Connective Tissue Diseases, Sepsis Lead to Higher Mortality Risk

According to a recent study, nearly 50% of patients with connective tissue disease, such as lupus, died when sepsis occurred.

Patients with connective tissue diseases (CTDs) face a higher risk of death when they are hospitalized in intensive care units (ICUs) for the treatment of sepsis, according to a new retrospective analysis.

Corresponding Marco Krasselt, MD, of the University Hospital of Leipzig, in Germany, and colleagues, noted that patients with CTDs are already known to be at higher risk of infection, owing to the frequent use of immunosuppressives to treat these patients.

In the case of patients with systemic lupus erythematosus (SLE), sepsis is the leading cause of premature death in female patients. Yet, the authors said outcomes of sepsis in patients with other CTDs, such as systemic sclerosis (SSc), dermato- or polymyositis, and Sjögren’s syndrome, are less well-understood, in part because of the relatively small incidence rates.

In their new report, published in the Journal of Intensive Care Medicine, Krasselt and colleagues described the outcomes of patients with CTDs who were admitted to the ICU at their university hospital between 2006 and 2019, using multivariate logistic regression to identify risk factors for sepsis mortality, and then calculating the predictive value of ICU scores such as the Sequential Organ Failure Assessment (SOFA).

A total of 44 patients were identified. A majority (68.2%) were female, and they had an average age of about 60 years. Most of the patients (61.4%) were diagnosed with SLE. Prior to admission, 25 patients had been treated with immunosuppressives and 36 had received glucocorticoids. Just 3 patients had previously received biologics (rituximab; Rituxan).

Overall, 49.0% of patients with CTD who were hospitalized with sepsis died.

“While we did not find a significant difference in mortality regarding the individual medication… the daily glucocorticoid dose was associated with mortality with significantly higher doses among non-survivors,” Krasselt and colleagues reported.

Though the overall rate of mortality was high, it was very high in patients with SSc, with 6 of 7 patients dying. Five of the 6 patients with SSc who died had interstitial lung disease, the investigators noted.

The authors also found that SOFA score at diagnosis was independently associated with mortality, as were 2 other ICU assessments examined in the study. The investigators calculated a SOFA cutoff score of 9 for predicting mortality with a high probability.

While the sample size in the study was small, the authors said the size is in line with similar research, noting that they were specifically focusing on a very specific category of patient: septic patients with CTD who were admitted to the ICU. The investigators said they cross-checked patient records in an effort to cut down on the risk that incorrect ICD-10 codes were used. Despite the inherent limitations of a retrospective study, the authors said they believe their study lends important insights into this patient group.

“To our knowledge, this is the first investigation to exclusively determine the mortality of ICU treatment requiring sepsis in patients with connective tissue diseases,” they concluded. The overall mortality is high and mainly driven by the increased mortality among patients with SSc.”

The results indicate physicians should be particularly vigilant with patients with high SOFA scores or a SSc diagnosis.


Krasselt M, Baerwald C, Petros S, Seifert O. Sepsis Mortality Is high in Patients With Connective Tissue Diseases Admitted to the Intensive Care Unit (ICU). J Intensive Care Med. 2022;37(3):401-407. doi:10.1177/0885066621996257

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