TABLE 7.
Features Associated With the 3 Major LD Phenotypes in JDM
Generalized LD | Partial LD | Focal LA | |
---|---|---|---|
JDM features associated with LD |
Joint contractures, calcinosis, muscle atrophy, chronic continuous illness course, malar/facial erythema, erythroderma, p155 autoantibody |
Joint contractures, calcinosis, muscle atrophy, chronic continuous illness course, malar/facial erythema, erythroderma |
Joint contractures, calcinosis, muscle atrophy, chronic continuous illness course, malar/facial erythema, panniculitis, dysphonia |
Fat loss distribution | Face, all extremities, abdomen/trunk, buccal fat loss, SQ fat loss on MRI of thighs with relative sparing of medial thigh, SQ fat loss and intraabdominal fat gain on abdominal MRI |
Face, upper > lower extremities, buccal fat loss, SQ fat loss on MRI of thighs with relative sparing of medial thigh |
Isolated area on extremity or buttock at site of calcinosis |
LD clinical features | Steatosis/NASH, ovarian enlargement, acanthosis nigricans, hypertrichosis, low leptin level |
Hepatomegaly/steatosis/NASH, ovarian enlargement, fat redistribution |
|
LD metabolic features | Hypertriglyceridemia, insulin resistance > diabetes, elevated testosterone |
Hypertriglyceridemia, insulin resistance > diabetes |
Hypertriglyceridemia |
Abbreviations: SQ = subcutaneous.