The Heart-SCC Puzzle

2) Pericardiocentesis and biopsy: Pericardiocentesis and biopsy are limited by their invasiveness. Cytology, bacteriology, and virology may be performed on the fluid and can be of important diagnostic value. If tamponade develops, emergency pericardiocentesis is needed.

Treatment

Low-dose steroids may be helpful for the control of pericardial involvement. The addition of colchicine can be an effective treatment for acute and recurrent pericarditis. This combination may be helpful in preventing recurrences in almost 90% of cases.¹⁹

Diagnosis and Management

Clinical signs suggesting cardiovascular involvement in SSc are often nonspecific. The first step in practice is to obtain a detailed history, a thorough physical examination and a standard EKG. Thereafter, the baseline evaluation consists of a 24 Holter monitoring, Doppler echocardiography, and SPECT. Assessment of Pro-BNP may be useful for the diagnosis of cardiomyopathy because its concentration is altered in patients with myocardial structural impairment even if asymptomatic: usually, pro-BNP levels are used to monitor patients with PAH.

If baseline studies are normal and the patient has no complaints, we recommend a biannual assessment with Holter and Echo-Doppler (See Figure 1, above). If Holter monitoring provides evidence of arrhythmias or conduction defects, further investigations are necessary and should be guided by the arrhythmic pattern. If the echocardiogram reveals cardiac hypertrophy, a reduced ejection fraction, or a diastolic dysfunction (inverted E/A ratio), more invasive investigations like left- and right-heart catheterization or angiography are needed. If SPECT reveals areas of inducible ischemia, coronary angiography should be performed.

Conclusion

Cardiac findings—ranging from fatal arrhythmias to congestive heart failure—remain serious manifestations of SSc and important sources of morbidity and mortality. These manifestations, which may reflect electrical, vascular, and myocardial pathology, are frequently asymptomatic and can occur alone or together. The rheumatologist must therefore confront the puzzle of cardiac involvement in SSc with a broad array of diagnostic approaches and, by determining which piece of the puzzle is jeopardizing the patient’s life, apply the right treatment. Hopefully, with a more aggressive approach and development of new modalities of treatment, improvements in patient outcome will occur and thereby change the course of this dangerous and puzzling complication of the disease.

Dr. Kaloudi is in the Division of Rheumatology at the University of Florence. Dr. Matucci Cerinic is professor of rheumatology and medicine and director of Division of Medicine and Rheumatology at the University of Florence.

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