Case Report Details Severe Calcinosis Universalis and Cardiac Dysfunction in Scleroderma

Medical researchers have documented the clinical presentation of a 31-year-old woman exhibiting a systemic sclerosis-predominant phenotype alongside severe left ventricular systolic dysfunction and widespread calcinosis universalis. Systemic sclerosis is a chronic autoimmune connective tissue disease characterized by vasculopathy, immune dysregulation, and tissue fibrosis. While localized calcinosis is a recognized complication of systemic sclerosis, the extensive manifestation of calcinosis universalis across multiple soft-tissue compartments remains an uncommon clinical occurrence.

The patient initially presented to clinicians with acute pulmonary oedema and newly diagnosed heart failure with reduced ejection fraction. Diagnostic evaluation using transthoracic echocardiography revealed significant cardiac alterations, including left ventricular dilatation, regional anteroseptal hypokinesia, an estimated left ventricular ejection fraction of 30 to 35 percent, elevated ventricular filling pressures, and the presence of a small pericardial effusion.

An investigation of the patient’s medical history showed approximately ten years of progressive skin sclerosis accompanied by the development of widespread, hard calcified masses.

Diagnostic imaging using conventional radiographs and computed tomography demonstrated extensive, widespread calcification affecting the subcutaneous, periarticular, and intramuscular soft tissues of the thoracoabdominal, pelvic, hip, and knee regions. These radiological findings were clinically compatible with calcinosis universalis. Furthermore, a computed tomography scan of the chest confirmed the presence of interstitial lung involvement.

Comprehensive serological profiling identified a broad and complex spectrum of circulating autoantibodies. Laboratory testing revealed strong positivity for U1-RNP/Sm, anti-Scl-70, anti-Ku, and anti-SSA antibodies, accompanied by additional immunological reactivity against anti-dsDNA, anti-Ro52, anti-Sm, and anti-nucleosome antigens.

The patient's phenotype was considered most consistent with systemic sclerosis with overlap serological features. Although the serological profile was broad, definitive diagnostic criteria for mixed connective tissue disease, systemic lupus erythematosus, and active inflammatory myopathy were not established.

The reporting authors emphasized that the underlying cause of the patient’s severe left ventricular dysfunction remains uncertain and should not be definitively attributed to systemic sclerosis. Because definitive myocardial tissue characterization and a complete etiological evaluation were unavailable, the case highlights the critical importance of distinguishing serological overlap profiles from clinically established overlap syndromes while separating unusual disease phenotypes from unproven causal relationships.

Subject of Research: Extensive calcinosis universalis and cardiac dysfunction in systemic sclerosis

Article Title: Extensive calcinosis universalis in systemic sclerosis with overlap autoantibodies and severe left ventricular systolic dysfunction: a case report

Article References: Kao, P. A., Sam, S., Sreng, D., Try, S., Chum, S., Heng, M., Cheang, K. H., & Chhar, B. (2026). Extensive calcinosis universalis in systemic sclerosis with overlap autoantibodies and severe left ventricular systolic dysfunction: a case report. BMC Rheumatology. https://doi.org/10.1186/s41927-026-00712-1

Image Credits: AI Generated

DOI: 10.1186/s41927-026-00712-1

Keywords: systemic sclerosis, calcinosis universalis, left ventricular systolic dysfunction, heart failure with reduced ejection fraction, acute pulmonary oedema, transthoracic echocardiography, interstitial lung disease, anti-Scl-70, U1-RNP, anti-Ku, anti-SSA, anti-dsDNA, anti-Sm, anti-Ro52, intramuscular calcification, pericardial effusion, case report