Case Reports of Sclerotic Fibrosis in the Heart

A pair of case reports published in Heliyon have shed more light on the connection between systemic sclerosis and fatal heart failure, highlighting a need for early diagnosis and treatment.

A systemic autoimmune disease

Systemic sclerosis was once known as scleroderma, as the most visible signs of this disease are on the skin. However, decades of previous research have conclusively proven that this is a systemic disease, as its effects are found in multiple organ systems, most critically the heart [1]. A form of heart failure affects approximately 15% of patients [2], and they are more likely to suffer from heart inflammation, heart fibrosis, and arterial blockages. According to the authors of these case reports, this leads to frequently fatal heart attacks, and they cite previous research showing that only half of people displayed symptoms of heart disease before a heart attack [3].

These authors also note that, although the exact origins of systemic sclerosis remain unclear, the disease first manifests in the microcirculation, the network of capillaries that deliver blood to cells, and this can be diagnosed through a relatively novel technique called laser speckle contrast analysis [4], which works by detecting differences in blood pressure between various parts of the body [5].

First case report

Before he was admitted to the authors’ hospital due to showing heart attack symptoms, this 45-year-old man had suffered from fever and skin necrosis for three months, along with a three-year history of Raynaud’s phenomenon: reduced blood flow to the extremities. Although this hospital diagnosed him with systemic sclerosis and sent him to intensive care with a panoply of treatments, he died two days later.

As expected, his heart had been severely inflamed and enlarged, with the thickened tissue that is a hallmark of systemic sclerosis. Tuberculosis had taken hold in his lungs, and his right lung had necrotic tissue. The exact cause of death was a wide variety of heart diseases, including inflammation, necrosis, and fibrosis.

Second case report

The 59-year-old woman in this report had skin stiffness of the hand for five years before being admitted to the authors’ hospital, where she was diagnosed with systemic sclerosis, lung fibrosis, infection, and pulmonary hypertension. Therefore, the treatments focused on the lungs and relieved some of the related symptoms.

However, she went into fatal cardiac arrest 10 days later. An autopsy confirmed that her heart had suffered many of the same symptoms as in the first case report, with substantial amounts of fibrosis and inflammation. She had also suffered severe fibrosis of the lungs. These heart and lung issues were determined to be the final causes of death.

Conclusion

Case reports are a morbid topic, but they illustrate the reality of disease, particularly age-related disease, and heart disease is still the leading immediate cause of death in the United States. While systemic sclerosis has been reported in adults of all ages [6], it appears to lead to inflammaging and the acceleration of many processes of aging [7]. Significantly more research will need to be done to determine the origins of systemic sclerosis along with potential cures. However, as these case reports make very clear, early diagnosis of systemic diseases and their potential cardiac issues is highly likely to be beneficial on the individual level.

Literature

[1] Bruni, C., & Ross, L. (2021). Cardiac involvement in systemic sclerosis: Getting to the heart of the matter. Best Practice & Research Clinical Rheumatology, 35(3), 101668.

[2] Denton, C. P., & Khanna, D. (2017). Systemic sclerosis. The Lancet, 390(10103), 1685-1699.

[3] Marijon, E., Uy-Evanado, A., Dumas, F., Karam, N., Reinier, K., Teodorescu, C., … & Chugh, S. S. (2016). Warning symptoms are associated with survival from sudden cardiac arrest. Annals of internal medicine, 164(1), 23-29.

[4] Lambrecht, V., Cutolo, M., De Keyser, F., Decuman, S., Ruaro, B., Sulli, A., … & Smith, V. (2016). Reliability of the quantitative assessment of peripheral blood perfusion by laser speckle contrast analysis in a systemic sclerosis cohort. Annals of the Rheumatic Diseases, 75(6), 1263-1264.

[5] Sulli, A., Ruaro, B., & Cutolo, M. (2014). Evaluation of blood perfusion by laser speckle contrast analysis in different areas of hands and face in patients with systemic sclerosis. Annals of the Rheumatic Diseases, 73(11), 2059-2061.

[6] Alba, M. A., Velasco, C., Simeón, C. P., Fonollosa, V., Trapiella, L., Egurbide, M. V., … & Espinosa, G. (2014). Early-versus late-onset systemic sclerosis: differences in clinical presentation and outcome in 1037 patients. Medicine, 93(2).

[7] Shen, C. Y., Lu, C. H., Wu, C. H., Li, K. J., Kuo, Y. M., Hsieh, S. C., & Yu, C. L. (2021). Molecular Basis of Accelerated Aging with Immune Dysfunction-Mediated Inflammation (Inflamm-Aging) in Patients with Systemic Sclerosis. Cells, 10(12), 3402.