Specific Scleroderma Traits at Diagnosis May Predict Morbidity and Mortality, Study Shows

Specific Scleroderma Traits at Diagnosis May Predict Morbidity and Mortality, Study Shows

Specific traits at the time of diagnosis, including symptoms and lung function status, may predict morbidity and mortality in people with scleroderma, a nine-year follow-up study shows.

The age and gender of the patient, type of scleroderma, presence of pulmonary fibrosis or hypertension, and lung function status are among the traits that were found to be predictors of morbidity and mortality in scleroderma patients.

The study reporting the findings is titled “Predictors of morbidity and mortality in early systemic sclerosis: Long-term follow-up data from a single-centre inception cohort,” and was published in the journal Autoimmunity Reviews.

Scleroderma patients are known to have a high variability of symptoms and characteristics at the time of diagnosis. Therefore, physicians and researchers have tried to identify predictors of morbidity and mortality at diagnosis to recognize groups of scleroderma patients at high risk, and to define the best treatment strategy for the patients.

This was the primary objective of the long-term, retrospective study, carried out by a research team from the National and Kapodistrian University of Athens in Greece.

Additionally, researchers tried to develop a model for three-, six-, and nine-year mortality that can distinguish between high- and low-risk patients, to be used in clinical practice.

To do so, researchers evaluated the symptoms and lung function of 115 scleroderma patients (mean age of 48.1, 54 patients with diffuse scleroderma) within 12 months of diagnosis.

Researchers found six independent predictors of mortality present at the time of scleroderma diagnosis: age at disease onset older than 45, male gender, having diffuse scleroderma, pulmonary fibrosis, pulmonary hypertension, and a DLCO (a measure of lung function) lower than 60%.

Mortality rates at three years were 14% and at 24% at six years for patients presenting three of those predictors, and 46% and 53%, respectively, for patients with four to six predictors.

The team also demonstrated that diffuse scleroderma, digital ulcers, and esophageal dysfunction can predict the occurrence of pulmonary fibrosis in the sixth year after scleroderma diagnosis.

Arrythmias at diagnosis predicted the development of pulmonary hypertension in the sixth year, while age and the presence of anti-Scl70 antibodies (a type of antibody typically present in scleroderma patients) dictated the presence of arrythmias, also in the sixth year of the disease.

The results suggest that some specific traits associated with scleroderma may be used to predict morbidity and mortality in these patients and guide treatment decisions.

“Recognizing groups of scleroderma patients at high risk could guide clinicians in decision-making processes and tailor care for individual patients,” the team concluded.

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