary clinical features are fever, rash, and arthritis or arthralgia. Cardiac involvement, especially valvular regurgitation, is less common. Only three cases of severe aortic and/or mitral regurgitation associated with AOSD have been reported.
Case Report: A 25-year-old man presented with fever evolving for 15 days, recent exertional dyspnea and diffuse arthromyalgia. On examination, the patient had a body temperature of 39.5°C and a pansystolic murmur at the mitral area radiating to the axilla and an early diastolic decrescendo murmur at the left sternal border with a modest intensity on cardiac auscultation. Laboratory tests revealed important leukocytosis with a predominance of neutrophils. Initial cardiac echocardiography ruled out infectious endocarditis, and revealed valvular dilated heart disease with preserved left ventricular ejection fraction with severe aortic and mitral regurgitation and a minimal tricuspid insufficiency. Pericarditis occurred secondarily. The evolution was marked by the onset of an acute left ventricular insufficiency and worsening of the pericarditis requiring a diuretic. The patient was diagnosed with AOSD using Yamaguchi’s classification criteria. He was started on 1 mg/kg/d corticosteroid therapy, and scheduled for double valve replacement. A good outcome was achieved.
Conclusion: It is paramount to identify all the clinical manifestations of AOSD with a systematic screening for cardiac involvement especially when the patient presents with discrete symptoms such as exertional dyspnea. Thereby, early diagnosis and adequate treatment reduce the disease’s critical complications.

References