30+ conditions more common in EBA patients ID’d in study

The strongest risk factors for EBA were lichen planus, cutaneous lupus

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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People with epidermolysis bullosa acquisita (EBA) are at a higher risk of developing other autoimmune disorders, certain types of cancers, and heart diseases, a study suggests.

The findings also indicate that other autoimmune diseases as well as some infections may be risk factors for EBA.

The study, “Risk factors and sequelae of epidermolysis bullosa acquisita: A propensity-matched global study in 1,344 patients,” was published in Frontiers in Immunology.

EBA is an autoimmune disease caused by antibodies against the protein type VII collagen. Because it’s so rare, there is little known about its risk factors, or what conditions people with EBA are prone to developing.

Better understanding the risk factors and complications associated with EBA would “significantly improve patient outcomes because risk factors could be addressed to prevent disease onset, and screening for diseases that subsequently develop after EBA diagnosis may contribute to their early detection,” wrote the researchers in Germany who used the Global Collaborative Network of TriNetX, a global healthcare database covering nearly 115 million people, to identify 1,344 people with a recorded diagnosis of EBA.

The researchers used statistical models to look for diseases frequently diagnosed before an EBA diagnosis — suggesting they could be risk factors — or were often diagnosed after EBA was diagnosed, indicating common complications, or sequalae, of the disease.

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Associations with EBA diagnosis

Results identified 31 diseases associated with an increased risk of EBA and 37 diseases that were disproportionately common among patients with an established EBA diagnosis.

Many of the EBA-associated conditions were other autoimmune diseases. The strongest risk factors for EBA were lichen planus and cutaneous lupus — autoimmune disorders that, like EBA, affect the skin. These diseases were also common disease sequelae; the risk of having them was increased more than fivefold in EBA patients compared to the general population.

The connection between EBA and other autoimmune disorders that affect the skin suggests these diseases might share similar disease-driving mechanisms, the researchers noted.

Although several autoimmune inflammatory disorders were linked to EBA in this analysis, inflammatory bowel diseases didn’t show a link with EBA. This is notable because a few isolated reports have suggested a possible link between EBA and inflammatory bowel disease.

“An association of EBA with [inflammatory bowel disease] had long been noted … However, most of these observations were made before the establishment of the current diagnostic EBA criteria [and] were based on single case reports, case report series and meta-analysis thereof,” the researchers wrote. “Considering the findings from our study, an association of EBA with inflammatory bowel disease seems rather unlikely.” Meta-analyses pool data from multiple published studies and analyze them collectively.

Certain infectious diseases — namely sepsis, dermatophytosis (ringworm), and the fungal infection candidiasis — were more common with EBA. The researchers said this was expected since EBA patients are often put on immune-suppressing therapy to manage their disease, which can increase infection risk. These infectious diseases were also significantly more common before the EBA diagnosis, however, suggesting they may be risk factors as well as common sequelae.

Heart and metabolic health problems are common complications of EBA, the results suggested. Analyses showed increased rates of heart failure, high blood pressure, stroke, and type 2 diabetes with EBA. People with EBA are also at increased risk of skin and liver cancers.

The finding that EBA patients are prone to heart problems has potential clinical implications. The researchers said “stringent control of known cardiovascular risk factors and frequent screening for cardiovascular and metabolic disease should be implemented in the management of EBA patients.”