Characteristics of patients with dermatomyositis from 2011-2021 at a tertiary care center

Characteristics of patients with juvenile dermatomyositis from 2001-2021 at a tertiary care center

“…Notably, dystrophic calcinosis occurred in 20.9% (9/40) of patients, which is consistent with the literature but significantly higher than the 1.9% of affected adult DM patients at UC Davis [1,10]. More rare and nonspecific dermatologic examination findings included the following: 12.5% with oral mucosal involvement (e.g., ulceration), extensor surface rash, and erythema, each.…”

“…Invasive testing includes EMG and muscle biopsy, whereas non-invasive methods include MRI and laboratory evaluation of creatinine kinase (CK), aldolase, aspartate aminotransferase (AST), alanine transaminase (ALT), and lactate dehydrogenase (LDH). As expected, in our juvenile cohort, invasive methods were utilized less frequently than non-invasive tests, with 19.2% (9/47) receiving muscle biopsy and only 2.1% (1/47) receiving EMG in contrast to 26.3% (41/156) of adult DM patients undergoing EMG at UC Davis [10]. Six in ten patients (59.6%, 28/47) received a muscle MRI for JDM diagnostic purposes, with 82% (23/28) of these revealing muscle disease involvement via evidence of edema.…”

“…However, given the young age and limited length of follow-up in most, this likely does not represent our cohort's potential to develop additional autoimmune disease later in life. One in every five had a family history of autoimmune disease, which was the same frequency seen in the adult DM population at UC Davis (21.3% and 21.8%, respectively), [10].…”

“…Strikingly, less than half of patients had a dermatologist involved in their JDM care (40.4%, 19/47). Like the adult DM population at UC Davis, the top two specialties that diagnosed dermatomyositis in our cohort were Rheumatology/Allergy (48.3%, 14/29) and Dermatology (31.0%, 9/29), followed by Pediatrics (20.7%, 6/29), [10]. The remaining 18 patients did not have sufficient records available regarding which specialty made their JDM diagnosis.…”

“…In addition, elevated AST averaged 121.9 (25/32); elevated aldolase levels averaged 24 (22/32); elevated ALT averaged 114.2 (20/32); and elevated CK averaged 6188.7 (19/32). Although the adult DM population at UC Davis demonstrated an association between low complement C3 or C4 levels with clinical/laboratory evidence of muscle destruction, complement levels were very rarely tested in the JDM population [10]. Instead, lymphopenia and von Willebrand Factor (vWF) antigen, which have been suggested as indicators for disease activity [12], were present in 42.9% (18/42) and 31% (13/42) of those tested, respectively.…”