Diagnostic delays, morbidity, mortality improving among patients with calciphylaxis

Key takeaways:

• Since 2006, patients with non-nephrogenic calciphylaxis have been diagnosed 13.9 days faster every year.
• In the same time period, patients with nephrogenic calciphylaxis have been diagnosed 4.38 days faster.

Time to diagnosis as well as morbidity outcomes and mortality rates among patients with calciphylaxis has steadily improved since 2006, according to a study.

“Calciphylaxis is characterized by vascular calcification, thrombosis and skin necrosis with high morbidity,” Alice J. Tan, BS, of the department of dermatology at Massachusetts General Hospital, and colleagues wrote. “Diagnosis can be challenging and may delay treatment initiation given the differing morphologies that can present depending on the clinical stage of disease, anatomic location affected, skin tone of patient and history of prior manipulation.”

Studies report varying opinions on the morbidity of the disease with some claiming low 1-year survival rates and others boasting an optimistic prognosis in light of evolving therapies. In this study, Tan and colleagues sought to evaluate the factors that contribute to diagnostic difficulty as well as the current status of morbidity outcomes and mortality rates.

The retrospective study included 302 adults diagnosed with calciphylaxis between Jan. 1, 2006, and Dec. 31, 2022.

Results showed that a greater delay in diagnosis was associated with non-nephrogenic calciphylaxis but not nephrogenic calciphylaxis, with a median time to diagnosis of 61 days vs. 33 days (P = .0004). However, since 2006, non-nephrogenic calciphylaxis patients have been diagnosed 13.9 days faster every year vs. 4.38 days faster for nephrogenic calciphylaxis patients.

Finger involvement was associated with high diagnostic delays with these patients experiencing 69.1 more days of delay vs. those with no finger involvement (P = .0001). On the other hand, those with arm and genitalia involvement were diagnosed 53.9 days (P = .01) and 35.6 days (P = .022) faster than their counterparts.

With advances in therapy, the number of complications per patient have decreased with time, especially for wound infections (P = .028), increase in lesion number (P = .012) and recurrent hospitalizations (P = .02), according to the study.

Mortality rates for nephrogenic and non-nephrogenic calciphylaxis are now 36.7% and 30.77%, respectively. According to the authors, these collective results underscore the need for “continued provider education to expedite diagnosis and ultimately improve morbidity and mortality in calciphylaxis patients.”