520 - Getting Under the Skin: Rehabilitating a Rare Dermatomyositis Variant

Case Diagnosis: MDA5-Autoantibody Dermatomyositis

Case Description: A 57-year-old African-American female was admitted to acute inpatient rehabilitation for debility after hospitalization for six months of progressive fatigue and weakness.

Physical examination revealed proximal muscle weakness and rashes in heliotrope, shawl, and extensor distributions. Preliminary workup suggested discoid lupus versus dermatomyositis. Immunosuppression therapy was initiated with prednisone, hydroxychloroquine, topical tacrolimus, and topical triamcinolone.

The patient began comprehensive inpatient rehabilitation to improve mobility and function for impaired strength, endurance, and ADL performance. Prehospital level of function was mobility by wheelchair. With rehabilitation and pharmacotherapy, the patient progressed to ambulation by rolling walker, improvement in Berg Balance Score from 10 to 43, and modified independence undertaking ADLs with GG0130 Self-Care Score increasing from 14 to 41.

During her rehabilitation course, coordinated dermatology and rheumatology evaluation expedited a comprehensive workup; additional testing established diagnosis of MDA5-autoantibody dermatomyositis. Pulmonology was consulted due to this condition's association with aggressive interstitial lung disease.

Discussions: Dermatomyositis causes significant morbidity due to progressive muscle weakness and varying cutaneous, cardiovascular, gastrointestinal, and respiratory manifestations; it is also strongly associated with malignancy. The rare MDA5-autoantibody subtype, which may disproportionately affect Asians, Africans-Americans, and females, is notably linked to progressive interstitial lung disease and a high mortality.

In cases of suspected anti-MDA5 dermatomyositis, clinicians should coordinate multidisciplinary medical care alongside comprehensive rehabilitation. Given this condition’s aggressive pulmonary sequelae, expedited workup by appropriate specialists is imperative for early management of underlying pathology; this process should not be delayed until discharge. Concurrent rehabilitation can further benefit patients by targeting disease-induced functional deficits, increasing baseline fitness, and preventing disuse atrophy/deconditioning. If resources are available, accelerated diagnostic evaluation and medical management should accompany this rehabilitative care.

Conclusions: Managing suspected dermatomyositis with early, intensive rehabilitation and multidisciplinary medical care can improve patient outcomes by expediting workup, hastening necessary treatment, and improving mobility and independence.