Combined systolic and diastolic heart failure as the first presentation of mixed connective tissue disease

Abstract

A 50-year-old woman presented with combined systolic and diastolic heart failure and Raynaud’s phenomenon with painful necrotic lesions around several nail beds. Several clinical criteria and the specific biochemical findings lead to the diagnosis of mixed connective tissue disease (MCTD) which is an autoimmune connective tissue disease characterized by cutaneous and visceral fibrosis and widespread vascular pathology. After treatment with diuretics, angiotensin-converting enzyme inhibitors (ACE-inhibitor) and beta blockers the heart failure symptoms disappeared and the ejection fraction improved.The severe pain in the hands due to Raynaud’s phenomenon diminished only after treatment with calcium channel blocker nifedipine (Adalat, 10 mg, PO, Bayer HealthCare, Germany) without affecting the cardiovascular status. Cardiac involvement in connective tissue disease is not uncommon, but to our knowledge we present here the first case of MCTD with a combined systolic and diastolic heart failure as primary manifestation.

Key Words:

• heart failure
• mixed connective tissue disease
• Raynaud’s phenomenon