Heart in a stone house: Systemic sclerosis with pericardial calcinosis

A 67-year-old female was diagnosed with systemic sclerosis (SSc) 10 years ago. She presented swollen, tight skin on her face and hands, with Raynaud’s phenomenon upon visit, and had been undergone medication with low dosage of prednisone combined with leflunomide and hydroxychloroquine for ten years. Three years ago, the patient experienced chest tightness and shortness of breath after exercise, and received electrocardiogram, which indicated atrial fibrillation with fast ventricular rate. The condition was managed with low-dose aspirin. Besides, the patient had recurrent acid reflux and burning sensation in the pharynx in the disease course. She visited our department after some 3 months of coughing and chest tightness, and was readmitted. Examination revealed tightened facial skin with telangiectasia (Figure 1A) as well as swollen and tightened skin on the fingers of both her hands, and Raynaud’s phenomenon occurred when exposed to cold (Figure 1B). The skin on the toes of the feet looked tumid and tight (Figure 1C). Further laboratory study of the antinuclear antibody spectrum noted positive anti-Scl-70 antibody, and electrocardiography exhibited rapid atrial fibrillation (Figure 1D). Chest X-ray revealed calcification of the entire pericardium, looking like a stone house (Figure 1E). Chest computed tomography (CT) noted patulous oesophagus (Figure 1F) and bilateral pleural effusion as well as extensive calcification of the pericardium (Figure 1G, 1H, 1I, 1J). Additional laboratory test findings included B-type natriuretic peptide (BNP) being 513 ng/L (normal value≤100) and hypersensitive troponin I being 0.046 μg/L (normal value 0–0.03). The patients was confirmed as SSc based on available evidences and consultation from the rheumatologists and immunologists. Despite a series of comprehensive treatment efforts such as oxygen inhalation, diuresis and improvement of cardiac function, the patient’s heart failure was further aggravated and severe hypoxemia occurred, which required tracheal intubation and ventilatory support. Palliative care was administered.

Figure 1.

(A) Facial skin with telangiectasia. (B and C) The skin is thickened. (D) ECG exhibits rapid atrial fibrillation. (E) Chest X-ray shows calcification of the entire pericardium (red arrow in Figure E). (F) Chest CT notes patulous oesophagus (red arrow in Figure F).(G, H, I, and J) Chest CT reveals pericardial calcification (red arrow in Figure G, H, I, and J), ECG, electrocardiograph; CT, computed tomography.

Scleroderma represents a group of rare conditions, affecting the skin in localized fashion or diffusely involving the blood vessels, internal organs and gastrointestinal tract, termed as systemic sclerosis. Peripheral calcinosis was not present in this patient. The patient had no previous history of tuberculosis, and was diagnosed with calcified constrictive pericarditis by chest CT. Calcific constrictive pericarditis may disturb normal diastolic filling of the heart, eventually resulting in the heart failure in patients.^[1] According to the New York Heart Association (NYHA) classification of heart failure, the patient’s heart function was rated as grade III. Additionally, calcific constrictive pericarditis is an extremely rare complication in SSc, and commonly treated conservatively except for only one case being treated with open pericardiectomy in previous report. ^[2] However, the SSc patient in current report was complicated with generalized calcific systolic pericarditis, and failed to respond to conservative medication, leading to worsening heart failure.

Funding Statement

None.