Does systemic sclerosis kill more than before worldwide?

Systemic sclerosis, a rare autoimmune disease that affects connective tissues and multiple organs, remains one of the deadliest rheumatic diseases. A recent analysis of World Health Organization data reveals that the absolute number of deaths related to this condition increased between 2001 and 2023, rising from 1.97 to 2.34 per million inhabitants. However, when accounting for population aging, the risk of dying from this disease actually decreased over the same period, dropping from 1.58 to 1.29 per million.

Women account for nearly 80% of victims, a consistent imbalance for decades. The risk of death also increases sharply with age, peaking between 80 and 84 years. Geographic disparities are significant: the Western Pacific region has the highest mortality, while the Americas and Europe show a gradual decline. Middle-income countries record the most concerning rates, with a notable increase in some, such as the Philippines and Thailand.

This trend is partly explained by better diagnosis and earlier management in wealthy countries, where modern treatments limit severe complications. Conversely, less developed regions still suffer from delays in access to care and a lack of awareness. The most severe forms, affecting the lungs, kidneys, or heart, remain difficult to treat without adequate resources.

Genetic and environmental differences also play a role. For example, certain Asian populations are more likely to develop severe lung complications. Exposure to chemicals or silica is also suspected of worsening the disease in certain areas.

Despite these uneven advances, systemic sclerosis continues to weigh heavily on global health, with over 85,000 deaths recorded in 23 years across 74 countries. Experts emphasize the need to improve early detection and access to innovative therapies, especially in areas where the disease is still progressing.

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About This Study

DOI: https://doi.org/10.1007/s10067-026-07995-2

Title: Global trends in systemic sclerosis-related mortality, 2001–2023: an epidemiological analysis using World Health Organization mortality data

Journal: Clinical Rheumatology

Publisher: Springer Science and Business Media LLC

Authors: Keith Pardillada Belangoy; Yoshito Nishimura; Ko Harada; Hideharu Hagiya; Quynh Thi Vu; Hanane Ouddoud; Judah Israel Ong Lescano; Michio Yamamoto; Tatsuaki Takeda; Hirofumi Hamano; Toshihiro Koyama; Yoshito Zamami